Polycystic liver diseases: congenital disorders of cholangiocyte signaling.

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.

journal_name

Gastroenterology

journal_title

Gastroenterology

authors

Strazzabosco M,Somlo S

doi

10.1053/j.gastro.2011.04.030

subject

Has Abstract

pub_date

2011-06-01 00:00:00

pages

1855-9, 1859.e1

issue

7

eissn

0016-5085

issn

1528-0012

pii

S0016-5085(11)00542-7

journal_volume

140

pub_type

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