Document Detail


Fluorescent in situ hybridisation (FISH) for hemizygous deletion at the elastin locus in patients with isolated supravalvular aortic stenosis.
MedLine Citation:
PMID:  9138154     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Both Williams syndrome and isolated supravalvular aortic stenosis (SVAS) are caused by mutations at the elastin locus. Deletion demonstrable by FISH is the hallmark of Williams syndrome, whereas the mutations reported so far in SVAS have been more subtle. FISH positive elastin hemizygosity has not been reported in isolated SVAS. This report records our experience of FISH for elastin deletion in isolated SVAS and specifically reports a patient with non-Williams related SVAS, positive for the elastin deletion by FISH.
Authors:
H Fryssira; R Palmer; K A Hallidie-Smith; J Taylor; D Donnai; W Reardon
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of medical genetics     Volume:  34     ISSN:  0022-2593     ISO Abbreviation:  J. Med. Genet.     Publication Date:  1997 Apr 
Date Detail:
Created Date:  1997-07-29     Completed Date:  1997-07-29     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  2985087R     Medline TA:  J Med Genet     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  306-8     Citation Subset:  IM    
Affiliation:
Mothercare Unit of Clinical Genetics and Fetal Medicine, Institute of Child Health, London, UK.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Alleles
Aortic Valve Stenosis / complications,  genetics*
Elastin / genetics*
Humans
In Situ Hybridization, Fluorescence
Male
Williams Syndrome / complications,  genetics
Chemical
Reg. No./Substance:
9007-58-3/Elastin
Comments/Corrections

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