Article
Loss of cilia causes embryonic lung hypoplasia, liver fibrosis, and cholestasis in the talpid3 ciliopathy mutant.
Organogenesis - 1 Jan 2000
Davey Megan G, McTeir Lynn, Barrie Andrew M, Freem Lucy J, Stephen Louise A
Abstract excerpt
Sonic hedgehog plays an essential role in maintaining hepatoblasts in a proliferative non-differentiating state during embryogenesis. Transduction of the Hedgehog signaling pathway is dependent on the presence of functional primary cilia and hepatoblasts, therefore, must require primary cilia for normal function. In congenital syndromes in which cilia are absent or non-functional (ciliopathies) hepatorenal...
Topics
- Animals
- Biliary Tract
- Cell Cycle Proteins
- Chick Embryo
- Chickens
- Cholestasis
- Cilia
- Gene Expression Regulation, Developmental
- Hedgehog Proteins
- Humans
- Liver
- Liver Cirrhosis
- Lung
- Mutation
- Patched Receptors
- Receptors, Cell Surface
- Signal Transduction