Intrahepatic Biliary Anomalies in a Patient With Mowat‐Wilson Syndrome Uncover a Role for the Zinc Finger Homeobox Gene zfhx1b in Vertebrate Biliary Development

ABSTRACT Background: zfhz1b is the causative gene for Mowat‐Wilson syndrome, in which patients demonstrate developmental delay and Hirschsprung disease, as well as other anomalies. Materials and Methods: We identified a patient with Mowat‐Wilson syndrome who also developed cholestasis and histopatho...

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Veröffentlicht in:	Journal of pediatric gastroenterology and nutrition 2011-03, Vol.52 (3), p.339-344
Hauptverfasser:	Cui, Shuang, Erlichman, Jessi, Russo, Pierre, Haber, Barbara A, Matthews, Randolph P
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals biliary atresia Biliary Atresia - etiology Biliary Atresia - genetics Biliary Atresia - metabolism Biliary Tract - abnormalities Biliary Tract - growth & development Biological and medical sciences cholestatic liver disease Facies Feeding. Feeding behavior Fundamental and applied biological sciences. Psychology Gastroenterology. Liver. Pancreas. Abdomen Genes, Homeobox Hepatocyte Nuclear Factor 1-beta - metabolism Hirschsprung disease Hirschsprung Disease - complications Hirschsprung Disease - genetics Hirschsprung Disease - metabolism Homeodomain Proteins - genetics Homeodomain Proteins - metabolism Humans Infant Intellectual Disability - complications Intellectual Disability - genetics Intellectual Disability - metabolism Liver - metabolism liver development Liver. Biliary tract. Portal circulation. Exocrine pancreas Male Malformations Medical sciences Microcephaly - complications Microcephaly - genetics Microcephaly - metabolism Mutation Oligoribonucleotides, Antisense - pharmacology Other diseases. Semiology Repressor Proteins - genetics Repressor Proteins - metabolism Stomach. Duodenum. Small intestine. Colon. Rectum. Anus Vertebrates: anatomy and physiology, studies on body, several organs or systems Zebrafish zfhx1b Zinc Finger E-box Binding Homeobox 2 Zinc Fingers
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