Randolph P Matthews

Randolph P Matthews

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Randolph P Matthews

Randolph P Matthews

Publications by authors named "Randolph P Matthews"

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Assessment of Bezlotoxumab Immunogenicity.

Clin Pharmacol Drug Dev 2019 Aug 14. Epub 2019 Aug 14.

Merck & Co., Inc., Kenilworth, New Jersey, USA.

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http://dx.doi.org/10.1002/cpdd.729DOI Listing
August 2019

Loss of a Candidate Biliary Atresia Susceptibility Gene, add3a, Causes Biliary Developmental Defects in Zebrafish.

J Pediatr Gastroenterol Nutr 2016 11;63(5):524-530

*Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia Research Institute †Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5074882PMC
http://dx.doi.org/10.1097/MPG.0000000000001375DOI Listing
November 2016

Methylation Microarray Studies Highlight PDGFA Expression as a Factor in Biliary Atresia.

PLoS One 2016 24;11(3):e0151521. Epub 2016 Mar 24.

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0151521PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4806872PMC
August 2016

Fructose leads to hepatic steatosis in zebrafish that is reversed by mechanistic target of rapamycin (mTOR) inhibition.

Hepatology 2014 Nov 1;60(5):1581-92. Epub 2014 Oct 1.

Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia Research Institute, Philadelphia, PA.

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http://dx.doi.org/10.1002/hep.27284DOI Listing
November 2014

CCBE1 mutation in two siblings, one manifesting lymphedema-cholestasis syndrome, and the other, fetal hydrops.

PLoS One 2013 26;8(9):e75770. Epub 2013 Sep 26.

Liver Center Laboratory, Department of Medicine, University of California San Francisco, San Francisco, California, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0075770PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3784396PMC
July 2014

p53-mediated biliary defects caused by knockdown of cirh1a, the zebrafish homolog of the gene responsible for North American Indian Childhood Cirrhosis.

PLoS One 2013 11;8(10):e77670. Epub 2013 Oct 11.

Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0077670PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3795688PMC
June 2014

Cholangiocarcinoma in a young woman with perinatally acquired HIV.

J Pediatr Gastroenterol Nutr 2013 Jun;56(6):e43-6

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.

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https://insights.ovid.com/crossref?an=00005176-201306000-000
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http://dx.doi.org/10.1097/MPG.0b013e3182618171DOI Listing
June 2013

Interferon-gamma directly mediates developmental biliary defects.

Zebrafish 2013 Jun 28;10(2):177-83. Epub 2013 Feb 28.

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia Research Institute , Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1089/zeb.2012.0815DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3678566PMC
June 2013

Evidence from human and zebrafish that GPC1 is a biliary atresia susceptibility gene.

Gastroenterology 2013 May 18;144(5):1107-1115.e3. Epub 2013 Jan 18.

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia Research Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1053/j.gastro.2013.01.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3736559PMC
May 2013

Mutations in vacuolar H+ -ATPase subunits lead to biliary developmental defects in zebrafish.

Dev Biol 2012 May 16;365(2):434-44. Epub 2012 Mar 16.

The Children's Hospital of Philadelphia Research Institute, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.ydbio.2012.03.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3337356PMC
May 2012

Significant liver disease in a patient with Y116H mutation in the MVK gene.

Am J Med Genet A 2011 Jun 5;155A(6):1461-4. Epub 2011 May 5.

Division of Gastroenterology, Hepatology and Nutrition, University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1002/ajmg.a.33915DOI Listing
June 2011

Disruption of planar cell polarity activity leads to developmental biliary defects.

Dev Biol 2011 Mar 4;351(2):229-41. Epub 2011 Jan 4.

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

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http://dx.doi.org/10.1016/j.ydbio.2010.12.041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3045807PMC
March 2011

Intrahepatic biliary anomalies in a patient with Mowat-Wilson syndrome uncover a role for the zinc finger homeobox gene zfhx1b in vertebrate biliary development.

J Pediatr Gastroenterol Nutr 2011 Mar;52(3):339-44

The Children's Hospital of Philadelphia Research Institute, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1097/MPG.0b013e3181ff2e5bDOI Listing
March 2011

The microRNA-30 family is required for vertebrate hepatobiliary development.

Gastroenterology 2009 Mar 9;136(3):1081-90. Epub 2008 Dec 9.

Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, University of Pennsylvania School of Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1053/j.gastro.2008.12.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2672911PMC
March 2009

TNFalpha-dependent hepatic steatosis and liver degeneration caused by mutation of zebrafish S-adenosylhomocysteine hydrolase.

Development 2009 Mar;136(5):865-75

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1242/dev.027565DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2685951PMC
March 2009

Transcription factor onecut3 regulates intrahepatic biliary development in zebrafish.

Dev Dyn 2008 Jan;237(1):124-31

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Pennsylvania, USA.

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http://doi.wiley.com/10.1002/dvdy.21407
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http://dx.doi.org/10.1002/dvdy.21407DOI Listing
January 2008

Zebrafish vps33b, an ortholog of the gene responsible for human arthrogryposis-renal dysfunction-cholestasis syndrome, regulates biliary development downstream of the onecut transcription factor hnf6.

Development 2005 Dec;132(23):5295-306

Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1242/dev.02140DOI Listing
December 2005

Inhibition of Jagged-mediated Notch signaling disrupts zebrafish biliary development and generates multi-organ defects compatible with an Alagille syndrome phenocopy.

Development 2004 Nov;131(22):5753-66

Department of Medicine, University of Pennsylvania School of Medicine, 421 Curie Boulevard, Philadelphia, PA 19104-6058, USA.

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http://dx.doi.org/10.1242/dev.01411DOI Listing
November 2004

The zebrafish onecut gene hnf-6 functions in an evolutionarily conserved genetic pathway that regulates vertebrate biliary development.

Dev Biol 2004 Oct;274(2):245-59

Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA.

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http://dx.doi.org/10.1016/j.ydbio.2004.06.016DOI Listing
October 2004

Cases in pediatric gastroenterology from The Children's Hospital of Philadelphia. A 5-year-old girl with massive hepatosplenomegaly, fever, and rash.

MedGenMed 2004 May 3;6(2):14. Epub 2004 May 3.

Division of Gastroenterology & Nutrition, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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May 2004

A 1-month-old with prolonged jaundice.

Pediatr Case Rev 2003 Apr;3(2):63-74

Department of Pediatrics, Children's Hospital of Philadelphia, Universityof Pennsylvania, 19104, USA.

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http://dx.doi.org/10.1097/01.PCA.0000057549.04708.DEDOI Listing
April 2003

Biliary atresia associated with a fatty acid oxidation defect.

J Pediatr Gastroenterol Nutr 2002 Nov;35(5):624-8

Department of Pediatrics, Division of Gastroenterology and Nutrition and Fred and Suzanne Biesecker Liver Center, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1097/00005176-200211000-00007DOI Listing
November 2002