Document Detail


Germline mutations in HRAS proto-oncogene cause Costello syndrome.
MedLine Citation:
PMID:  16170316     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Costello syndrome is a multiple congenital anomaly and mental retardation syndrome characterized by coarse face, loose skin, cardiomyopathy and predisposition to tumors. We identified four heterozygous de novo mutations of HRAS in 12 of 13 affected individuals, all of which were previously reported as somatic and oncogenic mutations in various tumors. Our observations suggest that germline mutations in HRAS perturb human development and increase susceptibility to tumors.
Authors:
Yoko Aoki; Tetsuya Niihori; Hiroshi Kawame; Kenji Kurosawa; Hirofumi Ohashi; Yukichi Tanaka; Mirella Filocamo; Kumi Kato; Yoichi Suzuki; Shigeo Kure; Yoichi Matsubara
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2005-09-18
Journal Detail:
Title:  Nature genetics     Volume:  37     ISSN:  1061-4036     ISO Abbreviation:  Nat. Genet.     Publication Date:  2005 Oct 
Date Detail:
Created Date:  2005-09-30     Completed Date:  2005-12-20     Revised Date:  2011-11-24    
Medline Journal Info:
Nlm Unique ID:  9216904     Medline TA:  Nat Genet     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1038-40     Citation Subset:  IM    
Affiliation:
Department of Medical Genetics, Tohoku University School of Medicine, 1-1 Seiryo-machi, Sendai 980-8574, Japan. aokiy@mail.tains.tohoku.ac.jp
Data Bank Information
Bank Name/Acc. No.:
OMIM/163950;  218040; RefSeq/NC_000001;  NC_000011;  NC_000012;  NC_000023;  NM_005343
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Amino Acid Sequence
Cardiomyopathies / genetics
Face / abnormalities
Female
Genetic Predisposition to Disease*
Germ-Line Mutation*
Humans
Intellectual Disability / genetics*
Molecular Sequence Data
Neoplasms / genetics
Proto-Oncogene Proteins p21(ras) / genetics*
Skin Abnormalities / genetics
Syndrome
Grant Support
ID/Acronym/Agency:
GTF04002//Telethon
Chemical
Reg. No./Substance:
EC 3.6.5.2/HRAS protein, human; EC 3.6.5.2/Proto-Oncogene Proteins p21(ras)

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


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