Document Detail


SOS1: a new player in the Noonan-like/multiple giant cell lesion syndrome.
MedLine Citation:
PMID:  19438935     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Noonan-like/multiple giant cell lesion syndrome is a rare condition with phenotypic overlap with Noonan syndrome (NS) and cherubism. PTPN11 gene mutations were described in several individuals with this phenotype, and it is recently considered as a variant phenotype of NS. Gain-of-function mutations in the SOS1 gene were recently described as the second major cause of NS. Here, we report for the first time the involvement of SOS1 gene in a family with the Noonan-like/multiple giant cell lesion phenotype.
Authors:
N Hanna; B Parfait; I M Talaat; M Vidaud; H H Elsedfy
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-05-05
Journal Detail:
Title:  Clinical genetics     Volume:  75     ISSN:  1399-0004     ISO Abbreviation:  Clin. Genet.     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-06-10     Completed Date:  2009-10-05     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0253664     Medline TA:  Clin Genet     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  568-71     Citation Subset:  IM    
Affiliation:
INSERM U745, Faculté des Sciences Pharmaceutiques et Biologiques, Université Paris Descartes, Paris Cedex 06, France. nadine.hanna@gmail.com
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MeSH Terms
Descriptor/Qualifier:
Adaptor Proteins, Signal Transducing / genetics
Amino Acid Substitution
Cherubism / complications,  genetics,  pathology
Child
Child, Preschool
DNA / analysis,  genetics
DNA Mutational Analysis
Giant Cells / metabolism,  pathology*
Humans
Male
Mandible / pathology
Noonan Syndrome / complications,  genetics,  pathology
Point Mutation
Protein Tyrosine Phosphatase, Non-Receptor Type 11 / genetics
Pulmonary Valve Stenosis / etiology
SOS1 Protein / genetics*
Chemical
Reg. No./Substance:
0/Adaptor Proteins, Signal Transducing; 0/SH3BP2 protein, human; 0/SOS1 Protein; 9007-49-2/DNA; EC 3.1.3.48/PTPN11 protein, human; EC 3.1.3.48/Protein Tyrosine Phosphatase, Non-Receptor Type 11

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


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