Document Detail


Founder mutation causing infantile GM1-gangliosidosis in the Gypsy population.
MedLine Citation:
PMID:  16466959     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The Gypsies are a trans-national founder population of Asian descent, whose genetic heritage is still incompletely characterized. Here, we describe the first founder mutation leading to a lysosomal storage disorder in this population: R59H in GLB1, which causes infantile GM1-gangliosidosis. The R59H carrier rate is approximately 2% in the general Gypsy population and approximately 10% in the Rudari sub-isolate. Haplotype analysis suggests that the Gypsy diaspora may have contributed to the spread of this mutation to South America.
Authors:
Ivanka Sinigerska; David Chandler; Vijesh Vaghjiani; Irfet Hassanova; Rebecca Gooding; Amelia Morrone; Ivo Kremensky; Luba Kalaydjieva
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2006-02-08
Journal Detail:
Title:  Molecular genetics and metabolism     Volume:  88     ISSN:  1096-7192     ISO Abbreviation:  Mol. Genet. Metab.     Publication Date:  2006 May 
Date Detail:
Created Date:  2006-04-24     Completed Date:  2006-06-30     Revised Date:  2007-08-13    
Medline Journal Info:
Nlm Unique ID:  9805456     Medline TA:  Mol Genet Metab     Country:  United States    
Other Details:
Languages:  eng     Pagination:  93-5     Citation Subset:  IM    
Affiliation:
Laboratory of Molecular Pathology, Medical University, Sofia, Bulgaria.
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MeSH Terms
Descriptor/Qualifier:
Founder Effect*
Gangliosidosis, GM1 / genetics*
Gypsies / genetics*
Humans
Infant
Point Mutation
beta-Galactosidase / genetics*
Grant Support
ID/Acronym/Agency:
//Wellcome Trust
Chemical
Reg. No./Substance:
EC 3.2.1.23/GLB1 protein, human; EC 3.2.1.23/beta-Galactosidase

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


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