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Priya Sunil Kishnani

Chen Family Distinguished Professor of Pediatrics
Pediatrics, Medical Genetics
Box 103856, DUMC, Durham, NC 27710
905 LaSalle Street, GSRB1, 4th Floor, Room 4010, Durham, NC 27710

Selected Grants


A GLOBAL PROSPECTIVE OBSERVATIONAL REGISTRY OF PATIENTS WITH POMPE DISEASE

Clinical TrialPrincipal Investigator · Awarded by Amicus Therapeutics, Inc. · 2024 - 2033

Unified Program for Therapeutics in Children

Inst. Training Prgm or CMEPreceptor · Awarded by National Institutes of Health · 2025 - 2030

Understanding cognitive and neurological pathologies in infantile Pompe disease CNS

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2022 - 2030

Phase 3 Efficacy and Safety of ALXN1850

Clinical TrialPrincipal Investigator · Awarded by Alexion Pharmaceuticals, Inc. · 2024 - 2027

Investigating the Central Nervous System Involvement in Pompe Disease to Facilitate Targeted Treatment Strategies and Improve Patient Management

ResearchPrincipal Investigator · Awarded by Acid Maltase Deficiency Association · 2025 - 2027

Medical Scientist Training Program

Inst. Training Prgm or CMEPreceptor · Awarded by National Institute of General Medical Sciences · 2022 - 2027

A Phase 3, Randomized, Double-blinded, Placebo-controlled, Multicenter Study to Evaluate Efficacy and Safety of ALXN1850

Clinical TrialPrincipal Investigator · Awarded by Alexion Pharmaceuticals, Inc. · 2024 - 2027

Gene therapy for glycogen storage disease type III

ResearchCo Investigator · Awarded by National Institute of Arthritis and Musculoskeletal and Skin Diseases · 2022 - 2027

Assessment of GYS1 knockdown on Pompe disease

ResearchCo Investigator · Awarded by Alnylam U.S., Inc. · 2025 - 2026

Clinical Pharmacokinetics and Safety Trials in Down Syndrome

Clinical TrialCo Investigator · Awarded by Eunice Kennedy Shriver National Institute of Child Health and Human Development · 2019 - 2026

Research Training in Allergy and Clinical Immunology

Inst. Training Prgm or CMEMentor · Awarded by University of North Carolina - Chapel Hill · 2000 - 2026

GYS2 knockdown on liver disease in hepatic GSD

ResearchCo Investigator · Awarded by Alnylam Pharmaceuticals · 2025 - 2026

LSD Registry

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2019 - 2026

Phase 1 Study of In Utero Enzyme Replacement Therapy for the Treatment of Lysosomal Storage Diseases

ResearchCo Investigator · Awarded by University of California - San Francisco · 2022 - 2026

Factors in Immune Response Affecting Long-term Treatment Outcomes in Pompe disease (CRIM)

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2022 - 2025

Amicus ATB200+-04

Clinical TrialPrincipal Investigator · Awarded by Amicus Therapeutics Corporation · 2019 - 2025

Down Syndrome Clinical Trials Network

ResearchPrincipal Investigator · Awarded by Lumind RDS Foundation · 2018 - 2025

An observational, longitudinal prospective, long-term registry of patients with hypophosphatasia

ResearchPrincipal Investigator · Awarded by Alexion Pharmaceuticals, Inc. · 2015 - 2025

Long Term Follow-up and Treatment Outcomes for Individuals with Pompe Disease

ResearchPrincipal Investigator · Awarded by University of Minnesota · 2023 - 2025

The Duke FUNCTION Center: Pioneering the comprehensive identification of combinatorial noncoding causes of disease

ResearchCo Investigator · Awarded by National Human Genome Research Institute · 2020 - 2025

GSD Ib Natural History Study

ResearchPrincipal Investigator · Awarded by Sophie's Hope Foundation · 2023 - 2025

Biomarker studies in plasma from patients with Gaucher disease

ResearchCo Investigator · Awarded by Shire Human Genetics Therapies · 2018 - 2025

Residency/Fellowship Training in Medical Genetics

FellowshipCo-Mentor · Awarded by Genzyme Corporation · 2022 - 2024

Duke CTSA (TL1) Year 5

Inst. Training Prgm or CMEMentor · Awarded by National Institutes of Health · 2018 - 2024

2024 Pompe Patient Meetings

Inst. Training Prgm or CMEPrincipal Investigator · Awarded by Orsini Speciality Pharmacy · 2024 - 2024

Untargeted transcriptomics of Phkg2-/- liver and plasma (the "Project")

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2022 - 2024

Residency in Medical Genetics at Duke University

ResearchCo-Mentor · Awarded by Genzyme Corporation · 2021 - 2024

Transfer of livers from WT mice in Study 1: MED Investigation

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2023 - 2024

Defining the clinical characteristics for clinical trial endpoints of GSD VI and IX

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2022 - 2024

CHARACTERIZATION OF GSD IX ¿2 and GSD VI MOUSE MODELS

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2022 - 2024

AAV Gene Therapy for the Treatment of GSD IX y2: Determination of Titer of Duke AAVs using Kriya Titering Methodologies

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2022 - 2024

AAV gene therapy for the treatment of Glycogen Storage Disease IX y2

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2022 - 2024

Targeted Therapy for Pompe Disease

ResearchCo Investigator · Awarded by National Institutes of Health · 2019 - 2024

Liver-directed AAV gene therapy for PHKG2-Glycogen Storage Disease IX (GSD IX y2)

FellowshipPrincipal Investigator · Awarded by National Institutes of Health · 2021 - 2024

Immunophenotyping of infantile Pompe disease

ResearchPrincipal Investigator · Awarded by Genzyme Corporation · 2019 - 2024

Transfer of AAV-hPHKG2 Materials for Bridging Study

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2023 - 2024

AAV-mPhkg2 Gene Therapy for the treatment of GSD IXy2

ResearchPrincipal Investigator · Awarded by Kriya Therapeutics · 2022 - 2023

Gene therapy by Lipid Nanoparticle-Mediated Delivery of Agl Messenger RNA in GSD III dogs

ResearchPrincipal Investigator · Awarded by Ultragenyx Pharmaceutical · 2017 - 2023

Sanofi NeoGAA

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2013 - 2023

Postdoctoral Training in Genomic Medicine Research

Inst. Training Prgm or CMECo-Mentor · Awarded by National Institutes of Health · 2017 - 2023

Immunophenotyping of patients with MPS II treated with enzyme replacement therapy

Clinical TrialPrincipal Investigator · Awarded by Takeda Pharmaceuticals U.S.A., Inc · 2022 - 2023

Optimized GAA (GAA) as potentially improved therapeutic for the treatment of Pompe disease

ResearchCo Investigator · Awarded by Codexis, Inc. · 2020 - 2022

Medical Scientist Training Program

Inst. Training Prgm or CMEMentor · Awarded by National Institutes of Health · 1997 - 2022

Identifying Pathogenic Non-Coding Mutations in Rare Mendelian Disease

ResearchCo-Principal Investigator · Awarded by National Institutes of Health · 2019 - 2022

Developing a management approach for patients with the "late-onset" Pompe disease GAA variant identified by newborn screening

ResearchPrincipal Investigator · Awarded by Amicus Therapeutics Corporation · 2018 - 2022

Serologic and genomic modifiers of beta-amyloid levels in an immuno-compromised cohort of adult Down syndrome patients

ResearchCo Investigator · Awarded by Infectious Diseases Society of America · 2021 - 2022

Understanding cognitive and neurological pathologies in infantile Pompe disease CNS

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2019 - 2021

Factors in Immune Response Affecting Long-term Treatment Outcomes in Pompe disease (CRIM)

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2019 - 2021

Long Term Follow-up and Treatment Outcomes for Individuals with Pompe Disease

ResearchPrincipal Investigator · Awarded by University of Minnesota · 2019 - 2021

GSD III Clinical Data Manuscript Preparation

ResearchPrincipal Investigator · Awarded by Ultragenyx Pharmaceutical · 2019 - 2021

Morquio A Registry Study

Clinical TrialPrincipal Investigator · Awarded by BioMarin Pharmaceutical, Inc. · 2017 - 2021

A study of patient -reported health masures in adult patients with Pompe disease

Clinical TrialPrincipal Investigator · Awarded by Amicus Therapeutics Corporation · 2017 - 2020

Pompe Developmental IPAD Study

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2015 - 2020

Feasibility of using Bortezomib-based immunosuppressive approach to deplete anti-AAV antibodies in mice

ResearchCo Investigator · Awarded by Asklepios BioPharmaceutical, Inc. · 2018 - 2020

A longitudinal retrospective chart review of adults with HPP treated with asfotase alfa

Clinical TrialPrincipal Investigator · Awarded by Alexion Pharmaceuticals, Inc. · 2017 - 2020

A Phase I Study of the Safety of AAV2/8 LSPhGAA in Late-onset Pompe Disease

Clinical TrialCo Investigator · Awarded by National Institutes of Health · 2017 - 2020

GAA-iTEM: Personalizing the Prediction of Anti-therapeutic Antibody Response in Pome Disease Patients

ResearchPrincipal Investigator · Awarded by EpiVax, Inc. · 2018 - 2020

LDN: CRIM responses in Pompe disease

ResearchPrincipal Investigator · Awarded by University of Minnesota · 2009 - 2019

Respiratory Muscle Training in Individuals With Pompe Disease

ResearchCollaborating Investigator · Awarded by National Institutes of Health · 2016 - 2019

GSD IV Database Study

ResearchPrincipal Investigator · Awarded by Roivant Sciences, Inc. · 2015 - 2019

Novel Approaches for Correcting Respiratory Insufficiency in Pompe Disease

ResearchMentor · Awarded by National Institutes of Health · 2014 - 2019

Factors in Immune Response Affecting Long-term Treatment Outcomes in Pompe disease

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2016 - 2018

Understanding cognitive and neurological pathologies in infantile Pompe disease

Clinical TrialPrincipal Investigator · Awarded by Genzyme Corporation · 2016 - 2018

A Retrospective Characterization of Response to Enzyme Replacement Therapy in Late-onset Pompe Disease

Clinical TrialPrincipal Investigator · Awarded by Amicus Therapeutics Corporation · 2017 - 2018

Preclinical study of use of SVP-Rapamycin to induce immune tolerance to ERT in Pompe disease mice

ResearchCo Investigator · Awarded by Selecta Biosciences, Inc. · 2017 - 2018

Evaluation of the use of Myozyme for treatment of glycogen storage disease type III and IV in murine disease models

ResearchCo Investigator · Awarded by Roivant Sciences, Inc. · 2015 - 2018

Duke Research Training Program for Pediatricians

Inst. Training Prgm or CMETraining Faculty · Awarded by National Institutes of Health · 2002 - 2018

Alexion Adult HPP PK Study

Clinical TrialPrincipal Investigator · Awarded by Alexion Pharmaceuticals, Inc. · 2016 - 2018

Identification of candidate genes for Pompe disease phenotype modifier genes

FellowshipPrincipal Investigator · Awarded by University of Minnesota · 2014 - 2017

In vivo efficacy studies of Fab-GAA treatment for GSDII, III, and IV in murine disease models

ResearchCo Investigator · Awarded by Valerion Therapeutics, Inc · 2015 - 2017

To test the ability of SVP to prevent immune response in Pompe disease mouse model.

ResearchPrincipal Investigator · Awarded by Selecta Biosciences, Inc. · 2015 - 2016

Supplemental Funding for Phase 1/2 study of Clenbuterol for the Treatment of Pompe Disease

Clinical TrialCo Investigator · Awarded by Roivant Sciences, Inc. · 2015 - 2016

Long-Term registry of patients with urea Cycle Disorders (UCD) HPN-100-014

Clinical TrialPrincipal Investigator · Awarded by Hyperion Therapeutics, Inc. · 2014 - 2016

The Duke Multidisciplinary Training Program in Pediatric Lung Disease

Inst. Training Prgm or CMEFaculty Member · Awarded by National Institutes of Health · 2010 - 2016

Pfizer/ACMG Foundation Clinical Genetics Combined Residency for Translational Genomic Scholars 2015-2016 Fellowship Award

FellowshipPrincipal Investigator · Awarded by American College of Medical Genetics and Genomics Foundation · 2015 - 2016

Phase 1/2 Study of Clenbuterol for the Treatment of Pompe Disease

Clinical TrialCo Investigator · Awarded by Food and Drug Administration · 2013 - 2016

UNC-Duke Collaborative Clinical Pharmacology Postdoctoral Training Program

Inst. Training Prgm or CMEMentor · Awarded by University of North Carolina - Chapel Hill · 2011 - 2016

Phase 3 BMN 701 in rhGAA Exposed Subjects with Late-Onset Pompe Disease

Clinical TrialPrincipal Investigator · Awarded by BioMarin Pharmaceutical, Inc. · 2014 - 2016

Phase 2 Study of Efficacy, Safety and Tolerability of R05186582 in Adults with Adolescents with Down Syndrome (Clematis)

Clinical TrialPrincipal Investigator · Awarded by F. Hoffmann-La Roche Ltd · 2014 - 2016

Roche Phase 2 Screening Study

Clinical TrialPrincipal Investigator · Awarded by Hoffman-La Roche, Inc. · 2013 - 2015

Novel strategy for diagnosis of Pompe patients using next generation sequencing technologies

ResearchPrincipal Investigator · Awarded by Universite de Sherbrooke · 2014 - 2015

Institutional Training Grant in Pediatric Cardiology

Inst. Training Prgm or CMEFaculty Member · Awarded by National Institutes of Health · 2009 - 2015

Late Onset Pompe Disease Patient Meeting 2015

ConferencePrincipal Investigator · Awarded by BioMarin Pharmaceutical, Inc. · 2015 - 2015

A retrospective, non-interventional, epidemiologic study of the natural history of patients with juvenile-onset hypophosphatasia (HPP)

ResearchPrincipal Investigator · Awarded by Alexion Pharmaceuticals, Inc. · 2014 - 2015

Lysosomal Disease Network Fellowship

FellowshipMentor · Awarded by University of Minnesota · 2013 - 2014

Clinical Trial Planning in Pompe Disease

ResearchCo Investigator · Awarded by National Institutes of Health · 2013 - 2014

CTSA UL

ResearchInvestigator · Awarded by National Institutes of Health · 2006 - 2012

Mechanisms for immune tolerance in Pompe Disease

ResearchCo Investigator · Awarded by National Institutes of Health · 2009 - 2011

A Clinical Trial of Donepezil for Down Syndrome

ResearchPrincipal Investigator · Awarded by National Institutes of Health · 2001 - 2004

External Relationships


  • Alexion Pharmaceuticals, Inc. a subsidiary of AstraZeneca
  • Alnylam
  • Amicus Therapeutics
  • Arobiotherapeutics
  • AskBio (owned by Bayer)
  • Astellas Gene Therapies (formerly Audentes Therapeutics, Inc)
  • Baebies, Inc.
  • Bayer
  • Denali
  • Genzyme Corporation (Sanofi Aventis)
  • Maze Therapeutics
  • Moderna Therapeutics, Inc.
  • Takeda Pharmaceutical Co. Ltd.

This faculty member (or a member of their immediate family) has reported outside activities with the companies, institutions, or organizations listed above. This information is available to institutional leadership and, when appropriate, management plans are in place to address potential conflicts of interest.