Polycystic liver diseases (PLD) are inherited disorders of the biliary epithelium, caused by genetic defects in proteins associated with intracellular organelles, mainly the endoplasmic reticulum and the cilium. PLD are characterized by the formation and progressive enlargement of multiple cysts scattered throughout the liver parenchyma, and include different entities, classified based on their pathology, inheritance pattern, involvement of the kidney and clinical features. PLD should be considered as congenital diseases of cholangiocyte signaling. Here, we will review the changes in signaling pathways involved in liver cyst formation and progression, and their impact on cholangiocyte physiology. Each pathway represents a potential target for therapies aimed at reducing disease progression.

Strazzabosco, M., Somlo, S. (2011). Polycystic Liver Diseases: Congenital Disorders of Cholangiocyte Signaling. GASTROENTEROLOGY, 140(7), 1855-1859 [10.1053/j.gastro.2011.04.030].

Polycystic Liver Diseases: Congenital Disorders of Cholangiocyte Signaling

STRAZZABOSCO, MARIO
Primo
;
2011

Abstract

Polycystic liver diseases (PLD) are inherited disorders of the biliary epithelium, caused by genetic defects in proteins associated with intracellular organelles, mainly the endoplasmic reticulum and the cilium. PLD are characterized by the formation and progressive enlargement of multiple cysts scattered throughout the liver parenchyma, and include different entities, classified based on their pathology, inheritance pattern, involvement of the kidney and clinical features. PLD should be considered as congenital diseases of cholangiocyte signaling. Here, we will review the changes in signaling pathways involved in liver cyst formation and progression, and their impact on cholangiocyte physiology. Each pathway represents a potential target for therapies aimed at reducing disease progression.
Articolo in rivista - Review Essay
Polycystic liver diseases, ADPKD, PCL
English
2011
140
7
1855
1859
none
Strazzabosco, M., Somlo, S. (2011). Polycystic Liver Diseases: Congenital Disorders of Cholangiocyte Signaling. GASTROENTEROLOGY, 140(7), 1855-1859 [10.1053/j.gastro.2011.04.030].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/20768
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