Document Detail


Further case of Rubinstein-Taybi syndrome due to a deletion in EP300.
MedLine Citation:
PMID:  19353645     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Rubinstein-Taybi syndrome (RSTS) is a heterogeneous disorder with approximately 45-55% of patients showing mutations in the CREB binding protein and a further 3% of patients having mutations in EP300. We report a male child with a deletion of exons 3-8 of the EP300 gene who has RSTS. He has a milder skeletal phenotype, a finding that has been described in other cases with EP300 mutations. The mother suffered from pre-eclampsia and HELLP syndrome in the pregnancy. She subsequently developed a mullerian tumor of her cervix 6 years after the birth of her son.
Authors:
Patricia Foley; David Bunyan; John Stratton; Michelle Dillon; Sally Ann Lynch
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  149A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-05-04     Completed Date:  2009-06-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  997-1000     Citation Subset:  IM    
Affiliation:
Our Lady's Childrens Hospital, National Centre for Medical Genetics, Dublin, Crumlin, Ireland.
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MeSH Terms
Descriptor/Qualifier:
Child
Chromosomes, Human, Pair 22
DNA Mutational Analysis
E1A-Associated p300 Protein / genetics*
Humans
Male
Rubinstein-Taybi Syndrome / genetics*
Sequence Deletion
Chemical
Reg. No./Substance:
EC 2.3.1.48/E1A-Associated p300 Protein; EC 2.3.1.48/EP300 protein, human

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


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