Hedgehog Proteins

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  Subject Areas on Research

  • A complex of distal appendage-associated kinases linked to human disease regulates ciliary trafficking and stability. 
  • A five-gene hedgehog signature developed as a patient preselection tool for hedgehog inhibitor therapy in medulloblastoma. 
  • A mutant PTH/PTHrP type I receptor in enchondromatosis. 
  • A novel role for primary cilia in airway remodeling. 
  • A role for sonic hedgehog signaling in the pathogenesis of human tracheoesophageal fistula. 
  • A small-molecule antagonist of the hedgehog signaling pathway. 
  • Aberrant Otx2 expression enhances migration and induces ectopic proliferation of hindbrain neuronal progenitor cells. 
  • Accumulation of hedgehog-responsive progenitors parallels alcoholic liver disease severity in mice and humans. 
  • Accumulation of natural killer T cells in progressive nonalcoholic fatty liver disease. 
  • Acquisition of a side population fraction augments malignant phenotype in ovarian cancer. 
  • Activation of Rac1 promotes hedgehog-mediated acquisition of the myofibroblastic phenotype in rat and human hepatic stellate cells. 
  • Activation of hedgehog signaling during fracture repair enhances osteoblastic-dependent matrix formation. 
  • Activity-dependent internalization of smoothened mediated by beta-arrestin 2 and GRK2. 
  • Aging reduces liver resiliency by dysregulating Hedgehog signaling. 
  • Alcohol activates the hedgehog pathway and induces related procarcinogenic processes in the alcohol-preferring rat model of hepatocarcinogenesis. 
  • BMP antagonism by Noggin is required in presumptive notochord cells for mammalian foregut morphogenesis. 
  • BMP signaling in the development of the mouse esophagus and forestomach. 
  • Beta-arrestin 2 regulates zebrafish development through the hedgehog signaling pathway. 
  • Beta-arrestin-mediated localization of smoothened to the primary cilium. 
  • Bile ductules and stromal cells express hedgehog ligands and/or hedgehog target genes in primary biliary cirrhosis. 
  • Bmp signaling regulates proximal-distal differentiation of endoderm in mouse lung development. 
  • Bone morphogenetic protein 2 opposes Shh-mediated proliferation in cerebellar granule cells through a TIEG-1-based regulation of Nmyc. 
  • Branching morphogenesis of the lung: new models for a classical problem. 
  • Cartilage-specific β-catenin signaling regulates chondrocyte maturation, generation of ossification centers, and perichondrial bone formation during skeletal development. 
  • Cellular and molecular responses of the uterus to embryo implantation can be elicited by locally applied growth factors. 
  • Characterization of subtle brain abnormalities in a mouse model of Hedgehog pathway antagonist-induced cleft lip and palate. 
  • Constitutive hedgehog signaling in chondrosarcoma up-regulates tumor cell proliferation. 
  • Contribution of hedgehog signaling to the establishment of left-right asymmetry in the sea urchin. 
  • Control of neuronal precursor proliferation in the cerebellum by Sonic Hedgehog. 
  • Cross-talk between Notch and Hedgehog regulates hepatic stellate cell fate in mice. 
  • Cutaneous wound healing: recruiting developmental pathways for regeneration. 
  • Decreased epithelial progesterone receptor A at the window of receptivity is required for preparation of the endometrium for embryo attachment. 
  • Desert Hedgehog/Patched 1 signaling specifies fetal Leydig cell fate in testis organogenesis. 
  • Developmental biology informs cancer: the emerging role of the hedgehog signaling pathway in upper gastrointestinal cancers. 
  • Developmental origins of precocial forelimbs in marsupial neonates. 
  • Developmental pathways in musculoskeletal neoplasia: involvement of the Indian Hedgehog-parathyroid hormone-related protein pathway. 
  • Don't hedge your bets: hedgehog signaling as a central mediator of endochondral bone development and cartilage diseases. 
  • EXT1 regulates chondrocyte proliferation and differentiation during endochondral bone development. 
  • Elaboration of tubules with active hedgehog drives parenchymal fibrogenesis in gestational alloimmune liver disease. 
  • Epicardial regeneration is guided by cardiac outflow tract and Hedgehog signalling. 
  • Epigenetic targeting of Hedgehog pathway transcriptional output through BET bromodomain inhibition. 
  • Epithelial progesterone receptor exhibits pleiotropic roles in uterine development and function. 
  • Evidence from normal expression and targeted misexpression that bone morphogenetic protein (Bmp-4) plays a role in mouse embryonic lung morphogenesis. 
  • Evolution and development of the mammalian dentition: insights from the marsupial Monodelphis domestica. 
  • Evolutionary genomics and adaptive evolution of the Hedgehog gene family (Shh, Ihh and Dhh) in vertebrates. 
  • Expression of bone morphogenetic protein-4 (BMP-4), bone morphogenetic protein-7 (BMP-7), fibroblast growth factor-8 (FGF-8) and sonic hedgehog (SHH) during branchial arch development in the chick. 
  • Expression of the human poliovirus receptor/CD155 gene is activated by sonic hedgehog. 
  • Failure of human rhombic lip differentiation underlies medulloblastoma formation. 
  • Fetal alcohol exposure impairs Hedgehog cholesterol modification and signaling. 
  • Fibroblast growth factor 10 (FGF10) and branching morphogenesis in the embryonic mouse lung. 
  • Fibroblast growth factor blocks Sonic hedgehog signaling in neuronal precursors and tumor cells. 
  • Formation of telomeric repeat-containing RNA (TERRA) foci in highly proliferating mouse cerebellar neuronal progenitors and medulloblastoma. 
  • Genetic interaction screens identify a role for hedgehog signaling in Drosophila border cell migration. 
  • Genome sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition. 
  • Genomics and expression profiles of the Hedgehog and Notch signaling pathways in sea urchin development. 
  • Glucocorticoid hedgehog agonists in neurogenesis. 
  • HES1 is a novel downstream modifier of the SHH-GLI3 Axis in the development of preaxial polydactyly. 
  • Hedgehog Signaling Regulates Metabolism and Polarization of Mammary Tumor-Associated Macrophages. 
  • Hedgehog Signaling Regulates Treg to Th17 Conversion Through Metabolic Rewiring in Breast Cancer. 
  • Hedgehog Signaling is Associated with Liver Response to Fractionated Irradiation in Mice. 
  • Hedgehog activity, epithelial-mesenchymal transitions, and biliary dysmorphogenesis in biliary atresia. 
  • Hedgehog and Notch signaling regulate self-renewal of undifferentiated pleomorphic sarcomas. 
  • Hedgehog blockade in steroid-refractory sclerotic chronic graft-versus-host disease. 
  • Hedgehog controls hepatic stellate cell fate by regulating metabolism. 
  • Hedgehog inhibitors with and without adjunctive therapy in treatment of locally advanced basal cell carcinoma. 
  • Hedgehog inhibits β-catenin activity in synovial joint development and osteoarthritis. 
  • Hedgehog pathway activation and epithelial-to-mesenchymal transitions during myofibroblastic transformation of rat hepatic cells in culture and cirrhosis. 
  • Hedgehog pathway activation parallels histologic severity of injury and fibrosis in human nonalcoholic fatty liver disease. 
  • Hedgehog pathway and pediatric nonalcoholic fatty liver disease. 
  • Hedgehog pathway inhibition in chondrosarcoma using the smoothened inhibitor IPI-926 directly inhibits sarcoma cell growth. 
  • Hedgehog regulates yes-associated protein 1 in regenerating mouse liver. 
  • Hedgehog signaling antagonist promotes regression of both liver fibrosis and hepatocellular carcinoma in a murine model of primary liver cancer. 
  • Hedgehog signaling blockade delays hepatocarcinogenesis induced by hepatitis B virus X protein. 
  • Hedgehog signaling in cholangiocytes. 
  • Hedgehog signaling in human medullary thyroid carcinoma: a novel signaling pathway. 
  • Hedgehog signaling in the liver. 
  • Hedgehog signaling is critical for normal liver regeneration after partial hepatectomy in mice. 
  • Hedgehog signaling maintains resident hepatic progenitors throughout life. 
  • Hedgehog signaling patterns mesoderm in the sea urchin. 
  • Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans. 
  • Hedgehog signaling requires motile cilia in the sea urchin. 
  • Hedgehog signalling in liver pathophysiology. 
  • Hedgehog signalling regulates liver sinusoidal endothelial cell capillarisation. 
  • Hedgehog-GLI signaling inhibition suppresses tumor growth in squamous lung cancer. 
  • Hedgehog-YAP Signaling Pathway Regulates Glutaminolysis to Control Activation of Hepatic Stellate Cells. 
  • Hedgehog-mediated epithelial-to-mesenchymal transition and fibrogenic repair in nonalcoholic fatty liver disease. 
  • Hedgehog-mediated mesenchymal-epithelial interactions modulate hepatic response to bile duct ligation. 
  • Hepatic accumulation of Hedgehog-reactive progenitors increases with severity of fatty liver damage in mice. 
  • Hepatocyte Smoothened Activity Controls Susceptibility to Insulin Resistance and Nonalcoholic Fatty Liver Disease. 
  • Identification of CD15 as a marker for tumor-propagating cells in a mouse model of medulloblastoma. 
  • Identification of a novel Smoothened antagonist that potently suppresses Hedgehog signaling. 
  • Identification of select glucocorticoids as Smoothened agonists: potential utility for regenerative medicine. 
  • Ihh controls cartilage development by antagonizing Gli3, but requires additional effectors to regulate osteoblast and vascular development. 
  • In vivo monitoring of cardiomyocyte proliferation to identify chemical modifiers of heart regeneration. 
  • Increased production of sonic hedgehog by ballooned hepatocytes. 
  • Independent requirements for Hedgehog signaling by both the anterior heart field and neural crest cells for outflow tract development. 
  • Indian hedgehog as a progesterone-responsive factor mediating epithelial-mesenchymal interactions in the mouse uterus. 
  • Intracardiac septation requires hedgehog-dependent cellular contributions from outside the heart. 
  • Intracellular biosynthesis of lipids and cholesterol by Scap and Insig in mesenchymal cells regulates long bone growth and chondrocyte homeostasis. 
  • Involvement and targeted intervention of dysregulated Hedgehog signaling in osteosarcoma. 
  • Involvement of Sonic hedgehog (Shh) in mouse embryonic lung growth and morphogenesis. 
  • Jagged2 controls the generation of motor neuron and oligodendrocyte progenitors in the ventral spinal cord. 
  • Kif7 promotes hedgehog signaling in growth plate chondrocytes by restricting the inhibitory function of Sufu. 
  • Leptin promotes the myofibroblastic phenotype in hepatic stellate cells by activating the hedgehog pathway. 
  • Liver cell-derived microparticles activate hedgehog signaling and alter gene expression in hepatic endothelial cells. 
  • LncRNA wires up Hippo and Hedgehog signaling to reprogramme glucose metabolism. 
  • Loss of androgen signaling in mesenchymal sonic hedgehog responsive cells diminishes prostate development, growth, and regeneration. 
  • Loss of pericyte smoothened activity in mice with genetic deficiency of leptin. 
  • MR Imaging-Based Radiomic Signatures of Distinct Molecular Subgroups of Medulloblastoma. 
  • MRI Radiogenomics of Pediatric Medulloblastoma: A Multicenter Study. 
  • Macrophage-derived Hedgehog ligands promotes fibrogenic and angiogenic responses in human schistosomiasis mansoni. 
  • Mechanisms of disease progression in NASH: new paradigms. 
  • Mixed signals: development of the testis. 
  • Modulating hedgehog signaling can attenuate the severity of osteoarthritis. 
  • Mouse Mesenchyme forkhead 2 (Mf2): expression, DNA binding and induction by sonic hedgehog during somitogenesis. 
  • Multiple hereditary exostosis and hedgehog signaling: implications for novel therapies. 
  • Mutations in fam20b and xylt1 reveal that cartilage matrix controls timing of endochondral ossification by inhibiting chondrocyte maturation. 
  • Mutations of the Sonic Hedgehog Pathway Underlie Hypothalamic Hamartoma with Gelastic Epilepsy. 
  • Myofibroblastic cells function as progenitors to regenerate murine livers after partial hepatectomy. 
  • N-myc alters the fate of preneoplastic cells in a mouse model of medulloblastoma. 
  • N-myc can substitute for insulin-like growth factor signaling in a mouse model of sonic hedgehog-induced medulloblastoma. 
  • NKT-associated hedgehog and osteopontin drive fibrogenesis in non-alcoholic fatty liver disease. 
  • Non-alcoholic steatohepatitis pathogenesis: role of repair in regulating the disease progression. 
  • OTX2 is critical for the maintenance and progression of Shh-independent medulloblastomas. 
  • Osteopontin is induced by hedgehog pathway activation and promotes fibrosis progression in nonalcoholic steatohepatitis. 
  • Outcomes of adjunctive therapies post hedgehog inhibitors in the management of locally advanced basal cell carcinoma: A systematic review and pooled analysis. 
  • PTHrP regulates growth plate chondrocyte differentiation and proliferation in a Gli3 dependent manner utilizing hedgehog ligand dependent and independent mechanisms. 
  • Paracrine Hedgehog signaling drives metabolic changes in hepatocellular carcinoma. 
  • Parathyroid hormone-related protein regulates glioma-associated oncogene transcriptional activation: lessons learned from bone development and cartilage neoplasia. 
  • Perturbations to the hedgehog pathway in sea urchin embryos. 
  • Potential role of Hedgehog signaling and microRNA-29 in liver fibrosis of IKKβ-deficient mouse. 
  • Prevention of Barrett esophagus and esophageal adenocarcinoma by smoothened inhibitor in a rat model of gastroesophageal reflux disease. 
  • Primary cilia attenuate hedgehog signalling in neoplastic chondrocytes. 
  • Probing the multimodal fungiform papilla: complex peripheral nerve endings of chorda tympani taste and mechanosensitive fibers before and after Hedgehog pathway inhibition. 
  • Protecting the hedgerow: p53 and hedgehog pathway interactions. 
  • Rare and low-frequency coding variants alter human adult height. 
  • Reduced lipoapoptosis, hedgehog pathway activation and fibrosis in caspase-2 deficient mice with non-alcoholic steatohepatitis. 
  • Regulation of Cholesterol Homeostasis by Hedgehog Signaling in Osteoarthritic Cartilage. 
  • Regulation of hedgehog signaling by Myc-interacting zinc finger protein 1, Miz1. 
  • Repair-related activation of hedgehog signaling in stromal cells promotes intrahepatic hypothyroidism. 
  • Repair-related activation of hedgehog signaling promotes cholangiocyte chemokine production. 
  • Reply. 
  • Reply: To PMID 24849310. 
  • Role for hedgehog pathway in regulating growth and function of invariant NKT cells. 
  • Role for hedgehog signaling in hepatic stellate cell activation and viability. 
  • Role of Hedgehog Signaling Pathway in NASH. 
  • SOX17 regulates uterine epithelial-stromal cross-talk acting via a distal enhancer upstream of Ihh. 
  • Sequential roles of Hedgehog and Wnt signaling in osteoblast development. 
  • Signals from dying hepatocytes trigger growth of liver progenitors. 
  • Smoothened inhibition leads to decreased proliferation and induces apoptosis in esophageal adenocarcinoma cells. 
  • Smoothened is a master regulator of adult liver repair. 
  • Smoothened signaling in vertebrates is facilitated by a G protein-coupled receptor kinase. 
  • Sonic Hedgehog-induced proliferation requires specific Gα inhibitory proteins. 
  • Sonic hedgehog from pharyngeal arch 1 epithelium is necessary for early mandibular arch cell survival and later cartilage condensation differentiation. 
  • Sonic hedgehog induces GLT-1 degradation via PKC delta to suppress its transporter activities. 
  • Sonic hedgehog is an autocrine viability factor for myofibroblastic hepatic stellate cells. 
  • Sonic hedgehog is required for cardiac outflow tract and neural crest cell development. 
  • Sonic hedgehog ligand partners with caveolin-1 for intracellular transport. 
  • Sonic hedgehog signaling pathway supports cancer cell growth during cancer radiotherapy. 
  • Sonic-hedgehog-mediated proliferation requires the localization of PKA to the cilium base. 
  • Sprinter: a novel transmembrane protein required for Wg secretion and signaling. 
  • Staggered cell-intrinsic timing of ath5 expression underlies the wave of ganglion cell neurogenesis in the zebrafish retina. 
  • Statins activate the canonical hedgehog-signaling and aggravate non-cirrhotic portal hypertension, but inhibit the non-canonical hedgehog signaling and cirrhotic portal hypertension. 
  • Structures of human Patched and its complex with native palmitoylated sonic hedgehog. 
  • Subgroup-specific structural variation across 1,000 medulloblastoma genomes. 
  • Suppressor of fused (Sufu) mediates the effect of parathyroid hormone-like hormone (Pthlh) on chondrocyte differentiation in the growth plate. 
  • Survivin as a therapeutic target in Sonic hedgehog-driven medulloblastoma. 
  • Tamoxifen-inducible gene deletion reveals a distinct cell type associated with trabecular bone, and direct regulation of PTHrP expression and chondrocyte morphology by Ihh in growth region cartilage. 
  • Targeted Therapy for Advanced Solid Tumors on the Basis of Molecular Profiles: Results From MyPathway, an Open-Label, Phase IIa Multiple Basket Study. 
  • Targeting sonic hedgehog-associated medulloblastoma through inhibition of Aurora and Polo-like kinases. 
  • Targeting stem cell behavior in desmoid tumors (aggressive fibromatosis) by inhibiting hedgehog signaling. 
  • The Meckel syndrome- associated protein MKS1 functionally interacts with components of the BBSome and IFT complexes to mediate ciliary trafficking and hedgehog signaling. 
  • The adventures of sonic hedgehog in development and repair. II. Sonic hedgehog and liver development, inflammation, and cancer. 
  • The hedgehog pathway in nonalcoholic fatty liver disease. 
  • The hedgehog pathway regulates remodelling responses to biliary obstruction in rats. 
  • The insecticide synergist piperonyl butoxide inhibits hedgehog signaling: assessing chemical risks. 
  • The mammalian twisted gastrulation gene functions in foregut and craniofacial development. 
  • The novel triad of dorsal agenesis of the pancreas with concurrent pancreatic ductal adenocarcinoma and nonalcoholic chronic calcific pancreatitis: a case series and review of the literature. 
  • The primary cilium as a Hedgehog signal transduction machine. 
  • The primary cilium: a signalling centre during vertebrate development. 
  • The role of Hedgehog signaling in fibrogenic liver repair. 
  • The role of hedgehog signalling in skeletal health and disease. 
  • The segment polarity gene porcupine encodes a putative multitransmembrane protein involved in Wingless processing. 
  • The spinocerebellar ataxia-associated gene Tau tubulin kinase 2 controls the initiation of ciliogenesis. 
  • The transcriptional landscape of Shh medulloblastoma. 
  • Thymosin beta-4 regulates activation of hepatic stellate cells via hedgehog signaling. 
  • Tissue interactions pattern the mesenchyme of the embryonic mouse lung. 
  • Transcriptional profiling of the Sonic hedgehog response: a critical role for N-myc in proliferation of neuronal precursors. 
  • Transcriptome changes affecting Hedgehog and cytokine signalling in the umbilical cord: implications for disease risk. 
  • Treatment response in the PIVENS trial is associated with decreased Hedgehog pathway activity. 
  • TrkB-Shc Signaling Protects against Hippocampal Injury Following Status Epilepticus. 
  • Twist is an integrator of SHH, FGF, and BMP signaling. 
  • Two Patched molecules engage distinct sites on Hedgehog yielding a signaling-competent complex. 
  • Unique and overlapping GLI1 and GLI2 transcriptional targets in neoplastic chondrocytes. 
  • Up-regulation of Hedgehog pathway is associated with cellular permissiveness for hepatitis C virus replication. 
  • Updating the Wnt pathways. 
  • Viral factors induce Hedgehog pathway activation in humans with viral hepatitis, cirrhosis, and hepatocellular carcinoma. 
  • Vismodegib Exerts Targeted Efficacy Against Recurrent Sonic Hedgehog-Subgroup Medulloblastoma: Results From Phase II Pediatric Brain Tumor Consortium Studies PBTC-025B and PBTC-032. 
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  Keywords of People

  • Choi, Steven Sok, Associate Professor of Medicine, Medicine, Gastroenterology
  • Hilton, Matthew James, Associate Professor in Orthopaedic Surgery, Cell Biology
  • Hogan, Brigid L. M., Research Professor of Cell Biology, Cell Biology
  • McCall, Shannon Jones, Associate Professor of Pathology, Pathology
  • McLendon, Roger Edwin, Professor of Pathology, Pathology
  • Pang, Herbert, Adjunct Assistant Professor in the Department of Biostatistics & Bioinformatics, Biostatistics & Bioinformatics
  • Sullivan, Daniel Carl, Professor Emeritus of Radiology, Radiology