cholangiopathies

Contribution of resident stem cells to liver and biliary tree regeneration in human diseases

Two distinct stem/progenitor cell populations of biliary origin have been identified in the adult liver and biliary tree. Hepatic Stem/progenitor Cells (HpSCs) are bipotent progenitor cells located within the canals of Hering and can be differentiated into mature hepatocytes and cholangiocytes; Biliary Tree Stem/progenitor Cells (BTSCs) are multipotent stem cells located within the peribiliary glands of large intrahepatic and extrahepatic bile ducts and able to differentiate into hepatic and pancreatic lineages.

Inflammatory and neoplastic cholangiopathies [Colangiopatie infiammatorie e neoplastiche]

L’albero biliare è costituito da cellule epiteliali mature, i colangiociti, ed è suddiviso in dotti biliari intra ed extraepatici. I colangiociti facilitano la secrezione e la modificazione dei costituenti della bile e fungono da condotti di trasporto della bile nell’intestino. L’alterazione della normale funzione del colangiocita può portare allo sviluppo di molteplici patologie biliari, conosciute come colangiopatie.

Secretin/secretin receptor signaling mediates biliary damage and liver fibrosis in early-stage primary biliary cholangitis

Primary biliary cholangitis (PBC) primarily targets cholangiocytes and is characterized by liver fibrosis and biliary proliferation. Activation of the secretin (Sct)/secretin receptor (SR) axis, expressed only by cholangiocytes, increases biliary proliferation, liver fibrosis, and bicarbonate secretion. We evaluated the effectiveness of SR antagonist treatment for early-stage PBC. Male and female dominant-negative TGF-β receptor II (dnTGF-βRII) (model of PBC) and wild-type mice at 12 wk of age were treated with saline or the SR antagonist, Sec 5-27, for 1 wk.

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