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other inherited cholestatic conditions are caused by known gene defects.20 22 Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal recessive disease characterised by defective biliary secretion of bile acids and was first described in patients of Amish ancestry.7 It starts in infancy and necessitates liver transplantation in the first decade of life. The FIC1 gene (ATPase class I type 8B (ATP8B1)) underlying the disease is located on chromosome 18q21.8 The protein encoded by this gene probably functions as a canalicular P type ATPase that participates in maintaining the distribution of aminophospholipids between the inner and outer leaflets of the plasma membrane.23 Recurrent familial intrahepatic cholestasis (benign recurrent intrahepatic cholestasis (BRIC)) is characterised by recurrent attacks of cholestasis but without progression to chronic liver disease. Consistent with PFIC1
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