Document Detail


Perspectives on craniosynostosis: sutural biology, some well-known syndromes, and some unusual syndromes.
MedLine Citation:
PMID:  19293680     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Perspectives on craniosynostosis are discussed under the following headings: sutural biology (anatomic and genetic categories of synostosis; sutures, suture systems, and types of craniosynostosis; well-known syndromes (Muenke syndrome and Pfeiffer syndrome); and unusual syndromes (thanatophoric dysplasia, Beare-Stevenson cutis gyrata syndrome, Crouzonodermoskeletal syndrome, Carpenter syndrome, Elejalde syndrome, hypomandibular faciocranial syndrome, and craniorhiny). Five of these syndromes are caused by fibroblast growth factor receptor (FGFR) mutations; one is caused by ras-like in rat brain 23 (RAB23) mutations; and three have Mendelian patterns of inheritance, but the molecular basis remains unknown to date.
Authors:
M Michael Cohen
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of craniofacial surgery     Volume:  20 Suppl 1     ISSN:  1536-3732     ISO Abbreviation:  J Craniofac Surg     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-03-18     Completed Date:  2009-10-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9010410     Medline TA:  J Craniofac Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  646-51     Citation Subset:  D    
Affiliation:
Department of Pediatrics, Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada. michael.cohen@dal.ca
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MeSH Terms
Descriptor/Qualifier:
Cranial Sutures / anatomy & histology,  pathology
Craniosynostoses / classification,  genetics*,  pathology*
Genes, Dominant
Genes, Recessive
Humans
Mutation
Polymorphism, Single Nucleotide
Receptors, Fibroblast Growth Factor / genetics
Syndrome
rab GTP-Binding Proteins / genetics
Chemical
Reg. No./Substance:
0/Receptors, Fibroblast Growth Factor; EC 3.6.1.-/RAB23 protein, human; EC 3.6.1.-/rab GTP-Binding Proteins
Comments/Corrections
Erratum In:
J Craniofac Surg. 2009 Sep;20(5):1629-30

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