Document Detail


Periventricular heterotopia in fragile X syndrome.
MedLine Citation:
PMID:  16924033     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The authors describe two unrelated individuals with fragile X syndrome (FXS) due to marked expansion and instability of the CGG trinucleotide repeats within the fragile X mental retardation 1 gene (FMR1) and periventricular heterotopia (PH). This observation suggests that the FMR1 gene is involved in neuronal migration and that abnormal neuronal migration, even beyond the resolution of MRI, contributes to the neurologic phenotype of FXS.
Authors:
F Moro; T Pisano; B Dalla Bernardina; R Polli; A Murgia; L Zoccante; F Darra; A Battaglia; T Pramparo; O Zuffardi; R Guerrini
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurology     Volume:  67     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2006 Aug 
Date Detail:
Created Date:  2006-08-22     Completed Date:  2006-09-13     Revised Date:  2011-11-24    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  713-5     Citation Subset:  AIM; IM    
Affiliation:
IRCCS Fondazione Stella Maris, Pisa, Italy.
Data Bank Information
Bank Name/Acc. No.:
OMIM/309550
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Brain Diseases / pathology*
Cerebral Ventricles / pathology*
Child, Preschool
Fragile X Mental Retardation Protein / genetics*
Fragile X Syndrome / genetics*,  pathology*
Genetic Predisposition to Disease / genetics
Humans
Male
Grant Support
ID/Acronym/Agency:
GGP05177//Telethon
Chemical
Reg. No./Substance:
0/FMR1 protein, human; 139135-51-6/Fragile X Mental Retardation Protein

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


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