| Fibulin-1 is required for morphogenesis of neural crest-derived structures. | |
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MedLine Citation:
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PMID: 18538758 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Here we report that mouse embryos homozygous for a gene trap insertion in the fibulin-1 (Fbln1) gene are deficient in Fbln1 and exhibit cardiac ventricular wall thinning and ventricular septal defects with double outlet right ventricle or overriding aorta. Fbln1 nulls also display anomalies of aortic arch arteries, hypoplasia of the thymus and thyroid, underdeveloped skull bones, malformations of cranial nerves and hemorrhagic blood vessels in the head and neck. The spectrum of malformations is consistent with Fbln1 influencing neural crest cell (NCC)-dependent development of these tissues. This is supported by evidence that Fbln1 expression is associated with streams of cranial NCCs migrating adjacent to rhombomeres 2-7 and that Fbln1-deficient embryos display patterning anomalies of NCCs forming cranial nerves IX and X, which derive from rhombomeres 6 and 7. Additionally, Fbln1-deficient embryos show increased apoptosis in areas populated by NCCs derived from rhombomeres 4, 6 and 7. Based on these findings, it is concluded that Fbln1 is required for the directed migration and survival of cranial NCCs contributing to the development of pharyngeal glands, craniofacial skeleton, cranial nerves, aortic arch arteries, cardiac outflow tract and cephalic blood vessels. |
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Authors:
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Marion A Cooley; Christine B Kern; Victor M Fresco; Andy Wessels; Robert P Thompson; Tim C McQuinn; Waleed O Twal; Corey H Mjaatvedt; Christopher J Drake; W Scott Argraves |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2008-05-03 |
Journal Detail:
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Title: Developmental biology Volume: 319 ISSN: 1095-564X ISO Abbreviation: Dev. Biol. Publication Date: 2008 Jul |
Date Detail:
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Created Date: 2008-07-08 Completed Date: 2008-08-12 Revised Date: 2010-12-03 |
Medline Journal Info:
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Nlm Unique ID: 0372762 Medline TA: Dev Biol Country: United States |
Other Details:
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Languages: eng Pagination: 336-45 Citation Subset: IM |
Affiliation:
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Department of Cell Biology and Anatomy, Medical University of South Carolina, 173 Ashley Avenue, Charleston, SC 29425, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Antigens, CD4 / genetics Calcium-Binding Proteins / deficiency, genetics* Cerebrovascular Circulation / genetics Chromosome Mapping Crosses, Genetic Endoplasmic Reticulum / physiology Fetal Heart / pathology, physiology Genotype Heart Ventricles / embryology, pathology Immunohistochemistry Mice Mice, Knockout Morphogenesis / physiology* Neural Crest / physiology* Polymerase Chain Reaction beta-Galactosidase / genetics |
| Grant Support | |
ID/Acronym/Agency:
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HL080168/HL/NHLBI NIH HHS; HL52813/HL/NHLBI NIH HHS; P01 HL052813-060010/HL/NHLBI NIH HHS; P01 HL052813-070010/HL/NHLBI NIH HHS; P01 HL052813-080010/HL/NHLBI NIH HHS; P01 HL052813-08S10010/HL/NHLBI NIH HHS; P01 HL052813-090010/HL/NHLBI NIH HHS; P01 HL052813-100010/HL/NHLBI NIH HHS; RR16434/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Antigens, CD4; 0/Calcium-Binding Proteins; 0/fibulin; EC 3.2.1.23/beta-Galactosidase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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