title: Molecular determinants of neural crest migration.
abstract: Normal septation of the cardiac outflow tract requires migration of neural crest cells from the posterior rhombencephalon to the branchial arches and developing conotruncal endocardial cushions. Proper migration of these cells is mediated by a variety of molecular cues. Adhesion molecules, such as integrins, are involved in the interaction of neural crest cells with the extracellular matrix, while cadherins allow neural crest cells to interact with each other during their migration. Pax3 appears to be important for proliferation of neural crest precursors, and connexin-43-mediated gap junction communication influences the rate of migration. Endothelin and its receptors are required for normal postmigratory differentiation. Platelet-derived growth factor and retinoic acid have roles in neural crest migration and differentiation as well. Finally, the similarity between the cardiovascular malformations seen in the DiGeorge and 22q11 deletion syndromes and animal models of neural crest deficiency has led to the examination of the role of genes located near or within the DiGeorge critical region in neural crest migration.
authors.affiliation: Joseph Stoke's Research Institute, Children's Hospital of Philadelphia, PA 19104-4318, USA. sbaldwin@mail.med.upenn.edu
authors.forename: K L
authors.initials: KL
authors.lastname: Maschhoff
authors.forename: H S
authors.initials: HS
authors.lastname: Baldwin
mesh_terms: D000818:Animals; D001011:Aorta / Q000002:abnormalities / Q000196:embryology; D015815:Cell Adhesion Molecules / Q000502:physiology; D002465:Cell Movement / Q000235:genetics / Q000502:physiology*; D002642:Chick Embryo; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics / Q000648:ultrastructure; D018031:Connexin 43 / Q000502:physiology; D004268:DNA-Binding Proteins / Q000502:physiology; D004062:DiGeorge Syndrome / Q000196:embryology / Q000235:genetics; D019332:Endothelin-1 / Q000172:deficiency / Q000235:genetics / Q000502:physiology; D016326:Extracellular Matrix Proteins / Q000502:physiology; D017629:Gap Junctions / Q000502:physiology / Q000648:ultrastructure; D006133:Growth Substances / Q000502:physiology; D006321:Heart / Q000196:embryology; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D008817:Mice, Mutant Strains; D009432:Neural Crest / Q000166:cytology*; D009436:Neural Tube Defects / Q000235:genetics / Q000378:metabolism; D020933:Neurotrophin 3 / Q000502:physiology; D000072041:PAX3 Transcription Factor; D051761:Paired Box Transcription Factors; D017978:Receptors, Growth Factor / Q000502:physiology; D018168:Receptors, Retinoic Acid / Q000502:physiology; D014157:Transcription Factors / Q000145:classification / Q000235:genetics / Q000502:physiology
chemical_list: D015815:Cell Adhesion Molecules; D018031:Connexin 43; D004268:DNA-Binding Proteins; D019332:Endothelin-1; D016326:Extracellular Matrix Proteins; D006133:Growth Substances; D020933:Neurotrophin 3; D000072041:PAX3 Transcription Factor; C489409:PAX3 protein, human; D051761:Paired Box Transcription Factors; D017978:Receptors, Growth Factor; D018168:Receptors, Retinoic Acid; D014157:Transcription Factors; C068686:Pax3 protein, mouse