Filamin A (Flna) is Required for Cell-cell Contact in Vascular Development and Cardiac Morphogenesis.
From: Division of Genetics and Department of Cardiology, Children’s Hospital Boston, Boston, MA 02215, USA.
Proceedings of the National Academy of Sciences of the United States of America
- Publish Date: Dec 2006
- ISSN: 0027-8424
- Volume: 103
- Issue: 52
- Pages: 19836-41
- Medium: Print
- Language: English
- Citation (JAMA): Feng Yuanyi, Chen Ming Hui, Moskowitz Ivan P, et al. Filamin A (Flna) is Required for Cell-cell Contact in Vascular Development and Cardiac Morphogenesis.. Proc. Natl. Acad. Sci. U.S.A. Dec 2006;103:19836-41
Abstract
Mutations in the human Filamin A (FLNA) gene disrupt neuronal migration to the cerebral cortex and cause cardiovascular defects. Complete loss of Flna in mice results in embryonic lethality with severe cardiac structural defects involving ventricles, atria, and outflow tracts, as well as widespread aberrant vascular patterning. Despite these widespread developmental defects, migration and motility of many cell types does not appear to be affected. Instead, Flna-null embryos display abnormal epithelial and endothelial organization and aberrant adherens junctions in developing blood vessels, heart, brain, and other tissues. Essential roles for FLNA in intercellular junctions provide a mechanism for the diverse developmental defects seen in patients with FLNA mutations.
Mesh Headings (Keywords): Animals, Brain, Cell Communication, Cell Movement, Cells, Cultured, Contractile Proteins, Embryo, Mammalian, Endothelial Cells, Heart, Heart Failure, Mice, Mice, Transgenic, Microfilament Proteins, Mutation, Myocardium, Neural Crest
Check for Full Text / PubMed Unique Identifier (PMID): 17172441
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