Histometric and serial section observations of the intrahepatic bile ducts in primary biliary cirrhosis

Augmented expression of hepatocytes growth factor activator inhibitor type 1 (HAI-1) in intrahepatic small bile ducts in primary biliary cirrhosis

“…The intrahepatic biliary tree is classified into the intrahepatic large and small bile ducts by their size and distributions in the portal tracts [14,15]. In this study, septal and interlobular bile ducts are called as small bile ducts.…”

“…It usually affects middle-aged women [4] and often leads to liver failure and liver transplantation [9,18]. PBC is characterized histologically as a cholangitis of small bile ducts (chronic nonsuppurative destructive cholangitis, CNSDC), eventually followed by extensive loss of small bile ducts [9,14,18]. PBC is serologically characterized by the presence of antimitochondrial antibodies [9] and B cell-and T cell-mediated immune reaction targeting an inner lipoyl domain of the E2 component of pyruvate dehydrogenase [3,8,13,24].…”

Molecular cloning, and characterization and expression of dihydrolipoamide acetyltransferase component of murine pyruvate dehydrogenase complex in bile duct cancer cells

“…1 The destruction of small intrahepatic bile ducts leads to impaired bile secretion, progressive scarring, and the eventual development of cirrhosis and liver failure in PBC patients. 2,3 Although auto-reactive immune responses against PDC-E2 have been well characterized in PBC, the lack of a transgenic mouse model so far has limited study of the mechanism of this disease induction and the development of novel approaches to therapy. 4 In an attempt to further explore the molecular events that orchestrate the tissue damage in mouse models, we cloned the murine cDNA sequence of this gene and analyzed the protein structure relationship as compared with the previously reported E2 component of human 5 and rat 6 alpha-ketoacid dehydrogenase complexes.…”

Activation of ATM signaling pathway is involved in oxidative stress-induced expression of mito-inhibitory p21WAF1/Cip1 in chronic non-suppurative destructive cholangitis in primary biliary cirrhosis: An immunohistochemical study

“…Clinically, PBC presents with chronic and progressive cholestasis and liver failure [1][2][3]. Histologically, PBC is characterized as chronic non-suppurative destructive cholangitis (CNSDC), involving intrahepatic small bile ducts, and is eventually followed by their extensive loss [2,3,5]. Although there have been many immunological studies of these damaged bile ducts [6][7][8], there has been relatively little effort to define the effecter mechanism of BECs preceding apoptosis in progressive bile duct loss in PBC [9].…”