Document Detail


Exclusion of MYF5, GSC, RUNX2, and TCOF1 mutation in a case of cerebro-costo-mandibular syndrome.
MedLine Citation:
PMID:  20177378     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Cerebro-costo-mandibular syndrome (CCMS) is an uncommon multiple congenital anomaly syndrome characterized by severe micrognathia, posterior rib-gap defects, and developmental delay. The cause of CCMS is unknown. Genes hypothesized to have a causal role in CCMS, include myogenic factor 5 (MYF5), goosecoid homeobox (GSC) and runt-related transcription factor 2 (RUNX2) [formerly known as core-binding factor (CBFA1)]. We report an infant with typical features of CCMS who, on prenatal ultrasound, was found to have severe micrognathia. We present the first image by three-dimensional computed tomography of posterior rib-defect, and we exclude mutations of the MYF5, GSC, RUNX2, and TCOF1 genes in our patient. Further molecular studies are needed to evaluate the cause of CCMS.
Authors:
Pen-Hua Su; Jia-Yuh Chen; Chin-Lung Chiang; Yan-Yan Ng; Suh-Jen Chen
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical dysmorphology     Volume:  19     ISSN:  1473-5717     ISO Abbreviation:  Clin. Dysmorphol.     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-03-10     Completed Date:  2010-05-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9207893     Medline TA:  Clin Dysmorphol     Country:  England    
Other Details:
Languages:  eng     Pagination:  51-5     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*,  radiography
Adult
Core Binding Factor Alpha 1 Subunit / genetics*
Craniofacial Abnormalities / genetics*,  radiography
Female
Goosecoid Protein / genetics*
Humans
Infant
Infant, Newborn
Male
Mutation / genetics*
Myogenic Regulatory Factor 5 / genetics*
Nuclear Proteins / genetics*
Phosphoproteins / genetics*
Pregnancy
Radiography, Thoracic
Skull / radiography
Syndrome
Chemical
Reg. No./Substance:
0/Core Binding Factor Alpha 1 Subunit; 0/GSC protein, human; 0/Goosecoid Protein; 0/MYF5 protein, human; 0/Myogenic Regulatory Factor 5; 0/Nuclear Proteins; 0/Phosphoproteins; 0/RUNX2 protein, human; 0/TCOF1 protein, human

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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