ArticleOrganogenesis
Loss of cilia causes embryonic lung hypoplasia, liver fibrosis, and cholestasis in the talpid3 ciliopathy mutant.
Article in Organogenesis. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 36 citations in OpenAlex.
- Amyloid precursor protein as a fibrosis marker in infants with biliary atresia.Pediatric research · 2025Article
- CPLANE protein INTU regulates growth and patterning of the mouse lungs through cilia-dependent Hh signaling.Developmental biology · 2024Article
- XIAP-mediated degradation of IFT88 disrupts HSC cilia to stimulate HSC activation and liver fibrosis.EMBO reports · 2024Article
- Biallelic mutations of TTC12 and TTC21B were identified in Chinese patients with multisystem ciliopathy syndromes.Human genomics · 2022Article
- TALPID3/KIAA0586 Regulates Multiple Aspects of Neuromuscular Patterning During Gastrointestinal Development in Animal Models and Human.Frontiers in molecular neuroscience · 2021Article
- The association between methylation patterns of DNAH17 and clinicopathological factors in hepatocellular carcinoma.Cancer medicine · 2019Article
- Retinoic acid regulates avian lung branching through a molecular network.Cellular and molecular life sciences : CMLS · 2017Article
- Canonical Sonic Hedgehog Signaling in Early Lung Development.Journal of developmental biology · 2017Review
- Hepatotoxicity with Vismodegib: An MD Anderson Cancer Center and Research on Adverse Drug Events and Reports Project.Drugs in R&D · 2017Article
- Expression analysis of Shh signaling members in early stages of chick lung development.Histochemistry and cell biology · 2016Article
- Utilizing the chicken as an animal model for human craniofacial ciliopathies.Developmental biology · 2016Review
- Mutations in human homologue of chicken talpid3 gene (KIAA0586) cause a hybrid ciliopathy with overlapping features of Jeune and Joubert syndromes.Journal of medical genetics · 2015Article
- Article
- Mutations in KIAA0586 Cause Lethal Ciliopathies Ranging from a Hydrolethalus Phenotype to Short-Rib Polydactyly Syndrome.American journal of human genetics · 2015Article
- Article
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Authors and funding
5 authors at 2 institutions in 1 country.
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Abstract
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 fibrocystic disease is common and primarily characterized by ductal plate malformations which underlie the formation of liver cysts, as well as less commonly, by hepatic fibrosis, although a role for abnormal Hedgehog signal transduction has not been implicated in these phenotypes. We have examined liver, lung and rib development in the talpid(3) chicken mutant, a ciliopathy model in which abnormal Hedgehog signaling is well characterized. We find that the talpid(3) phenotype closely models that of human short-rib polydactyly syndromes which are caused by the loss of cilia, and exhibit hypoplastic lungs and liver failure. Through an analysis of liver and lung development in the talpid(3) chicken, we propose that cilia in the liver are essential for the transduction of Hedgehog signaling during hepatic development. The talpid(3) chicken represents a useful resource in furthering our understanding of the pathology of ciliopathies beyond the treatment of thoracic insufficiency as well as generating insights into the role Hedgehog signaling in hepatic development.
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