The morbidity and mortality from chronic biliary diseases (i.e., the cholangiopathies) remains substantial. End-stage liver disease from biliary causes of cirrhosis (e.g., primary biliary cirrhosis [
Structural and Functional Characteristics of the Bile Duct SystemT his session focused on (1) the role of cell adhesion molecules in the development and morphogenesis of the biliary epithelium; (2) the identification of facultative stem cells, which are activated in certain pathologic conditions and play a role in the development of hepatocytes and bile ducts; (3) the use of sophisticated techniques, which are important in the identification of specific branches of the biliary tree that differentially respond to toxins/injury; and (4) the concept that the biliary epithelium is heterogeneous with regard to morphology and secretory, proliferative, and apoptotic events. Overall, this session provided important information relevant to the functional structure of the biliary tree in normal and pathologic conditions and on the possible identification of hepatic stem cells.
LPIN1-related myolysis constitutes a major cause of early-onset rhabdomyolysis and occasionally in adults. Heterozygous LPIN1 mutations may cause mild muscular symptoms. No major defects of LPIN2 or LPIN3 genes were associated with muscular manifestations.
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