Cholangiocyte injury and ductopenic syndromes.
Cholangiopathies are characterized by a predominantly bile duct-directed inflammatory response that leads to bile duct injury and, if the injury is persistent, bile duct loss and the chance of developing bile duct cancer (cholangiocarcinoma). Although the cholangiopathies have broad range of etiologies and pathogenesis (e.g., inherited disorders, autoimmune disorders, infections, drug-induced, ischemia, and unknown etiology), all share the common pathogenetic target-the biliary epithelial cell (cholangiocyte). For the most part, the pathogenesis of these diseases is poorly understood, which correspondingly has restricted clinicians to nonspecific and usually ineffective therapies for these disorders. Nevertheless, significant advances toward the understanding of the mechanisms involved in cholangiocyte-directed inflammation, biliary fibrosis, cholangiocyte death, and cholangiocarcinoma have unfolded over the past 15 years that may provide us new hopes and schemes for treatment of these disorders.
Xia
Xuefeng
X
The University of Texas Houston Medical School, Houston, Texas 77030, USA.
Demorrow
Sharon
S
Francis
Heather
H
Glaser
Shannon
S
Alpini
Gianfranco
G
Marzioni
Marco
M
Fava
Giammarco
G
Lesage
Gene
G
D000818:Animals; D017209:Apoptosis; D001649:Bile Duct Diseases / Q000378:metabolism / Q000473:pathology*; D001652:Bile Ducts / Q000378:metabolism / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000378:metabolism / Q000473:pathology; D018281:Cholangiocarcinoma / Q000378:metabolism / Q000473:pathology*; D003094:Collagen / Q000378:metabolism; D018450:Disease Progression; D006801:Humans; D008954:Models, Biological
D003094:Collagen