Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease.

作者: Mitsue Yasoshima , Yasunori Sato , Shinichi Furubo , Kazuo Kizawa , Takahiro Sanzen

DOI: 10.1002/PATH.2472

关键词: Basement membraneType IV collagenCongenital hepatic fibrosisMatrigelPathologyDuctal Plate MalformationEndocrinologyInternal medicineBiologyCaroli diseaseLamininIntrahepatic bile ducts

摘要: Congenital hepatic fibrosis (CHF) and Caroli's disease are though to result from ductal plate malformation, the basal laminar components play important roles in biliary differentiation during development. To clarify involvement of this study examined immunohistochemical expression laminin type IV collagen livers CHF disease. Using polycystic kidney (PCK) rat, an animal model with CHF, vivo vitro experiments were also performed. Immunostaining showed that basement membrane around intrahepatic bile ducts was degraded disease, PCK rats. The degradation observed foci cholangiocarcinoma situ In vitro, cholangiocytes found overexpress plasminogen a serine proteinase, tissue-type activator (tPA). When cultured Matrigel, amounts gel significantly reduced, addition alpha2-antiplasmin culture medium inhibited Matrigel. These results suggest overexpression tPA leads generation excessive plasmin, subsequent plasmin-dependent lysis extracellular matrix molecules may contribute dysgenesis including progressive cystic dilatation addition, it is suggested once develops epithelium tends transform into invasive carcinoma, due instability ducts.

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