Document Detail


Recurrent aberrations identified by array-CGH in patients with Mayer-Rokitansky-Küster-Hauser syndrome.
MedLine Citation:
PMID:  20797712     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To identify genetic causes of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.
DESIGN: Prospective laboratory study.
SETTING: University hospital.
PATIENT(S): Fifty-six patients with MRKH syndrome.
INTERVENTION(S): Identification of microdeletions and -duplications in a group of 48 MRKH patients by array-CGH. Results obtained by array-CGH were confirmed by RT-qPCR. Sequential analysis of two candidate genes LHX1 and HNF1B in a group of 56 MRKH patients.
MAIN OUTCOME MEASURE(S): Identification of chromosomal regions and genes (recurrent and private) associated with MRKH syndrome.
RESULT(S): We could delineate three definitively relevant regions (1q21.1, 17q12, and 22q11.21) and suggest that LHX1 und HNF1B are candidate genes for MRKH syndrome, because we identified recurrent deletions affecting these genes and a possible causative missense mutation in LHX1.
CONCLUSION(S): Our findings suggest that different chromosomal regions are associated with MRKH syndrome.
Authors:
Susanne Ledig; Cordula Schippert; Reiner Strick; Matthias W Beckmann; Patricia G Oppelt; Peter Wieacker
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-08-24
Journal Detail:
Title:  Fertility and sterility     Volume:  95     ISSN:  1556-5653     ISO Abbreviation:  Fertil. Steril.     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-03-22     Completed Date:  2011-05-11     Revised Date:  2011-12-06    
Medline Journal Info:
Nlm Unique ID:  0372772     Medline TA:  Fertil Steril     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1589-94     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.
Affiliation:
Institut für Humangenetik, Westfälische Wilhelms-Universität, Münster, Germany. sledig@uni-muenster.de
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MeSH Terms
Descriptor/Qualifier:
46, XX Disorders of Sex Development* / genetics
Abnormalities, Multiple* / genetics
Case-Control Studies
Chromosome Aberrations* / statistics & numerical data
Chromosomes, Human, Pair 1 / genetics
Chromosomes, Human, Pair 17 / genetics
Chromosomes, Human, Pair 22 / genetics
Cohort Studies
Comparative Genomic Hybridization*
Female
Gene Frequency
Genetic Association Studies
Humans
Uterus / abnormalities
Vagina / abnormalities

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


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