Article
Biliary dysgenesis in the PCK rat, an orthologous model of autosomal recessive polycystic kidney disease.
The American journal of pathology - 1 Nov 2004
Masyuk Tatyana V, Huang Bing Q, Masyuk Anatoliy I, Ritman Erik L, Torres Vicente E, Wang Xiaofang, Harris Peter C, Larusso Nicholas F
Abstract excerpt
Hepatic polycystic disease occurs alone or in combination with polycystic kidney disease (PKD). In autosomal recessive PKD (ARPKD), liver lesions are the major cause of morbidity and mortality in older patients. ARPKD is caused by a mutation to PKHD1 and the PCK rat is an orthologous model of disease. Recently, we showed that fibrocystin, Pkhd1 protein, is localized to primary cilia in rat cholangiocytes and that...
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