R
Randolph P. Matthews
Researcher at University of Pennsylvania
Publications - 32
Citations - 1328
Randolph P. Matthews is an academic researcher from University of Pennsylvania. The author has contributed to research in topics: Zebrafish & Gene knockdown. The author has an hindex of 18, co-authored 28 publications receiving 1188 citations. Previous affiliations of Randolph P. Matthews include Children's Hospital of Philadelphia.
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Inhibition of Jagged-mediated Notch signaling disrupts zebrafish biliary development and generates multi-organ defects compatible with an Alagille syndrome phenocopy
Kristin Lorent,Sang-Yeob Yeo,Takaya Oda,Settara C. Chandrasekharappa,Ajay B. Chitnis,Randolph P. Matthews,Michael Pack +6 more
TL;DR: Compound jagged and notch gene knockdowns alter zebrafish biliary, kidney, pancreatic, cardiac and craniofacial development in a manner compatible with an AGS phenocopy, confirming an evolutionarily conserved role for Notch signaling in vertebrate liver development, and support the zebra fish as a model system for diseases of the human biliary system.
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Mutations in VIPAR cause an arthrogryposis, renal dysfunction and cholestasis syndrome phenotype with defects in epithelial polarization
Andrew R. Cullinane,Anna Straatman-Iwanowska,Andreas Zaucker,Yoshiyuki Wakabayashi,Christopher K. Bruce,Guanmei Luo,Fatimah Rahman,Figen Gürakan,Eda Utine,Tanju Başarir Özkan,Jonas Denecke,Jurica Vuković,Maja Di Rocco,Hanna Mandel,Hakan Cangul,Hakan Cangul,Randolph P. Matthews,Steve G. Thomas,Joshua Z. Rappoport,Irwin M. Arias,Hartwig Wolburg,A.S. Knisely,Deirdre Kelly,Ferenc Müller,Eamonn R. Maher,Paul Gissen,Paul Gissen +26 more
TL;DR: It is shown that VIPAR forms a functional complex with VPS33B that interacts with RAB11A that has diverse functions in the pathways regulating apical-basolateral polarity in the liver and kidney.
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The microRNA-30 family is required for vertebrate hepatobiliary development.
Nicholas J. Hand,Zankhana R. Master,Steven F. EauClaire,Daniel Weinblatt,Randolph P. Matthews,Joshua R. Friedman +5 more
TL;DR: One of these miRNAs, the biliary miRNA miR-30a, is required for biliary development in zebrafish and is the first demonstration of a functional role for miRNA in hepatic organogenesis.
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Evidence from human and zebrafish that GPC1 is a biliary atresia susceptibility gene.
Shuang Cui,Melissa Leyva–Vega,Ellen A. Tsai,Steven F. EauClaire,Joseph T. Glessner,Hakon Hakonarson,Marcella Devoto,Marcella Devoto,Barbara Haber,Nancy B. Spinner,Randolph P. Matthews +10 more
TL;DR: Based on genetic analysis of patients with BA and zebrafish, GPC1 appears to be a BA susceptibility gene, and these findings support a role for Hedgehog signaling in the pathogenesis of BA.
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Fructose leads to hepatic steatosis in zebrafish that is reversed by mechanistic target of rapamycin (mTOR) inhibition
TL;DR: Assessment of the contribution of ER stress, oxidative stress, and Torc1 up‐regulation in the development of steatohepatitis in fructose‐treated larval zebrafish indicates that Torc 1 activation is required for hepatic lipid accumulation across models of NAFLD, and in patients.