ZFIN ID: ZDB-LAB-120209-1
Matthews Lab
PI/Director: Matthews, Randy
Contact Person: Matthews, Randy
Email: matthews@email.chop.edu
Address: Division of GI, Hepatology & Nutrition The Children's Hospital of Philadelphia University of Pennsylvania School of Medicine Dept. of Pediatrics Philadelphia PA USA
Country: United States
Phone: (267) 426-0221
Line Designation: p

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Cui, Shuang Alice Post-Doc EauClaire, Steven Technical Staff

Tang, V., Cofer, Z.C., Cui, S., Sapp, V., Loomes, K.M., Matthews, R.P. (2016) Loss of a Candidate Biliary Atresia Susceptibility Gene, add3a, Causes Biliary Developmental Defects in Zebrafish. Journal of pediatric gastroenterology and nutrition. 63(5):524-530
Cofer, Z.C., Cui, S., EauClaire, S.F., Kim, C., Tobias, J.W., Hakonarson, H., Loomes, K.M., Matthews, R.P. (2016) Methylation Microarray Studies Highlight PDGFA Expression as a Factor in Biliary Atresia. PLoS One. 11:e0151521
Sapp, V., Gaffney, L., EauClaire, S.F., Matthews, R.P. (2014) Fructose leads to hepatic steatosis in zebrafish that is reversed by mTOR inhibition. Hepatology (Baltimore, Md.). 60(5):1581-92
Wilkins, B.J., Lorent, K., Matthews, R.P., and Pack, M. (2013) p53-Mediated Biliary Defects Caused by Knockdown of cirh1a, the Zebrafish Homolog of the Gene Responsible for North American Indian Childhood Cirrhosis. PLoS One. 8(10):e77670
Cui, S., Eauclaire, S.F., and Matthews, R.P. (2013) Interferon-Gamma Directly Mediates Developmental Biliary Defects. Zebrafish. 10(2):177-83
Cui, S., Leyva-Vega, M., Tsai, E.A., Eauclaire, S.F., Glessner, J.T., Hakonarson, H., Devoto, M., Haber, B.A., Spinner, N.B., and Matthews, R.P. (2013) Evidence from Human and Zebrafish that GPC1 is a Biliary Atresia Susceptibility Gene. Gastroenterology. 144(5):1107-1115
Eauclaire, S.F., Cui, S., Ma, L., Matous, J., Marlow, F.L., Gupta, T., Burgess, H.A., Abrams, E.W., Kapp, L.D., Granato, M., Mullins, M.C., and Matthews, R.P. (2012) Mutations in vacuolar H(+)-ATPase subunits lead to biliary developmental defects in zebrafish. Developmental Biology. 365(2):434-444
Clark, B.S., Cui, S., Miesfeld, J.B., Klezovitch, O., Vasioukhin, V., and Link, B.A. (2012) Loss of Llgl1 in retinal neuroepithelia reveals links between apical domain size, Notch activity and neurogenesis. Development (Cambridge, England). 139(9):1599-1610
Cui, S., Capecci, L.M., and Matthews, R.P. (2011) Disruption of planar cell polarity activity leads to developmental biliary defects. Developmental Biology. 351(2):229-241
Cui, S., Erlichman, J., Russo, P., Haber, B.A., and Matthews, R.P. (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. Journal of pediatric gastroenterology and nutrition. 52(3):339-344
Matthews, R.P., Eauclaire, S.F., Mugnier, M., Lorent, K., Cui, S., Ross, M.M., Zhang, Z., Russo, P., and Pack, M. (2011) DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia. Hepatology (Baltimore, Md.). 53(3):905-914
Cullinane, A.R., Straatman-Iwanowska, A., Zaucker, A., Wakabayashi, Y., Bruce, C.K., Luo, G., Rahman, F., Gürakan, F., Utine, E., Ozkan, T.B., Denecke, J., Vukovic, J., Di Rocco, M., Mandel, H., Cangul, H., Matthews, R.P., Thomas, S.G., Rappoport, J.Z., Arias, I.M., Wolburg, H., Knisely, A.S., Kelly, D.A., Muller, F., Maher, E.R., and Gissen, P. (2010) Mutations in VIPAR cause an arthrogryposis, renal dysfunction and cholestasis syndrome phenotype with defects in epithelial polarization. Nature Genetics. 42(4):303-312
Matthews, R.P., Lorent, K., Mañoral-Mobias, R., Huang, Y., Gong, W., Murray, I.V., Blair, I.A., and Pack, M. (2009) TNF{alpha}-dependent hepatic steatosis and liver degeneration caused by mutation of zebrafish s-adenosylhomocysteine hydrolase. Development (Cambridge, England). 136(5):865-875
Matthews, R.P., Lorent, K., and Pack, M. (2008) Transcription factor onecut3 regulates intrahepatic biliary development in zebrafish. Developmental Dynamics : an official publication of the American Association of Anatomists. 237(1):124-131
Cui, S., Otten, C., Rohr, S., Abdelilah-Seyfried, S., and Link, B.A. (2007) Analysis of aPKClambda and aPKCzeta reveals multiple and redundant functions during vertebrate retinogenesis. Molecular and cellular neurosciences. 34(3):431-444
Cui, S. (2007) Analysis of APKC and LGL function in retinal development. Ph.D. Thesis. :192p
Matthews, R.P., Plumb-Rudewiez, N., Lorent, K., Gissen, P., Johnson, C.A., Lemaigre, F., and Pack, M. (2005) 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 (Cambridge, England). 132(23):5295-5306
Matthews, R.P., Lorent, K., Russo, P., and Pack, M. (2004) The zebrafish onecut gene hnf-6 functions in an evolutionarily conserved genetic pathway that regulates vertebrate biliary development. Developmental Biology. 274(2):245-259