Document Detail

The neural crest in cardiac congenital anomalies.
MedLine Citation:
PMID:  22595346     Owner:  NLM     Status:  MEDLINE    
This review discusses the function of neural crest as they relate to cardiovascular defects. The cardiac neural crest cells are a subpopulation of cranial neural crest discovered nearly 30 years ago by ablation of premigratory neural crest. The cardiac neural crest cells are necessary for normal cardiovascular development. We begin with a description of the crest cells in normal development, including their function in remodeling the pharyngeal arch arteries, outflow tract septation, valvulogenesis, and development of the cardiac conduction system. The cells are also responsible for modulating signaling in the caudal pharynx, including the second heart field. Many of the molecular pathways that are known to influence specification, migration, patterning and final targeting of the cardiac neural crest cells are reviewed. The cardiac neural crest cells play a critical role in the pathogenesis of various human cardiocraniofacial syndromes such as DiGeorge, Velocardiofacial, CHARGE, Fetal Alcohol, Alagille, LEOPARD, and Noonan syndromes, as well as Retinoic Acid Embryopathy. The loss of neural crest cells or their dysfunction may not always directly cause abnormal cardiovascular development, but are involved secondarily because crest cells represent a major component in the complex tissue interactions in the head, pharynx and outflow tract. Thus many of the human syndromes linking defects in the heart, face and brain can be better understood when considered within the context of a single cardiocraniofacial developmental module with the neural crest being a key cell type that interconnects the regions.
Anna Keyte; Mary Redmond Hutson
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2012-05-15
Journal Detail:
Title:  Differentiation; research in biological diversity     Volume:  84     ISSN:  1432-0436     ISO Abbreviation:  Differentiation     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-07-03     Completed Date:  2012-11-12     Revised Date:  2013-07-03    
Medline Journal Info:
Nlm Unique ID:  0401650     Medline TA:  Differentiation     Country:  England    
Other Details:
Languages:  eng     Pagination:  25-40     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 International Society of Differentiation. Published by Elsevier B.V. All rights reserved.
Department of Pediatrics (Neonatology), Neonatal-Perinatal Research Institute, Box 103105, Duke University Medical Center, Durham, NC 27710, USA.
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MeSH Terms
Abnormalities, Multiple / etiology
Cell Differentiation*
Cell Proliferation
Craniofacial Abnormalities / etiology*
Disease Models, Animal
Heart / embryology*
Heart Defects, Congenital / etiology*
Metabolic Networks and Pathways
Myocytes, Cardiac / cytology,  metabolism
Neural Crest / cytology*,  embryology
Neurons / cytology,  metabolism
Grant Support

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