Document Detail


X-linked recessive inheritance of radial ray deficiencies in a family with four affected males.
MedLine Citation:
PMID:  11571552     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Radial ray deficiencies are frequently associated with additional clinical anomalies and have a heterogeneous aetiology. X-linked forms are extremely rare. We report a family in which four male relatives show bilateral absence of the radius with presence of the thumbs and associated anomalies. The segregation of the phenotype is suggestive for X-linked recessive inheritance. This is confirmed by performing linkage analysis using 24 markers spanning the X chromosome in which a maximum lod score of 1.93 for DXS8067 and DXS1001 is obtained. We defined a critical region of maximal 16.2 cM on the X chromosome with haplotype analysis.
Authors:
R J Galjaard; N Kostakoglu; J J Hoogeboom; G J Breedveld; H C van der Linde; S E Hovius; B A Oostra; L A Sandkuijl; A N Akarsu; P Heutink
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  European journal of human genetics : EJHG     Volume:  9     ISSN:  1018-4813     ISO Abbreviation:  Eur. J. Hum. Genet.     Publication Date:  2001 Sep 
Date Detail:
Created Date:  2001-09-25     Completed Date:  2001-12-04     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9302235     Medline TA:  Eur J Hum Genet     Country:  England    
Other Details:
Languages:  eng     Pagination:  653-8     Citation Subset:  IM    
Affiliation:
Department of Clinical Genetics, Erasmus University/University Hospital, Rotterdam, The Netherlands. r.j.h.galjaard@kgen.fgg.eur.nl
Data Bank Information
Bank Name/Acc. No.:
OMIM/312190;  314390
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*,  pathology
Adult
Child
Child, Preschool
DNA / genetics
Family Health
Fatal Outcome
Female
Haplotypes
Humans
Infant
Linkage (Genetics)
Lod Score
Male
Microsatellite Repeats
Pedigree
Radius / abnormalities*
Thumb / abnormalities*
X Chromosome / genetics*
Chemical
Reg. No./Substance:
9007-49-2/DNA

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


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