Document Detail


The role of 9qh+ in phenotypic and genotypic heterogeneity in a Van der Woude syndrome pedigree.
MedLine Citation:
PMID:  20660977     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Van der Woude syndrome (VWS) (OMIM 119300) is a dominantly inherited developmental disorder that is characterized by pits and/or sinuses of the lower lip and cleft lip and/or cleft palate. Mutations in the interferon regulatory factor 6 gene (IRF6) have been recently identified in patients with VWS, with more than 60 mutations reported. We report the phenotypic variants of the syndrome in a family and present the application of the multicolor chromosome banding (mBAND) analysis in the identification of complex intrachromosome rearrangements of chromosome 9 in a child with VWS. The authors conclude that increased heterochromatin on chromosome 9 did not have any effect on the phenotypic expression of the syndrome in the family that was studied.
Authors:
G A Moghe; M S Kaur; A M Thomas; T Raseswari; M Swapna; L Rao
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the Indian Society of Pedodontics and Preventive Dentistry     Volume:  28     ISSN:  0970-4388     ISO Abbreviation:  J Indian Soc Pedod Prev Dent     Publication Date:    2010 Apr-Jun
Date Detail:
Created Date:  2010-07-27     Completed Date:  2010-11-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8710631     Medline TA:  J Indian Soc Pedod Prev Dent     Country:  India    
Other Details:
Languages:  eng     Pagination:  104-9     Citation Subset:  D    
Affiliation:
Department of Pedodontics and Preventive Dentistry, Panineeya Institute of Dental Sciences and Research Centre, Dilsukhnagar, Hyderabad, Andhra Pradesh, India. drgayatrimoghe@gmail.com
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MeSH Terms
Descriptor/Qualifier:
Child
Chromosome Aberrations*
Chromosome Banding / methods
Chromosomes, Human, Pair 9 / genetics*
Cleft Lip / genetics
Cleft Palate / genetics
Genetic Heterogeneity
Heterochromatin
Humans
In Situ Hybridization, Fluorescence
Interferon Regulatory Factors / genetics
Karyotyping
Lip / abnormalities*
Lod Score
Male
Oral Fistula / genetics*
Pedigree
Syndrome
Chemical
Reg. No./Substance:
0/Heterochromatin; 0/Interferon Regulatory Factors

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


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