Document Detail


Transcription factor GATA3 and the human HDR syndrome.
MedLine Citation:
PMID:  11577985     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Recently, a member of the GATA-binding family of transcription factors was shown to be involved in the human hypoparathyroidism, sensorineural deafness and renal anomalies (HDR) syndrome. Deletion-mapping studies and subsequent mutation analysis revealed that haploinsufficiency for GATA3 is the underlying mechanism of the HDR syndrome. Here we discuss the clinical characteristics of the HDR syndrome and present an overview of the role of GATA3 and related GATA-binding transcription factors during vertebrate embryonic development and their involvement in human disease.
Authors:
H Van Esch; K Devriendt
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Cellular and molecular life sciences : CMLS     Volume:  58     ISSN:  1420-682X     ISO Abbreviation:  Cell. Mol. Life Sci.     Publication Date:  2001 Aug 
Date Detail:
Created Date:  2001-10-01     Completed Date:  2001-10-18     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  9705402     Medline TA:  Cell Mol Life Sci     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  1296-300     Citation Subset:  IM    
Affiliation:
Centre for Human Genetics, University of Leuven, Belgium.
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MeSH Terms
Descriptor/Qualifier:
Animals
Chromosome Mapping
Chromosomes, Human, Pair 10*
DNA-Binding Proteins / genetics*
Deafness / genetics
Female
GATA3 Transcription Factor
Hearing Loss, Sensorineural / genetics*
Humans
Hypoparathyroidism / genetics*
Kidney / abnormalities*
Male
Mice
Pedigree
Syndrome
Trans-Activators / genetics*
Transcription Factors / genetics*
Zinc Fingers
Chemical
Reg. No./Substance:
0/DNA-Binding Proteins; 0/GATA3 Transcription Factor; 0/GATA3 protein, human; 0/Gata3 protein, mouse; 0/Trans-Activators; 0/Transcription Factors

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


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