Primary cilia as dynamic and diverse signalling hubs in development and disease

P Mill, ST Christensen, LB Pedersen - Nature Reviews Genetics, 2023 - nature.com
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 …

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 …

[PDF][PDF] The ascending pathophysiology of cholestatic liver disease

PLM Jansen, A Ghallab, N Vartak, R Reif… - …, 2017 - Wiley Online Library
In this review we develop the argument that cholestatic liver diseases, particularly primary
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 …

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 …

[HTML][HTML] Primary cilium-dependent signaling mechanisms

R Pala, N Alomari, SM Nauli - International journal of molecular sciences, 2017 - mdpi.com
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 …

[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 …

[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 …

[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 …

[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 …