Document Detail


Molecular pathology of the fragile X syndrome.
MedLine Citation:
PMID:  8239933     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fragile X syndrome is the most common form of familial mental retardation (one in 1250 males and one in 2500 females, characterized by prominent dysmorphic features, macro-orchidism, and varying degrees of mental retardation. Diagnosis of this syndrome has relied on cytogenetic demonstration of the fragile site at position Xq27.3. A gene associated with the fragile X syndrome, FMR-1, has been isolated and mapped to the region of the X chromosome that corresponds to the region of the fragile site. Expansion of a trinucleotide repeat, CGG, and abnormal methylation of a CpG island account for the majority of mutations identified in FMR-1. These molecular characteristics have greatly enhanced the identification of affected individuals and carriers of the premutation who were not detected cytogenetically.
Authors:
G J Tsongalis; L M Silverman
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Archives of pathology & laboratory medicine     Volume:  117     ISSN:  0003-9985     ISO Abbreviation:  Arch. Pathol. Lab. Med.     Publication Date:  1993 Nov 
Date Detail:
Created Date:  1993-12-07     Completed Date:  1993-12-07     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7607091     Medline TA:  Arch Pathol Lab Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1121-5     Citation Subset:  AIM; IM    
Affiliation:
Department of Hospital Laboratories and Pathology, University of North Carolina Hospitals, Chapel Hill.
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MeSH Terms
Descriptor/Qualifier:
Blotting, Southern
Cytogenetics
Female
Fragile X Syndrome / diagnosis,  genetics*
Gene Expression
Humans
Male
Mutation
Pedigree

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


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