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Laura Barisoni

Professor of Pathology
Pathology
Box 3712, Durham, NC 27710
40 Duke Medicine Circle, Box 3712, Durham, NC 27710

Selected Publications


Rationale and design of the Nephrotic Syndrome Study Network (NEPTUNE) Match in glomerular diseases: designing the right trial for the right patient, today.

Journal Article Kidney Int · February 2024 Glomerular diseases are classified using a descriptive taxonomy that is not reflective of the heterogeneous underlying molecular drivers. This limits not only diagnostic and therapeutic patient management, but also impacts clinical trials evaluating target ... Full text Link to item Cite

Tubular and Glomerular Size by Cortex Depth as Predictor of Progressive CKD after Radical Nephrectomy for Tumor.

Journal Article J Am Soc Nephrol · September 1, 2023 SIGNIFICANCE STATEMENT: Glomerular size differs by cortex depth. Larger nephrons are prognostic of progressive kidney disease, but it is unknown whether this risk differs by cortex depth or by glomeruli versus proximal or distal tubule size. We studied the ... Full text Link to item Cite

Age-Based Versus Young-Adult Thresholds for Nephrosclerosis on Kidney Biopsy and Prognostic Implications for CKD.

Journal Article J Am Soc Nephrol · August 1, 2023 SIGNIFICANCE STATEMENT: Nephrosclerosis (glomerulosclerosis, interstitial fibrosis, and tubular atrophy) is the defining pathology of both kidney aging and CKD. Optimal thresholds for nephrosclerosis that identify persons with a progressive disease are unk ... Full text Link to item Cite

Progenitor hierarchy among parietal epithelial cells depicted at the single-cell level.

Journal Article Kidney Int · July 2023 The role of parietal epithelial cells (PECs) in kidney function and disease was recently revisited. Building on previous studies of human kidney tissue, in the current issue, Liu et al. further characterize PECs using single-cell RNA sequencing data and co ... Full text Link to item Cite

An atlas of healthy and injured cell states and niches in the human kidney.

Journal Article Nature · July 2023 Understanding kidney disease relies on defining the complexity of cell types and states, their associated molecular profiles and interactions within tissue neighbourhoods1. Here we applied multiple single-cell and single-nucleus assays (>400,000 nuclei or  ... Full text Link to item Cite

Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network.

Journal Article Am J Kidney Dis · June 2023 RATIONALE & OBJECTIVE: Adolescent- and adult-onset minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulonephropathy Network (CureGN) ... Full text Link to item Cite

Daratumumab Use Prior to Kidney Transplant and T Cell-Mediated Rejection: A Case Report.

Journal Article Am J Kidney Dis · May 2023 There is growing interest in daratumumab in the solid organ transplant realm owing to the potential immunomodulatory effects on CD38-expressing cells, primarily plasma cells, as they have a key role in antibody production. In particular there is interest i ... Full text Link to item Cite

Clinical Relevance of Computationally Derived Attributes of Peritubular Capillaries from Kidney Biopsies.

Journal Article Kidney360 · May 1, 2023 KEY POINTS: Computational image analysis allows for the extraction of new information from whole-slide images with potential clinical relevance. Peritubular capillary (PTC) density is decreased in areas of interstitial fibrosis and tubular atrophy when mea ... Full text Link to item Cite

Inaxaplin for Proteinuric Kidney Disease in Persons with Two APOL1 Variants.

Journal Article N Engl J Med · March 16, 2023 BACKGROUND: Persons with toxic gain-of-function variants in the gene encoding apolipoprotein L1 (APOL1) are at greater risk for the development of rapidly progressive, proteinuric nephropathy. Despite the known genetic cause, therapies targeting proteinuri ... Full text Link to item Cite

Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis.

Journal Article Kidney Int · March 2023 The diagnosis of nephrotic syndrome relies on clinical presentation and descriptive patterns of injury on kidney biopsies, but not specific to underlying pathobiology. Consequently, there are variable rates of progression and response to therapy within dia ... Full text Link to item Cite

Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease.

Journal Article Am J Kidney Dis · March 2023 RATIONALE & OBJECTIVE: The effects of race, ethnicity, socioeconomic status (SES), and disease severity on acute care utilization in patients with glomerular disease are unknown. STUDY DESIGN: Prospective cohort study. SETTING & PARTICIPANTS: 1,456 adults ... Full text Link to item Cite

Decoding the Encoder

Conference Conference Proceedings - IEEE SOUTHEASTCON · January 1, 2023 Autoencoders are used in a variety of safety-critical applications. Uncertainty quantification is a key component to bolster the trustworthiness of such models. With the growing complexity of the autoencoder design and the dataset they are trained on, ther ... Full text Cite

Banff Digital Pathology Working Group: Image Bank, Artificial Intelligence Algorithm, and Challenge Trial Developments.

Journal Article Transpl Int · 2023 The Banff Digital Pathology Working Group (DPWG) was established with the goal to establish a digital pathology repository; develop, validate, and share models for image analysis; and foster collaborations using regular videoconferencing. During the calls, ... Full text Link to item Cite

A glomerular transcriptomic landscape of apolipoprotein L1 in Black patients with focal segmental glomerulosclerosis.

Journal Article Kidney Int · July 2022 Apolipoprotein L1 (APOL1)-associated focal segmental glomerulosclerosis (FSGS) is the dominant form of FSGS in Black individuals. There are no targeted therapies for this condition, in part because the molecular mechanisms underlying APOL1's pathogenic con ... Full text Link to item Cite

Kidney Biopsy Features Most Predictive of Clinical Outcomes in the Spectrum of Minimal Change Disease and Focal Segmental Glomerulosclerosis.

Journal Article J Am Soc Nephrol · July 2022 BACKGROUND: Heterogeneity in disease course and treatment response among patients with MCD/FSGS necessitates a granular evaluation of kidney tissue features. This study aimed to identify histologic and ultrastructural descriptors of structural changes most ... Full text Link to item Cite

A reference tissue atlas for the human kidney.

Journal Article Sci Adv · June 10, 2022 Kidney Precision Medicine Project (KPMP) is building a spatially specified human kidney tissue atlas in health and disease with single-cell resolution. Here, we describe the construction of an integrated reference map of cells, pathways, and genes using un ... Full text Link to item Cite

Quantification of Glomerular Structural Lesions: Associations With Clinical Outcomes and Transcriptomic Profiles in Nephrotic Syndrome.

Journal Article Am J Kidney Dis · June 2022 RATIONALE & OBJECTIVE: The current classification system for focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) does not fully capture the complex structural changes in kidney biopsies nor the clinical and molecular heterogeneity of ... Full text Link to item Cite

Impact of Consensus Definitions on Identification of Glomerular Lesions by Light and Electron Microscopy.

Journal Article Kidney Int Rep · January 2022 INTRODUCTION: In 2020, a working group of 13 renal pathologists published consensus definitions for 47 individual glomerular lesions found on light microscopy (LM) and 47 glomerular lesions and 9 normal structures found on electron microscopy (EM). METHODS ... Full text Link to item Cite

Rationale and design of the Nephrotic Syndrome Study Network (NEPTUNE) Match in glomerular diseases: designing the right trial for the right patient, today.

Journal Article Kidney Int · February 2024 Glomerular diseases are classified using a descriptive taxonomy that is not reflective of the heterogeneous underlying molecular drivers. This limits not only diagnostic and therapeutic patient management, but also impacts clinical trials evaluating target ... Full text Link to item Cite

Tubular and Glomerular Size by Cortex Depth as Predictor of Progressive CKD after Radical Nephrectomy for Tumor.

Journal Article J Am Soc Nephrol · September 1, 2023 SIGNIFICANCE STATEMENT: Glomerular size differs by cortex depth. Larger nephrons are prognostic of progressive kidney disease, but it is unknown whether this risk differs by cortex depth or by glomeruli versus proximal or distal tubule size. We studied the ... Full text Link to item Cite

Age-Based Versus Young-Adult Thresholds for Nephrosclerosis on Kidney Biopsy and Prognostic Implications for CKD.

Journal Article J Am Soc Nephrol · August 1, 2023 SIGNIFICANCE STATEMENT: Nephrosclerosis (glomerulosclerosis, interstitial fibrosis, and tubular atrophy) is the defining pathology of both kidney aging and CKD. Optimal thresholds for nephrosclerosis that identify persons with a progressive disease are unk ... Full text Link to item Cite

Progenitor hierarchy among parietal epithelial cells depicted at the single-cell level.

Journal Article Kidney Int · July 2023 The role of parietal epithelial cells (PECs) in kidney function and disease was recently revisited. Building on previous studies of human kidney tissue, in the current issue, Liu et al. further characterize PECs using single-cell RNA sequencing data and co ... Full text Link to item Cite

An atlas of healthy and injured cell states and niches in the human kidney.

Journal Article Nature · July 2023 Understanding kidney disease relies on defining the complexity of cell types and states, their associated molecular profiles and interactions within tissue neighbourhoods1. Here we applied multiple single-cell and single-nucleus assays (>400,000 nuclei or  ... Full text Link to item Cite

Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network.

Journal Article Am J Kidney Dis · June 2023 RATIONALE & OBJECTIVE: Adolescent- and adult-onset minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulonephropathy Network (CureGN) ... Full text Link to item Cite

Daratumumab Use Prior to Kidney Transplant and T Cell-Mediated Rejection: A Case Report.

Journal Article Am J Kidney Dis · May 2023 There is growing interest in daratumumab in the solid organ transplant realm owing to the potential immunomodulatory effects on CD38-expressing cells, primarily plasma cells, as they have a key role in antibody production. In particular there is interest i ... Full text Link to item Cite

Clinical Relevance of Computationally Derived Attributes of Peritubular Capillaries from Kidney Biopsies.

Journal Article Kidney360 · May 1, 2023 KEY POINTS: Computational image analysis allows for the extraction of new information from whole-slide images with potential clinical relevance. Peritubular capillary (PTC) density is decreased in areas of interstitial fibrosis and tubular atrophy when mea ... Full text Link to item Cite

Inaxaplin for Proteinuric Kidney Disease in Persons with Two APOL1 Variants.

Journal Article N Engl J Med · March 16, 2023 BACKGROUND: Persons with toxic gain-of-function variants in the gene encoding apolipoprotein L1 (APOL1) are at greater risk for the development of rapidly progressive, proteinuric nephropathy. Despite the known genetic cause, therapies targeting proteinuri ... Full text Link to item Cite

Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis.

Journal Article Kidney Int · March 2023 The diagnosis of nephrotic syndrome relies on clinical presentation and descriptive patterns of injury on kidney biopsies, but not specific to underlying pathobiology. Consequently, there are variable rates of progression and response to therapy within dia ... Full text Link to item Cite

Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease.

Journal Article Am J Kidney Dis · March 2023 RATIONALE & OBJECTIVE: The effects of race, ethnicity, socioeconomic status (SES), and disease severity on acute care utilization in patients with glomerular disease are unknown. STUDY DESIGN: Prospective cohort study. SETTING & PARTICIPANTS: 1,456 adults ... Full text Link to item Cite

Decoding the Encoder

Conference Conference Proceedings - IEEE SOUTHEASTCON · January 1, 2023 Autoencoders are used in a variety of safety-critical applications. Uncertainty quantification is a key component to bolster the trustworthiness of such models. With the growing complexity of the autoencoder design and the dataset they are trained on, ther ... Full text Cite

Banff Digital Pathology Working Group: Image Bank, Artificial Intelligence Algorithm, and Challenge Trial Developments.

Journal Article Transpl Int · 2023 The Banff Digital Pathology Working Group (DPWG) was established with the goal to establish a digital pathology repository; develop, validate, and share models for image analysis; and foster collaborations using regular videoconferencing. During the calls, ... Full text Link to item Cite

A glomerular transcriptomic landscape of apolipoprotein L1 in Black patients with focal segmental glomerulosclerosis.

Journal Article Kidney Int · July 2022 Apolipoprotein L1 (APOL1)-associated focal segmental glomerulosclerosis (FSGS) is the dominant form of FSGS in Black individuals. There are no targeted therapies for this condition, in part because the molecular mechanisms underlying APOL1's pathogenic con ... Full text Link to item Cite

Kidney Biopsy Features Most Predictive of Clinical Outcomes in the Spectrum of Minimal Change Disease and Focal Segmental Glomerulosclerosis.

Journal Article J Am Soc Nephrol · July 2022 BACKGROUND: Heterogeneity in disease course and treatment response among patients with MCD/FSGS necessitates a granular evaluation of kidney tissue features. This study aimed to identify histologic and ultrastructural descriptors of structural changes most ... Full text Link to item Cite

A reference tissue atlas for the human kidney.

Journal Article Sci Adv · June 10, 2022 Kidney Precision Medicine Project (KPMP) is building a spatially specified human kidney tissue atlas in health and disease with single-cell resolution. Here, we describe the construction of an integrated reference map of cells, pathways, and genes using un ... Full text Link to item Cite

Quantification of Glomerular Structural Lesions: Associations With Clinical Outcomes and Transcriptomic Profiles in Nephrotic Syndrome.

Journal Article Am J Kidney Dis · June 2022 RATIONALE & OBJECTIVE: The current classification system for focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) does not fully capture the complex structural changes in kidney biopsies nor the clinical and molecular heterogeneity of ... Full text Link to item Cite

Impact of Consensus Definitions on Identification of Glomerular Lesions by Light and Electron Microscopy.

Journal Article Kidney Int Rep · January 2022 INTRODUCTION: In 2020, a working group of 13 renal pathologists published consensus definitions for 47 individual glomerular lesions found on light microscopy (LM) and 47 glomerular lesions and 9 normal structures found on electron microscopy (EM). METHODS ... Full text Link to item Cite

Case Report: Unusual Aggregation of Different Glomerulopathies in a Family Resolved by Genetic Testing and Reverse Phenotyping.

Journal Article Front Pediatr · 2022 Glomerular diseases (GDs) are a major cause of chronic kidney disease in children. The conventional approach to diagnosis of GDs includes clinical evaluation and, in most cases, kidney biopsy to make a definitive diagnosis. However, in many cases, clinical ... Full text Link to item Cite

A user-friendly tool for cloud-based whole slide image segmentation with examples from renal histopathology.

Journal Article Commun Med (Lond) · 2022 BACKGROUND: Image-based machine learning tools hold great promise for clinical applications in pathology research. However, the ideal end-users of these computational tools (e.g., pathologists and biological scientists) often lack the programming experienc ... Full text Link to item Cite

Deep learning segmentation of glomeruli on kidney donor frozen sections.

Journal Article J Med Imaging (Bellingham) · November 2021 Purpose: Recent advances in computational image analysis offer the opportunity to develop automatic quantification of histologic parameters as aid tools for practicing pathologists. We aim to develop deep learning (DL) models to quantify nonsclerotic and s ... Full text Link to item Cite

APOL1 genotype-associated morphologic changes among patients with focal segmental glomerulosclerosis.

Journal Article Pediatr Nephrol · September 2021 BACKGROUND: The G1 and G2 alleles of apolipoprotein L1 (APOL1) are common in the Black population and associated with increased risk of focal segmental glomerulosclerosis (FSGS). The molecular mechanisms linking APOL1 risk variants with FSGS are not clearl ... Full text Link to item Cite

Compounds targeting OSBPL7 increase ABCA1-dependent cholesterol efflux preserving kidney function in two models of kidney disease.

Journal Article Nat Commun · August 2, 2021 Impaired cellular cholesterol efflux is a key factor in the progression of renal, cardiovascular, and autoimmune diseases. Here we describe a class of 5-arylnicotinamide compounds, identified through phenotypic drug discovery, that upregulate ABCA1-depende ... Full text Link to item Cite

Deep-Learning-Driven Quantification of Interstitial Fibrosis in Digitized Kidney Biopsies.

Journal Article Am J Pathol · August 2021 Interstitial fibrosis and tubular atrophy (IFTA) on a renal biopsy are strong indicators of disease chronicity and prognosis. Techniques that are typically used for IFTA grading remain manual, leading to variability among pathologists. Accurate IFTA estima ... Full text Link to item Cite

Ferroptotic stress promotes the accumulation of pro-inflammatory proximal tubular cells in maladaptive renal repair.

Journal Article Elife · July 19, 2021 Overwhelming lipid peroxidation induces ferroptotic stress and ferroptosis, a non-apoptotic form of regulated cell death that has been implicated in maladaptive renal repair in mice and humans. Using single-cell transcriptomic and mouse genetic approaches, ... Full text Open Access Link to item Cite

Improving data quality in observational research studies: Report of the Cure Glomerulonephropathy (CureGN) network.

Journal Article Contemp Clin Trials Commun · June 2021 BACKGROUND: High data quality is of crucial importance to the integrity of research projects. In the conduct of multi-center observational cohort studies with increasing types and quantities of data, maintaining data quality is challenging, with few publis ... Full text Link to item Cite

Large-scale, three-dimensional tissue cytometry of the human kidney: a complete and accessible pipeline.

Journal Article Lab Invest · May 2021 The advent of personalized medicine has driven the development of novel approaches for obtaining detailed cellular and molecular information from clinical tissue samples. Tissue cytometry is a promising new technique that can be used to enumerate and chara ... Full text Link to item Cite

Large-scale, three-dimensional tissue cytometry of the human kidney: a complete and accessible pipeline.

Journal Article Lab Invest · May 2021 The advent of personalized medicine has driven the development of novel approaches for obtaining detailed cellular and molecular information from clinical tissue samples. Tissue cytometry is a promising new technique that can be used to enumerate and chara ... Full text Link to item Cite

Podocyte density is reduced in kidney allografts with high-risk APOL1 genotypes at transplantation.

Journal Article Clin Transplant · April 2021 Variants in apolipoprotein L1 (APOL1) gene are associated with nondiabetic kidney diseases in black subjects and reduced kidney transplant graft survival. Living and deceased black kidney donors (n = 107) were genotyped for APOL1 variants. To determine whe ... Full text Link to item Cite

Assessment of a computerized quantitative quality control tool for whole slide images of kidney biopsies.

Journal Article J Pathol · March 2021 Inconsistencies in the preparation of histology slides and whole-slide images (WSIs) may lead to challenges with subsequent image analysis and machine learning approaches for interrogating the WSI. These variabilities are especially pronounced in multicent ... Full text Link to item Cite

Rationale and design of the Kidney Precision Medicine Project.

Journal Article Kidney Int · March 2021 Chronic kidney disease (CKD) and acute kidney injury (AKI) are common, heterogeneous, and morbid diseases. Mechanistic characterization of CKD and AKI in patients may facilitate a precision-medicine approach to prevention, diagnosis, and treatment. The Kid ... Full text Link to item Cite

Patient perspectives and involvement in precision medicine research.

Journal Article Kidney Int · March 2021 The Kidney Precision Medicine Project will advance understanding of chronic kidney disease attributed to diabetes or hypertension and acute kidney injury through a protocol kidney biopsy used for deep phenotyping with state-of-the-art methodology. To guide ... Full text Link to item Cite

Development and evaluation of deep learning-based segmentation of histologic structures in the kidney cortex with multiple histologic stains.

Journal Article Kidney Int · January 2021 The application of deep learning for automated segmentation (delineation of boundaries) of histologic primitives (structures) from whole slide images can facilitate the establishment of novel protocols for kidney biopsy assessment. Here, we developed and v ... Full text Link to item Cite

A multimodal and integrated approach to interrogate human kidney biopsies with rigor and reproducibility: guidelines from the Kidney Precision Medicine Project.

Journal Article Physiol Genomics · January 1, 2021 Comprehensive and spatially mapped molecular atlases of organs at a cellular level are a critical resource to gain insights into pathogenic mechanisms and personalized therapies for diseases. The Kidney Precision Medicine Project (KPMP) is an endeavor to g ... Full text Link to item Cite

Digital pathology and computational image analysis in nephropathology.

Journal Article Nat Rev Nephrol · November 2020 The emergence of digital pathology - an image-based environment for the acquisition, management and interpretation of pathology information supported by computational techniques for data extraction and analysis - is changing the pathology ecosystem. In par ... Full text Link to item Cite

Consensus definitions for glomerular lesions by light and electron microscopy: recommendations from a working group of the Renal Pathology Society.

Journal Article Kidney Int · November 2020 Over the past 2 decades, scoring systems for multiple glomerular diseases have emerged, as have consortia of pathologists and nephrologists for the study of glomerular diseases, including correlation of pathologic findings with clinical features and outcom ... Full text Link to item Cite

Modelling kidney disease using ontology: insights from the Kidney Precision Medicine Project.

Journal Article Nat Rev Nephrol · November 2020 An important need exists to better understand and stratify kidney disease according to its underlying pathophysiology in order to develop more precise and effective therapeutic agents. National collaborative efforts such as the Kidney Precision Medicine Pr ... Full text Link to item Cite

Single cell transcriptomics of mouse kidney transplants reveals a myeloid cell pathway for transplant rejection.

Journal Article JCI Insight · October 15, 2020 Myeloid cells are increasingly recognized as major players in transplant rejection. Here, we used a murine kidney transplantation model and single cell transcriptomics to dissect the contribution of myeloid cell subsets and their potential signaling pathwa ... Full text Open Access Link to item Cite

Banff Digital Pathology Working Group: Going digital in transplant pathology.

Journal Article Am J Transplant · September 2020 The Banff Digital Pathology Working Group (DPWG) was formed in the time leading up to and during the joint American Society for Histocompatibility and Immunogenetics/Banff Meeting, September 23-27, 2019, held in Pittsburgh, Pennsylvania. At the meeting, th ... Full text Link to item Cite

The longitudinal relationship between patient-reported outcomes and clinical characteristics among patients with focal segmental glomerulosclerosis in the Nephrotic Syndrome Study Network.

Journal Article Clin Kidney J · August 2020 BACKGROUND: Understanding the relationship between clinical and patient-reported outcomes (PROs) will help support clinical care and future clinical trial design of novel therapies for focal segmental glomerulosclerosis (FSGS). METHODS: FSGS patients ≥8 ye ... Full text Link to item Cite

Integrin-β1 is required for the renal cystogenesis caused by ciliary defects.

Journal Article Am J Physiol Renal Physiol · May 1, 2020 Defects in the function of primary cilia are commonly associated with the development of renal cysts. On the other hand, the intact cilium appears to contribute a cystogenic signal whose effectors remain unclear. As integrin-β1 is required for the cystogen ... Full text Link to item Cite

Ultrastructural Characterization of Proteinuric Patients Predicts Clinical Outcomes.

Journal Article J Am Soc Nephrol · April 2020 BACKGROUND: The analysis and reporting of glomerular features ascertained by electron microscopy are limited to few parameters with minimal predictive value, despite some contributions to disease diagnoses. METHODS: We investigated the prognostic value of ... Full text Link to item Cite

Prediction of transcriptional signatures in glomeruli of a mouse model of nephropathy using the human Global Prior rank

Conference The FASEB Journal · April 2020 BackgroundDifferential gene expression is commonly used to provide insight into the molecular mechanisms of disease. Each human or murine cell contains roughly 20000 protein coding genes and 30 to 50% are expected ... Full text Cite

Research Highlights

Journal Article Transplantation · November 1, 2019 Full text Cite

Efficacy of the pharmacologic chaperone migalastat in a subset of male patients with the classic phenotype of Fabry disease and migalastat-amenable variants: data from the phase 3 randomized, multicenter, double-blind clinical trial and extension study.

Journal Article Genet Med · September 2019 PURPOSE: Outcomes in patients with Fabry disease receiving migalastat during the phase 3 FACETS trial (NCT00925301) were evaluated by phenotype. METHODS: Data were evaluated in two subgroups of patients with migalastat-amenable GLA variants: "classic pheno ... Full text Link to item Cite

Prevalence of Cardiovascular Disease Risk Factors in Childhood Glomerular Diseases.

Journal Article J Am Heart Assoc · July 16, 2019 Background Cardiovascular disease is a major cause of morbidity and mortality in children with chronic kidney disease. We sought to determine the prevalence of cardiovascular risk factors in children with glomerular disease and to describe current practice ... Full text Link to item Cite

SMPDL3b modulates insulin receptor signaling in diabetic kidney disease.

Journal Article Nat Commun · June 19, 2019 Sphingomyelin phosphodiesterase acid-like 3b (SMPDL3b) is a lipid raft enzyme that regulates plasma membrane (PM) fluidity. Here we report that SMPDL3b excess, as observed in podocytes in diabetic kidney disease (DKD), impairs insulin receptor isoform B-de ... Full text Link to item Cite

Pegunigalsidase alfa, a novel PEGylated enzyme replacement therapy for Fabry disease, provides sustained plasma concentrations and favorable pharmacodynamics: A 1-year Phase 1/2 clinical trial.

Journal Article J Inherit Metab Dis · May 2019 Pegunigalsidase alfa, a novel PEGylated, covalently crosslinked form of α-galactosidase A developed as enzyme replacement therapy (ERT) for Fabry disease (FD), was designed to increase plasma half-life and reduce immunogenicity, thereby enhancing efficacy ... Full text Link to item Cite

Health-related quality of life in glomerular disease.

Journal Article Kidney Int · May 2019 There is scant literature describing the effect of glomerular disease on health-related quality of life (HRQOL). The Cure Glomerulonephropathy study (CureGN) is an international longitudinal cohort study of children and adults with four primary glomerular ... Full text Link to item Cite

CureGN Study Rationale, Design, and Methods: Establishing a Large Prospective Observational Study of Glomerular Disease.

Journal Article Am J Kidney Dis · February 2019 RATIONALE & OBJECTIVES: Glomerular diseases, including minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and immunoglobulin A (IgA) nephropathy, share clinical presentations, yet result from multiple biological mechanisms. ... Full text Link to item Cite

Organoid single cell profiling identifies a transcriptional signature of glomerular disease.

Journal Article JCI Insight · January 10, 2019 Podocyte injury is central to many forms of kidney disease, but transcriptional signatures reflecting podocyte injury and compensation mechanisms are challenging to analyze in vivo. Human kidney organoids derived from pluripotent stem cells (PSCs), a poten ... Full text Link to item Cite

Open-Label Clinical Trials of Oral Pulse Dexamethasone for Adults with Idiopathic Nephrotic Syndrome.

Journal Article Am J Nephrol · 2019 BACKGROUND: In adults with primary focal segmental glomerulosclerosis (FSGS), daily prednisone may induce complete remissions (CR) and partial remissions (PR), but relapses are frequent and adverse events are common. METHODS: We carried out 2 open-label, u ... Full text Link to item Cite

Plasma Zonulin Levels in Childhood Nephrotic Syndrome.

Journal Article Front Pediatr · 2019 Objective: We conducted this study to test the hypothesis that plasma zonulin levels are elevated in pediatric patients with nephrotic syndrome compared to healthy controls. Study Design: Plasma zonulin levels were measured by ELISA in 114 children enrolle ... Full text Link to item Cite

APOL1-G0 protects podocytes in a mouse model of HIV-associated nephropathy.

Journal Article PLoS One · 2019 African polymorphisms in the gene for Apolipoprotein L1 (APOL1) confer a survival advantage against lethal trypanosomiasis but also an increased risk for several chronic kidney diseases (CKD) including HIV-associated nephropathy (HIVAN). APOL1 is expressed ... Full text Open Access Link to item Cite

OPMI: The ontology of precision medicine and investigation and its support for clinical data and metadata representation and analysis

Conference CEUR Workshop Proceedings · January 1, 2019 Consortia conducting precision medicine studies face a major challenge of integrating big data including clinical and biomedical data. In this study, we report our development of the community-driven Ontology of Precision Medicine and Investigation (OPMI) ... Cite

Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study.

Journal Article Kidney Int Rep · November 2018 INTRODUCTION: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), in ... Full text Link to item Cite

An eQTL Landscape of Kidney Tissue in Human Nephrotic Syndrome.

Journal Article Am J Hum Genet · August 2, 2018 Expression quantitative trait loci (eQTL) studies illuminate the genetics of gene expression and, in disease research, can be particularly illuminating when using the tissues directly impacted by the condition. In nephrology, there is a paucity of eQTL stu ... Full text Link to item Cite

Reproducibility and Feasibility of Strategies for Morphologic Assessment of Renal Biopsies Using the Nephrotic Syndrome Study Network Digital Pathology Scoring System.

Journal Article Arch Pathol Lab Med · May 2018 Context Testing reproducibility is critical for the development of methodologies for morphologic assessment. Our previous study using the descriptor-based Nephrotic Syndrome Study Network Digital Pathology Scoring System (NDPSS) on glomerular images reveal ... Full text Link to item Cite

Global glomerulosclerosis with nephrotic syndrome; the clinical importance of age adjustment.

Journal Article Kidney Int · May 2018 Globally sclerotic glomeruli (GSG) occur with both normal aging and kidney disease. However, it is unknown whether any GSG or only GSG exceeding that expected for age is clinically important. To evaluate this, we identified patients with a glomerulopathy t ... Full text Link to item Cite

Migalastat improves diarrhea in patients with Fabry disease: clinical-biomarker correlations from the phase 3 FACETS trial.

Journal Article Orphanet J Rare Dis · April 27, 2018 BACKGROUND: Fabry disease is frequently characterized by gastrointestinal symptoms, including diarrhea. Migalastat is an orally-administered small molecule approved to treat the symptoms of Fabry disease in patients with amenable mutations. METHODS: We eva ... Full text Link to item Cite

Interstitial fibrosis scored on whole-slide digital imaging of kidney biopsies is a predictor of outcome in proteinuric glomerulopathies.

Journal Article Nephrol Dial Transplant · February 1, 2018 BACKGROUND: Interstitial fibrosis (IF), tubular atrophy (TA) and interstitial inflammation (II) are known determinants of progression of renal disease. Standardized quantification of these features could add value to current classification of glomerulopath ... Full text Link to item Cite

Digital pathology in nephrology clinical trials, research, and pathology practice.

Journal Article Curr Opin Nephrol Hypertens · November 2017 PURPOSE OF REVIEW: In this review, we will discuss (i) how the recent advancements in digital technology and computational engineering are currently applied to nephropathology in the setting of clinical research, trials, and practice; (ii) the benefits of ... Full text Link to item Cite

APOL1 variants change C-terminal conformational dynamics and binding to SNARE protein VAMP8.

Journal Article JCI insight · July 2017 APOL1 variants in African populations mediate resistance to trypanosomal infection but increase risk for kidney diseases through unknown mechanisms. APOL1 is expressed in glomerular podocytes and does not vary with underlying kidney disease diagnoses or AP ... Full text Open Access Cite

Digital pathology imaging as a novel platform for standardization and globalization of quantitative nephropathology.

Journal Article Clin Kidney J · April 2017 The introduction of digital pathology to nephrology provides a platform for the development of new methodologies and protocols for visual, morphometric and computer-aided assessment of renal biopsies. Application of digital imaging to pathology made substa ... Full text Link to item Cite

ABNORMAL GLOBAL GLOMERULOSCLEROSIS IN NEPHROTIC SYNDROME

Conference AMERICAN JOURNAL OF KIDNEY DISEASES · April 1, 2017 Link to item Cite

APOL1-G0 or APOL1-G2 Transgenic Models Develop Preeclampsia but Not Kidney Disease.

Journal Article J Am Soc Nephrol · December 2016 APOL1 risk variants are associated with kidney disease in blacks, but the mechanisms of renal injury associated with APOL1 risk variants are unknown. Because APOL1 is unique to humans and some primates, we created transgenic (Tg) mice using the promoter of ... Full text Open Access Link to item Cite

A Rare Case of Hepatitis C-Associated Cryoglobulinemic Duodenal Vasculitis.

Journal Article ACG Case Rep J · August 2016 Intestinal involvement of cryoglobulinemia is an uncommon manifestation and marker of severe vasculitis. We describe the case of a woman admitted to our service for management of acute renal failure and progressive gastrointestinal symptoms after initiatin ... Full text Link to item Cite

Reproducibility of the NEPTUNE descriptor-based scoring system on whole-slide images and histologic and ultrastructural digital images.

Journal Article Mod Pathol · July 2016 The multicenter Nephrotic Syndrome Study Network (NEPTUNE) digital pathology scoring system employs a novel and comprehensive methodology to document pathologic features from whole-slide images, immunofluorescence and ultrastructural digital images. To est ... Full text Link to item Cite

STANDARDIZED ASSESSMENT OF DIGITAL RENAL BIOPSY WHOLE SLIDE IMAGES

Conference NEPHROLOGY DIALYSIS TRANSPLANTATION · May 1, 2016 Link to item Cite

Integrative Genomics Identifies Novel Associations with APOL1 Risk Genotypes in Black NEPTUNE Subjects.

Journal Article J Am Soc Nephrol · March 2016 APOL1 variants have been associated with renal phenotypes in blacks. To refine clinical outcomes and discover mechanisms of APOL1-associated kidney injury, we analyzed clinical and genomic datasets derived from 90 black subjects in the Nephrotic Syndrome S ... Full text Link to item Cite

Complete Remission in the Nephrotic Syndrome Study Network.

Journal Article Clin J Am Soc Nephrol · January 7, 2016 BACKGROUND AND OBJECTIVES: This analysis from the Nephrotic Syndrome Study Network (NEPTUNE) assessed the phenotypic and pathology characteristics of proteinuric patients undergoing kidney biopsy and defined the frequency and factors associated with comple ... Full text Link to item Cite

Morphometry Predicts Early GFR Change in Primary Proteinuric Glomerulopathies: A Longitudinal Cohort Study Using Generalized Estimating Equations.

Journal Article PLoS One · 2016 OBJECTIVE: Most predictive models of kidney disease progression have not incorporated structural data. If structural variables have been used in models, they have generally been only semi-quantitative. METHODS: We examined the predictive utility of quantit ... Full text Link to item Cite

The Application of Digital Pathology to Improve Accuracy in Glomerular Enumeration in Renal Biopsies.

Journal Article PLoS One · 2016 BACKGROUND: In renal biopsy reporting, quantitative measurements, such as glomerular number and percentage of globally sclerotic glomeruli, is central to diagnostic accuracy and prognosis. The aim of this study is to determine the number of glomeruli and p ... Full text Link to item Cite

Tissue transcriptome-driven identification of epidermal growth factor as a chronic kidney disease biomarker.

Journal Article Sci Transl Med · December 2, 2015 Chronic kidney disease (CKD) affects 8 to 16% people worldwide, with an increasing incidence and prevalence of end-stage kidney disease (ESKD). The effective management of CKD is confounded by the inability to identify patients at high risk of progression ... Full text Link to item Cite

Morphology in the Digital Age: Integrating High-Resolution Description of Structural Alterations With Phenotypes and Genotypes.

Journal Article Semin Nephrol · May 2015 Conventional light microscopy has been used to characterize and classify renal diseases, evaluate histopathology in studies and trials, and educate renal pathologists and nephrologists. The advent of digital pathology, in which a glass slide can be scanned ... Full text Link to item Cite

Inactivation of integrin-β1 prevents the development of polycystic kidney disease after the loss of polycystin-1.

Journal Article Journal of the American Society of Nephrology : JASN · April 2015 Dysregulation of polycystin-1 (PC1) leads to autosomal dominant polycystic kidney disease (ADPKD), a disorder characterized by the formation of multiple bilateral renal cysts, the progressive accumulation of extracellular matrix (ECM), and the development ... Full text Cite

MetaboLic Glomerular Injury

Chapter · January 1, 2014 MetaboLic diseases are as a result of acquired or inborn defects of enzymes or hormones. They can affect the carbohydrate, protein, or Lipid metaboLism. The most common form of metaboLic disease in humans is diabetes melLitus (DM) manifesting primarily hyp ... Full text Cite

Diabetic nephropathy: Is it time yet for routine kidney biopsy?

Journal Article World journal of diabetes · December 2013 Diabetic nephropathy (DN) is one of the most important long-term complications of diabetes. Patients with diabetes and chronic kidney disease have an increased risk of all-cause mortality, cardiovascular mortality, and kidney failure. The clinical diagnosi ... Full text Open Access Cite

Digital pathology evaluation in the multicenter Nephrotic Syndrome Study Network (NEPTUNE).

Journal Article Clin J Am Soc Nephrol · August 2013 Pathology consensus review for clinical trials and disease classification has historically been performed by manual light microscopy with sequential section review by study pathologists, or multi-headed microscope review. Limitations of this approach inclu ... Full text Link to item Cite

Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations.

Journal Article Lupus · July 2013 Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catas ... Full text Open Access Cite

Design of the Nephrotic Syndrome Study Network (NEPTUNE) to evaluate primary glomerular nephropathy by a multidisciplinary approach.

Journal Article Kidney Int · April 2013 The Nephrotic Syndrome Study Network (NEPTUNE) is a North American multicenter collaborative consortium established to develop a translational research infrastructure for nephrotic syndrome. This includes a longitudinal observational cohort study, a pilot ... Full text Link to item Cite

TRPC6 enhances angiotensin II-induced albuminuria (vol 22, pg 526, 2011)

Journal Article JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · January 1, 2013 Link to item Cite

Safety and pharmacodynamic effects of a pharmacological chaperone on α-galactosidase A activity and globotriaosylceramide clearance in Fabry disease: report from two phase 2 clinical studies.

Journal Article Orphanet J Rare Dis · November 24, 2012 BACKGROUND: Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme α-galactosidase A (α-Gal A), which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues. We report on the safety and pharmacodynamics o ... Full text Link to item Cite

Novel quantitative method to evaluate globotriaosylceramide inclusions in renal peritubular capillaries by virtual microscopy in patients with fabry disease.

Journal Article Arch Pathol Lab Med · July 2012 CONTEXT: Assessing the amount of globotriaosylceramide inclusions in renal peritubular capillaries by a semiquantitative approach is a standard and useful measure of therapeutic efficacy in Fabry disease, achievable by light microscopy analysis. OBJECTIVE: ... Full text Link to item Cite

Collapsing glomerulopathy in 19 patients with systemic lupus erythematosus or lupus-like disease.

Journal Article Clin J Am Soc Nephrol · June 2012 BACKGROUND AND OBJECTIVES: Collapsing glomerulopathy is a podocytopathy with segmental or global wrinkling and collapse of capillary walls and overlying epithelial cell proliferation. Idiopathic collapsing glomerulopathy is a distinct clinicopathologic ent ... Full text Link to item Cite

Coexistence of ANCA-associated glomerulonephritis and anti-phospholipase A2 receptor antibody-positive membranous nephropathy

Journal Article Clinical Kidney Journal · April 1, 2012 Antibodies to myeloperoxidase (MPO) and proteinase 3 (PR3) have been demonstrated to mediate anti-neutrophil cytoplasmic antibody (ANCA)-associated disease. For membranous nephropathy, antibodies to the podocyte-expressed phospholipase A2 receptor (anti-PL ... Full text Cite

Coexistence of ANCA-associated glomerulonephritis and anti-phospholipase A(2) receptor antibody-positive membranous nephropathy.

Journal Article Clin Kidney J · April 2012 Antibodies to myeloperoxidase (MPO) and proteinase 3 (PR3) have been demonstrated to mediate anti-neutrophil cytoplasmic antibody (ANCA)-associated disease. For membranous nephropathy, antibodies to the podocyte-expressed phospholipase A(2) receptor (anti- ... Full text Link to item Cite

Glomerular grievances and CKD.

Journal Article Advances in chronic kidney disease · March 2012 Full text Cite

Podocyte biology in segmental sclerosis and progressive glomerular injury.

Journal Article Adv Chronic Kidney Dis · March 2012 During the past 2 decades, progress has been made in understanding the biology and mechanisms of podocyte injury and the relationship of these processes to glomerulosclerosis. In addition, studies of human biopsies and animal models have provided insights ... Full text Link to item Cite

Dysregulation of the microvasculature in nonlesional non-sun-exposed skin of patients with lupus nephritis.

Journal Article The Journal of rheumatology · March 2012 ObjectiveMembrane endothelial protein C receptor (mEPCR) is highly expressed in peritubular capillaries of kidneys from patients with active and poorly responsive lupus nephritis (LN). We investigated the hypothesis that changes in the microvascul ... Full text Cite

The inducible deletion of Drosha and microRNAs in mature podocytes results in a collapsing glomerulopathy.

Journal Article Kidney Int · October 2011 Micro-RNAs (miRNAs) are short (average 22 nucleotides) noncoding regulatory RNAs that inhibit gene expression by targeting complementary 3'-untranslated regions of protein-encoding mRNAs for translational repression or degradation. miRNAs play key roles in ... Full text Link to item Cite

Quantitative evaluation of acute renal transplant dysfunction with low-dose three-dimensional MR renography.

Journal Article Radiology · September 2011 PURPOSE: To assess prospectively the ability of quantitative low-dose three-dimensional magnetic resonance (MR) renography to help identify the cause of acute graft dysfunction. MATERIALS AND METHODS: This HIPAA-compliant study was approved by the institut ... Full text Link to item Cite

Wnt/β-catenin pathway in podocytes integrates cell adhesion, differentiation, and survival.

Journal Article J Biol Chem · July 22, 2011 Diabetic kidney disease (DKD) is the single most common cause of albuminuria and end-stage kidney disease in the United States. We found increased expression of Wnt/β-catenin (Ctnnb1) pathway transcripts and proteins in glomeruli and podocytes of patients ... Full text Link to item Cite

TNFR2 interposes the proliferative and NF-κB-mediated inflammatory response by podocytes to TNF-α.

Journal Article Lab Invest · March 2011 The development of proliferative podocytopathies has been linked to ligation of tumor necrosis factor receptor 2 (TNFR2) expressed on the renal parenchyma; however, the TNFR2-positive cells within the kidney responsible for podocyte injury are unknown. We ... Full text Link to item Cite

TRPC6 enhances angiotensin II-induced albuminuria.

Journal Article J Am Soc Nephrol · March 2011 Mutations in the canonical transient receptor potential cation channel 6 (TRPC6) are responsible for familial forms of adult onset focal segmental glomerulosclerosis (FSGS). The mechanisms by which TRPC6 mutations cause kidney disease are not well understo ... Full text Link to item Cite

Expression of endothelial protein C receptor in cortical peritubular capillaries associates with a poor clinical response in lupus nephritis.

Journal Article Rheumatology (Oxford) · May 2009 OBJECTIVE: To study the membrane expression of endothelial protein C receptor (mEPCR) in the renal microvasculature in lupus nephritis (LN) as a potential marker of injury and/or prognostic indicator for response to therapy. METHODS: mEPCR expression was a ... Full text Link to item Cite

Glomerular type 1 angiotensin receptors augment kidney injury and inflammation in murine autoimmune nephritis.

Journal Article J Clin Invest · April 2009 Studies in humans and animal models indicate a key contribution of angiotensin II to the pathogenesis of glomerular diseases. To examine the role of type 1 angiotensin (AT1) receptors in glomerular inflammation associated with autoimmune disease, we genera ... Full text Link to item Cite

Current therapies for lupus nephritis in an ethnically heterogeneous cohort.

Journal Article The Journal of rheumatology · February 2009 ObjectiveTo evaluate responses to mycophenolate mofetil (MMF) and intravenous cyclophosphamide (CYC) in lupus nephritis in a multiethnic population.MethodsThis was a retrospective study of all patients with systemic lupus erythematosus (S ... Full text Cite

Advances in the biology and genetics of the podocytopathies: implications for diagnosis and therapy.

Journal Article Archives of pathology & laboratory medicine · February 2009 ContextEtiologic factors and pathways leading to altered podocyte phenotype are clearly numerous and involve the activity of different cellular function.ObjectiveTo focus on recent discoveries in podocyte biology and genetics and their re ... Full text Cite

Cogan's syndrome and development of ANCA-associated renal vasculitis after lengthy disease remission.

Journal Article Clinical and experimental rheumatology · January 2009 Cite

VEGF inhibition and renal thrombotic microangiopathy [13]

Journal Article New England Journal of Medicine · July 10, 2008 Cite

VEGF inhibition and renal thrombotic microangiopathy.

Journal Article N Engl J Med · July 10, 2008 Link to item Cite

Current views on collapsing glomerulopathy.

Journal Article J Am Soc Nephrol · July 2008 Collapsing glomerulopathy is a proliferative disease defined by segmental or global wrinkling of the glomerular basement membranes associated with podocyte proliferation. These lesions are particularly poor responders to standard therapies. First described ... Full text Link to item Cite

Notch signaling: a common pathway of injury in podocytopathies?

Journal Article Journal of the American Society of Nephrology : JASN · June 2008 Full text Cite

VEGF inhibition and renal thrombotic microangiopathy.

Journal Article N Engl J Med · March 13, 2008 The glomerular microvasculature is particularly susceptible to injury in thrombotic microangiopathy, but the mechanisms by which this occurs are unclear. We report the cases of six patients who were treated with bevacizumab, a humanized monoclonal antibody ... Full text Link to item Cite

Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease.

Journal Article Am J Surg Pathol · October 2007 Autoimmune pancreatitis (AIP) is a mass-forming chronic fibroinflammatory condition centered on the pancreatobiliary system and characterized by predominant immunoglobulin G4 (IgG4)-positive plasma cells. Recent reports have brought to light the multiorgan ... Full text Link to item Cite

COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement.

Journal Article J Am Soc Nephrol · October 2007 Primary coenzyme Q(10) (CoQ(10)) deficiency includes a group of rare autosomal recessive disorders primarily characterized by neurological and muscular symptoms. Rarely, glomerular involvement has been reported. The COQ2 gene encodes the para-hydroxybenzoa ... Full text Link to item Cite

CD20-positive infiltrates in renal allograft biopsies with acute cellular rejection are not associated with worse graft survival.

Journal Article Am J Transplant · August 2007 We examined rejection outcome and graft survival in 58 adult patients with acute cellular rejection Banff type I (ARI) or II (ARII), within 1 year after transplantation, with or without CD20-positive infiltrates. Antibody-mediated rejection was not examine ... Full text Link to item Cite

A proposed taxonomy for the podocytopathies: a reassessment of the primary nephrotic diseases.

Journal Article Clin J Am Soc Nephrol · May 2007 A spectrum of proteinuric glomerular diseases results from podocyte abnormalities. The understanding of these podocytopathies has greatly expanded in recent years, particularly with the discovery of more than a dozen genetic mutations that are associated w ... Full text Link to item Cite

Collapsing glomerulopathy: an inflammatory podocytopathy?

Journal Article Curr Opin Nephrol Hypertens · May 2007 PURPOSE OF REVIEW: Collapsing glomerulopathy is a relatively new and debated podocytopathy. Among several conjectures, inflammatory injury orchestrated by podocytes is emerging to explain the pathogenesis of collapsing glomerulopathy. Here, we briefly summ ... Full text Link to item Cite

Acute renal failure after initiation of tenofovir disoproxil fumarate.

Journal Article Ren Fail · 2007 Despite initial evidence suggesting a relatively benign safety profile, several subsequent case reports have detailed nephrotoxicity in patients using tenofovir disoproxil fumarate for the treatment of HIV. We report a case of rapid renal failure that deve ... Full text Link to item Cite

Collapsing glomerulopathy.

Journal Article Journal of the American Society of Nephrology : JASN · October 2006 Collapsing glomerulopathy (CG) has become an important cause of ESRD. First delineated from other proteinuric glomerular lesions in the 1980s, CG is now recognized as a common, distinct pattern of proliferative parenchymal injury that portends a rapid loss ... Full text Cite

CX3CR1+ interstitial dendritic cells form a contiguous network throughout the entire kidney.

Journal Article Kidney international · August 2006 Dendritic cells (DCs) interface innate and adaptive immunity in nonlymphoid organs; however, the exact distribution and types of DC within the kidney are not known. We utilized CX3CR1GFP/+ mice to characterize the anatomy and phenotype of tissue-resident C ... Full text Cite

Introduction to Renal Biopsy

Chapter · January 1, 2006 Full text Cite

The kd/kd mouse is a model of collapsing glomerulopathy.

Journal Article J Am Soc Nephrol · October 2005 Collapsing glomerulopathy (CG) is associated with disorders that markedly perturb the phenotype of podocytes. The kd/kd mouse has been studied for immune and genetic causes of microcystic tubulointerstitial nephritis with little attention to its glomerular ... Full text Link to item Cite

Proliferative lupus nephritis and leukocytoclastic vasculitis during treatment with etanercept.

Journal Article J Rheumatol · April 2005 Tumor necrosis factor-alpha (TNF-alpha) is a proinflammatory cytokine. Agents that neutralize TNF-alpha are effective in the treatment of disorders such as rheumatoid arthritis, juvenile rheumatoid arthritis (JRA), spondyloarthropathies, and inflammatory b ... Link to item Cite

NIH/NYU Podocyte Disease Registry

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · November 1, 2003 Link to item Cite

Pathogenesis and treatment of HIV-associated renal diseases: lessons from clinical and animal studies, molecular pathologic correlations, and genetic investigations.

Conference Ann Intern Med · August 5, 2003 HIV infection is associated with several renal syndromes, including acute renal failure. Chronic renal failure directly linked to HIV infection includes thrombotic microangiopathic renal diseases, immune-mediated glomerulonephritides, and HIV-associated ne ... Link to item Cite

Update in podocyte biology: putting one's best foot forward.

Journal Article Current opinion in nephrology and hypertension · May 2003 Purpose of reviewThe rapidly developing field of podocyte cell biology is reviewed, focusing on papers published in the last 12 months.Recent findingsFour areas of particular progress can be discerned. First, podocytes proliferate during ... Full text Cite

Podocyte biology and the emerging understanding of podocyte diseases.

Journal Article Am J Nephrol · 2003 The understanding of the unique molecular apparatus of the podocyte has increased dramatically in recent years. This new knowledge has improved the diagnosis and classification of the diseases that have been termed podocytopathies. Podocyte injury frequent ... Full text Link to item Cite

Anti-mouse mesangial cell serum induces acute glomerulonephropathy in mice.

Journal Article Nephron. Experimental nephrology · January 2003 In order to develop a model in mouse similar to anti- Thy-1 nephritis in the rat, we prepared sheep antiserum against SV40-transformed mouse mesangial (MES 13) cells. In vivo, the anti-mouse mesangial cell serum-treated mice showed severe azotemia that pea ... Full text Cite

Laser-capture microdissection.

Journal Article Methods in molecular medicine · January 2003 Full text Cite

The changing epidemiology of focal segmental glomerulosclerosis (FSGS): A 28-year single center study.

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 2002 Link to item Cite

Morphologic analysis of podocyte diseases: Role of activity and chronicity indices.

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 2002 Link to item Cite

Modulation of podocyte phenotype in collapsing glomerulopathies.

Journal Article Microsc Res Tech · May 15, 2002 Podocytes are well-differentiated postmitotic cells whose function is largely based on their complex cytoskeletal architecture. In diseases with proteinuria, podocytes undergo morphologic changes. Podocytes react to an injurious stimulus by a reorganizatio ... Full text Link to item Cite

Mice lacking the p53-effector gene Gadd45a develop a lupus-like syndrome.

Journal Article Immunity · April 2002 This study addresses the biological function of the p53-effector genes Gadd45a and p21 in the immune system. We find that Gadd45a is a negative regulator of T cell proliferation because, compared to wild-type cells, Gadd45a(-/-) T cells have a lower thresh ... Full text Link to item Cite

Differentiation- and stress-dependent nuclear cytoplasmic redistribution of myopodin, a novel actin-bundling protein

Journal Article Journal of Cell Biology · November 12, 2001 We report the cloning and functional characterization of myopodin, the second member of the synaptopodin gene family. Myopodin shows no significant homology to any known protein except synaptopodin. Northern blot analysis resulted in a 3.6-kb transcript fo ... Cite

Differentiation- and stress-dependent nuclear cytoplasmic redistribution of myopodin, a novel actin-bundling protein.

Journal Article J Cell Biol · October 29, 2001 We report the cloning and functional characterization of myopodin, the second member of the synaptopodin gene family. Myopodin shows no significant homology to any known protein except synaptopodin. Northern blot analysis resulted in a 3.6-kb transcript fo ... Full text Link to item Cite

Podocyte cell cycle regulation and proliferation in collapsing glomerulopathies.

Journal Article Kidney Int · July 2000 BACKGROUND: Mature podocytes are growth-arrested because of the expression of cyclin-dependent kinase inhibitors. Under pathological conditions, podocytes may undergo mitosis, but not cell division. Exceptions to this rule are collapsing glomerulopathies ( ... Full text Link to item Cite

HIV-1 induces renal epithelial dedifferentiation in a transgenic model of HIV-associated nephropathy.

Journal Article Kidney Int · July 2000 BACKGROUND: Human immunodeficiency virus-associated nephropathy (HIVAN) is the most common cause of renal failure in HIV-1-seropositive patients. Recent studies using an HIV-1 transgenic mouse model have demonstrated that expression of HIV-1 in the kidney ... Full text Link to item Cite

The dysregulated podocyte phenotype: a novel concept in the pathogenesis of collapsing idiopathic focal segmental glomerulosclerosis and HIV-associated nephropathy.

Journal Article J Am Soc Nephrol · January 1999 Podocytes are highly differentiated, postmitotic cells, whose function is largely based on their complex cytoarchitecture. The differentiation of podocytes coincides with progressive expression of maturity markers, including WT-1, CALLA, C3b receptor, GLEP ... Full text Link to item Cite

Expression of sarcomeric Z-band associated proteins in heart failure

Conference LABORATORY INVESTIGATION · January 1, 1999 Link to item Cite

Polycystic kidney disease in SBM transgenic mice: role of c-myc in disease induction and progression.

Journal Article The American journal of pathology · January 1998 SBM mouse is a unique transgenic model of polycystic kidney disease (PKD) produced by dysregulation of c-myc in the kidneys. Our previous demonstration that c-myc is overexpressed in human autosomal polycystic kidney disease (ADPKD) prompted us to investig ... Cite

C-myc-induced apoptosis in polycystic kidney disease is Bcl-2 and p53 independent.

Journal Article J Exp Med · December 1, 1997 The SBM mouse is a unique transgenic model of polycystic kidney disease (PKD) induced by the dysregulated expression of c-myc in renal tissue. In situ hybridization analysis demonstrated intense signal for the c-myc transgene overlying tubular cystic epith ... Full text Link to item Cite

Podocyte (P) dedifferentiation predicts glomerulosclerosis (GS) in nephrotic syndrome (NS).

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1997 Link to item Cite

Pathogenesis of HIV-associated nephropathy (HIV-AN) in transgenic mice.

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1997 Link to item Cite

Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study.

Journal Article Kidney Int · November 1996 A review of all native kidney biopsies at our center from 1974 to 1993 identified 43 cases of idiopathic focal segmental glomerulosclerosis (FSGS) with predominantly collapsing features and lacking evidence of HIV-1 infection or intravenous drug use. No ca ... Full text Link to item Cite

Renal disease in anti-phospholipid antibody syndrome (APLS).

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1996 Link to item Cite

Analysis of the role of membrane polarity in polycystic kidney disease of transgenic SBM mice.

Journal Article Am J Pathol · December 1995 Altered membrane polarity has been proposed as an important pathogenetic factor in the development of renal cysts in polycystic kidney disease. To determine whether this alteration in epithelial phenotype is a primary or secondary phenomenon, we examined t ... Link to item Cite

FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS) - A 20-YEAR EPIDEMIOLOGIC PERSPECTIVE

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1994 Link to item Cite

NEPHROTIC PROTEINURIA (NP) IN HYPERTENSIVE NEPHROSCLEROSIS

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1994 Link to item Cite

[Fine needle aspiration of pancreatic masses. A study of 81 cases].

Journal Article Pathologica · November 1990 Study of 81 cases. Fine Needle Aspiration Biopsy (FNAB) was performed under computerized thomography scan (CT) in 81 patients with documented pancreatic mass (54 with neoplasia and 27 with chronic pancreatitis). This procedure, when associated to the immed ... Cite