Primary cilia as dynamic and diverse signalling hubs in development and disease
Primary cilia, antenna-like sensory organelles protruding from the surface of most vertebrate
cell types, are essential for regulating signalling pathways during development and adult …
cell types, are essential for regulating signalling pathways during development and adult …
Cholangiocyte pathobiology
JM Banales, RC Huebert, T Karlsen… - Nature Reviews …, 2019 - nature.com
Cholangiocytes, the epithelial cells lining the intrahepatic and extrahepatic bile ducts, are
highly specialized cells residing in a complex anatomic niche where they participate in bile …
highly specialized cells residing in a complex anatomic niche where they participate in bile …
[PDF][PDF] The ascending pathophysiology of cholestatic liver disease
In this review we develop the argument that cholestatic liver diseases, particularly primary
biliary cholangitis and primary sclerosing cholangitis (PSC), evolve over time with …
biliary cholangitis and primary sclerosing cholangitis (PSC), evolve over time with …
When cilia go bad: cilia defects and ciliopathies
M Fliegauf, T Benzing, H Omran - Nature reviews Molecular cell biology, 2007 - nature.com
Defects in the function of cellular organelles such as peroxisomes, lysosomes and
mitochondria are well-known causes of human diseases. Recently, another organelle has …
mitochondria are well-known causes of human diseases. Recently, another organelle has …
The primary cilium at a glance
P Satir, LB Pedersen… - Journal of cell …, 2010 - journals.biologists.com
The primary cilium, which was first so named by Sergei Sorokin (Sorokin, 1968), is a solitary
organelle that emanates from the cell surface of most mammalian cell types during growth …
organelle that emanates from the cell surface of most mammalian cell types during growth …
[HTML][HTML] Primary cilium-dependent signaling mechanisms
Primary cilia are hair-like organelles and play crucial roles in vertebrate development,
organogenesis, health, and many genetic disorders. A primary cilium is a mechano-sensory …
organogenesis, health, and many genetic disorders. A primary cilium is a mechano-sensory …
[HTML][HTML] Biliary Atresia–emerging diagnostic and therapy opportunities
U Lendahl, VCH Lui, PHY Chung, PKH Tam - EBioMedicine, 2021 - thelancet.com
Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In
this review, we describe recent progress in the understanding of liver development with a …
this review, we describe recent progress in the understanding of liver development with a …
[HTML][HTML] Autosomal dominant polycystic kidney disease: the last 3 years
VE Torres, PC Harris - Kidney international, 2009 - Elsevier
Autosomal dominant polycystic kidney disease is the most prevalent, potentially lethal
monogenic disorder. It has large inter-and intra-familial variability explained to a large extent …
monogenic disorder. It has large inter-and intra-familial variability explained to a large extent …
[HTML][HTML] Physiology of cholangiocytes
JH Tabibian, AI Masyuk, TV Masyuk… - Comprehensive …, 2013 - ncbi.nlm.nih.gov
Cholangiocytes are epithelial cells that line the intra-and extrahepatic ducts of the biliary
tree. The main physiologic function of cholangiocytes is modification of hepatocyte-derived …
tree. The main physiologic function of cholangiocytes is modification of hepatocyte-derived …
[PDF][PDF] The biliary HCO3− umbrella: A unifying hypothesis on pathogenetic and therapeutic aspects of fibrosing cholangiopathies
U Beuers, S Hohenester, LJM de Buy Wenniger… - …, 2010 - Wiley Online Library
This review focuses on the hypothesis that biliary HCO secretion in humans serves to
maintain an alkaline pH near the apical surface of hepatocytes and cholangiocytes to …
maintain an alkaline pH near the apical surface of hepatocytes and cholangiocytes to …