Article
Polycystic liver disease is a disorder of cotranslational protein processing.
Trends in molecular medicine - 1 Jan 2005
Drenth Joost P H, Martina Jose A, van de Kerkhof Rolf, Bonifacino Juan S, Jansen Jan B M J
Abstract excerpt
Autosomal-dominant polycystic liver disease (PCLD) is a rare disorder that is characterized by the progressive development of fluid-filled biliary epithelial cysts in the liver. Positional cloning has identified two genes that are mutated in patients with polycystic liver disease, PRKCSH and SEC63, which encode the beta-subunit of glucosidase II and Sec63, respectively. Both proteins are components of the...
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