Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
2011-6-6
pubmed:abstractText
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.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
1528-0012
pubmed:author
pubmed:copyrightInfo
Copyright © 2011 AGA Institute. Published by Elsevier Inc. All rights reserved.
pubmed:issnType
Electronic
pubmed:volume
140
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1855-9, 1859.e1
pubmed:meshHeading
pubmed:year
2011
pubmed:articleTitle
Polycystic liver diseases: congenital disorders of cholangiocyte signaling.
pubmed:affiliation
Liver Center and Section of Digestive Diseases, Department of Internal Medicine, School of Medicine, Yale University, New Haven, Connecticut 06520, USA. mario.strazzabosco@yale.edu
pubmed:publicationType
Journal Article, Review, Research Support, N.I.H., Extramural