Document Detail


Schöpf-Schulz-Passarge syndrome resulting from a homozygous nonsense mutation, p.Cys107X, in WNT10A.
MedLine Citation:
PMID:  21834823     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Schöpf-Schulz-Passarge syndrome (SSPS; MIM224750) is a rare autosomal recessive form of ectodermal dysplasia that was recently shown to result from mutations in the WNT10A gene. We now report a 59-year-old woman with SSPS in whom a homozygous nonsense mutation (p.Cys107X) in WNT10A was detected. Mutations in this gene may also underlie odonto-onycho-dermal dysplasia and other ectodermal dysplasia syndromes. To date, 16 different WNT10A mutations have been reported, although considerable clinical and molecular overlap exists. This report demonstrates the molecular basis of a further case of SSPS and highlights the clinical features of this unusual ectodermal dysplasia syndrome.
Authors:
Gabriela Petrof; Kenneth Fong; Joey E Lai-Cheong; Sarah E Cockayne; John A McGrath
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Publication Detail:
Type:  Journal Article     Date:  2011-06-29
Journal Detail:
Title:  The Australasian journal of dermatology     Volume:  52     ISSN:  1440-0960     ISO Abbreviation:  Australas. J. Dermatol.     Publication Date:  2011 Aug 
Date Detail:
Created Date:  2011-08-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0135232     Medline TA:  Australas J Dermatol     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  224-6     Citation Subset:  IM    
Copyright Information:
© 2011 The Authors; Australasian Journal of Dermatology © 2011 The Australasian College of Dermatologists.
Affiliation:
St John's Institute of Dermatology, King's College London, London Rupert Hallam Department of Dermatology, Royal Hallamshire Hospital, Sheffield, UK.
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