RandolphPMatthewsMD

PubMed

• 28 citations
  Loss of a Candidate Biliary Atresia Susceptibility Gene, add3a, Causes Biliary Developmental Defects in Zebrafish.
  Vivian Tang, Zenobia C. Cofer, Shuang Cui, Valerie Sapp, Kathleen M. Loomes, Randolph P. Matthews> ;Journal of Pediatric Gastroenterology and Nutrition. 2016 Nov 1
• 16 citations
  Methylation Microarray Studies Highlight PDGFA Expression as a Factor in Biliary Atresia.
  Zenobia C. Cofer, Shuang Cui, Steven F. EauClaire, Cecilia Kim, John W. Tobias, Hakon Hakonarson, Kathleen M. Loomes, Randolph P. Matthews> ;Plos One. 2016 Mar 24
• 85 citations
  Fructose leads to hepatic steatosis in zebrafish that is reversed by mechanistic target of rapamycin (mTOR) inhibition
  Valerie Sapp, Leah Gaffney, Steven F. EauClaire, Randolph P. Matthews> ;Hepatology. 2014 Nov 1
• 27 citations
  p53-Mediated Biliary Defects Caused by Knockdown of cirh1a, the Zebrafish Homolog of the Gene Responsible for North American Indian Childhood Cirrhosis
  Benjamin J. Wilkins, Kristin Lorent, Randolph P. Matthews, Michael Pack> ;Plos One. 2013 Oct 11
• 23 citations
  CCBE1 Mutation in Two Siblings, One Manifesting Lymphedema-Cholestasis Syndrome, and the Other, Fetal Hydrops
  Sohela Shah, Laura K. Conlin, Luis M. Gomez, Øystein Aagenaes, Kristin Eiklid, A.S. Knisely, Michael T. Mennuti, Randolph P. Matthews, Nancy B. Spinner, Laura N. Bull> ;Plos One. 2013 Sep 26
• 9 citations
  Interferon-gamma directly mediates developmental biliary defects.
  Shuang Cui, Steven F. EauClaire, Randolph P. Matthews> ;Zebrafish. 2013 Jun 5
• Cholangiocarcinoma in a young woman with perinatally acquired HIV
  Richard M. Rutstein, Joshua P. Cantor, Sudha A. Anupindi, Randolph P. Matthews> ;Journal of Pediatric Gastroenterology and Nutrition. 2013 Jun 1
• 90 citations
  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, Barbara Haber, Nancy B. Spinner, Randolph P....> ;Gastroenterology. 2013 May 1
• 22 citations
  Mutations in vacuolar H+-ATPase subunits lead to biliary developmental defects in zebrafish
  Steven F. EauClaire, Shuang Cui, Liyuan Ma, James Matous, Florence L. Marlow, Tripti Gupta, Harold A. Burgess, Elliott W. Abrams, Lee Kapp, Michael Granato, Mary C. Mu...> ;Developmental Biology. 2012 May 15
• 9 citations
  Significant liver disease in a patient with Y116H mutation in the MVK gene.
  Melissa Leyva-Vega, Pamela F. Weiss, Jaya Ganesh, Laura K. Conlin, Nancy B. Spinner, Randolph P. Matthews> ;American Journal of Medical Genetics. Part A. 2011 Jun 1
• 29 citations
  Disruption of planar cell polarity activity leads to developmental biliary defects.
  Shuang Cui, Louis M. Capecci, Randolph P. Matthews> ;Developmental Biology. 2011 Mar 15
• 13 citations
  Intrahepatic biliary anomalies in a patient with Mowat-Wilson syndrome uncover a role for the zinc finger homeobox gene zfhx1b in vertebrate biliary development.
  Shuang Cui, Jessi Erlichman, Pierre Russo, Barbara Haber, Randolph P. Matthews> ;Journal of Pediatric Gastroenterology and Nutrition. 2011 Mar 1
• 45 citations
  DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia.
  Randolph P. Matthews, Steven F. EauClaire, Monica R. Mugnier, Kristin Lorent, Shuang Cui, Megan M. Ross, Zhe Zhang, Pierre Russo, Michael Pack> ;Hepatology. 2011 Mar 1
• 126 citations
  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, Tan...> ;Nature Genetics. 2010 Apr 1
• 69 citations
  TNFα-dependent hepatic steatosis and liver degeneration caused by mutation of zebrafish s-adenosylhomocysteine hydrolase
  Randolph P. Matthews, Kristin Lorent, Rafael Mañoral-Mobias, Yuehua Huang, Weilong Gong, Ian V.J. Murray, Ian A. Blair, Michael Pack> ;Development. 2009 Mar 1
• 107 citations
  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> ;Gastroenterology. 2009 Mar 1
• 23 citations
  Transcription factor onecut3 regulates intrahepatic biliary development in zebrafish.
  Randolph P. Matthews, Kristin Lorent, Michael Pack> ;Developmental Dynamics. 2008 Jan 1
• 49 citations
  Zebrafish vps33b, an ortholog of the gene responsible for human arthrogryposis-renal dysfunction-cholestasis syndrome, regulates biliary development downstream of the ...
  Randolph P. Matthews, Nicolas Plumb-Rudewiez, Kristin Lorent, Paul Gissen, Colin A. Johnson, Frédéric P. Lemaigre, Michael Pack> ;Development. 2005 Dec 1
• 162 citations
  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> ;Development. 2004 Nov 15
• 65 citations
  The zebrafish onecut gene hnf-6 functions in an evolutionarily conserved genetic pathway that regulates vertebrate biliary development.
  Randolph P. Matthews, Kristin Lorent, Pierre Russo, Michael Pack> ;Developmental Biology. 2004 Oct 15
• 1 citations
  Cases in pediatric gastroenterology from The Children's Hospital of Philadelphia. A 5-year-old girl with massive hepatosplenomegaly, fever, and rash.
  Randolph P. Matthews, Barbara Haber, Petar Mamula, David A. Piccoli> ;Medgenmed. 2004 May 3
• A 1-month-old with prolonged jaundice.
  Randolph P. Matthews, Kurt A. Brown> ;Pediatric Case Reviews. 2003 Apr 1
• 3 citations
  Biliary atresia associated with a fatty acid oxidation defect.
  Randolph P. Matthews, Pierre Russo, Gerard T. Berry, David A. Piccoli, Elizabeth B. Rand> ;Journal of Pediatric Gastroenterology and Nutrition. 2002 Nov 1
• 22 citations
  Group C streptococcal meningitis: case report and review of the literature.
  Samir S. Shah, Randolph P. Matthews, Christopher Cohen> ;The Pediatric Infectious Disease Journal. 2001 Apr 1
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