Drosophila atrophin homolog functions as a transcriptional corepressor in multiple developmental processes.

Abstract	Dentatorubral-pallidoluysian atrophy is a progressive neurodegenerative disease caused by the expansion of a polyglutamine repeats within the Atrophin-1 protein. The in vivo function of Atrophin-1 is unknown. We have characterized a Drosophila gene encoding an Atrophin family protein. Analysis of mutant phenotypes indicates that Drosophila Atrophin is required in diverse developmental processes including early embryonic patterning. Drosophila Atrophin genetically interacts with the transcription repressor even-skipped and is required for its repressive function in vivo. Drosophila Atrophin directly binds to Even-skipped in vitro. Furthermore, both human Atrophin-1 and Drosophila Atrophin repress transcription in vivo when tethered to DNA, and poly-Q expansion in Atrophin-1 reduces this repressive activity. We propose that Atrophin proteins function as versatile transcriptional corepressors and discuss a model that deregulation of transcription may contribute to the pathogenesis of neurodegeneration.
Authors	Sheng Zhang, Lei Xu, Janet Lee, Tian Xu
Journal	Cell (Cell) Vol. 108 Issue 1 Pg. 45-56 (Jan 11 2002) ISSN: 0092-8674 [Print] United States
PMID	11792320 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References	Bacterial Proteins DNA-Binding Proteins Drosophila Proteins Hkb protein, Drosophila Homeodomain Proteins Nerve Tissue Proteins Nuclear Proteins Repressor Proteins Transcription Factors atrophin-1 eve protein, Drosophila
Topics	Animals Bacterial Proteins Body Patterning (genetics) Cloning, Molecular DNA-Binding Proteins (genetics, metabolism) Drosophila (genetics) Drosophila Proteins Gene Expression Regulation, Developmental (physiology) Homeodomain Proteins (genetics, metabolism) Nerve Tissue Proteins (genetics, metabolism) Nuclear Proteins (genetics) Phenotype Repressor Proteins (genetics, metabolism) Transcription Factors Transcriptional Activation (physiology)

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