Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice
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چکیده
منابع مشابه
06-P012 Haploinsufficiency of Sox17 causes defective maturation of fetal livers in C57BL6 mice
ro is the acquisition of protective barrier function in the skin a process involving differentiation of keratinocytes to form a stratum corneum of unique lipid composition and structure. Harlequin Ichthyosis (HI) is a severe hyperkeratotic skin disease caused by mutations in the ABCA12 transport protein and which results in profound barrier defects. In keratinocytes ABCA12 is thought to regulat...
متن کاملNon-Invasive Diagnostic Methods for Differentiation of Biliary Atresia from Neonatal Hepatitis in Upper Egypt
Background Cholestatic jaundice in infancy is always pathologic and mainly caused by biliary atresia (BA), and neonatal hepatitis (NH). The early discrimination of both conditions is critical for the outcome of BA. We aimed to assess different non-invasive diagnostic tools in differentiating BA from NH. Materials and Methods Forty infants (25 boys, 15 girls) with cholestatic jaundice and final...
متن کاملEmbryonic cholecystitis and defective gallbladder contraction in the Sox17-haploinsufficient mouse model of biliary atresia.
The gallbladder excretes cytotoxic bile acids into the duodenum through the cystic duct and common bile duct system. Sox17 haploinsufficiency causes biliary atresia-like phenotypes and hepatitis in late organogenesis mouse embryos, but the molecular and cellular mechanisms underlying this remain unclear. In this study, transcriptomic analyses revealed the early onset of cholecystitis in Sox17+/...
متن کاملExtrahepatic biliary atresia versus neonatal hepatitis
Mowat, A. P., Psacharopoulos, H. D., and Williams, R. (1976). Archives of Disease in Childhood, 51, 763. Extrahepatic biliary atresia versus neonatal hepatitis: review of 137 prospectively investigated infants. In a prospective regional survey of neonatal hepatitis syndrome 32 infants had extrahepatic biliary atresia (EHBA) and 103 had hepatitis. No cause for the lesion was found in infants wit...
متن کاملThe Role of Appendix in the Management of Biliary Atresia Associated with Bowel Atresia
Biliary atresia is characterized by the progressive obliteration of extra- and intrahepatic biliary duct system leading to the obstruction of bile flow in infancy. The cause(s) of biliary atresia remain unclear and many surgical options for bypassing the atretic segment have been described.1-3 Biliary atresia may be associated with small bowel atresia. The surgical interventions for biliary dr...
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ژورنال
عنوان ژورنال: Development
سال: 2013
ISSN: 1477-9129,0950-1991
DOI: 10.1242/dev.086702