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Enhanced SUMOylation in polyglutamine diseases.

Abstract：

:Small ubiquitin-like modifiers (SUMOs) are proteins homologous to ubiquitin that possibly regulate intranuclear protein localization, nuclear transport, and ubiquitination. We examined patients of DRPLA, SCA1, MJD, and Huntington's disease and found that neurons in affected regions of the brain react strongly to SUMO-1, a family member of SUMOs. Western blot with a transgenic mouse expressing mutant ataxin-1 showed the increase of SUMOylated proteins in the cerebellar cortex, which we named ESCA1 and ESCA2. These results indicated activation of SUMO-1 system in polyglutamine diseases and predicted its involvement in the pathology.

journal_name

Biochem Biophys Res Commun

journal_title

Biochemical and biophysical research communications

authors

Ueda H,Goto J,Hashida H,Lin X,Oyanagi K,Kawano H,Zoghbi HY,Kanazawa I,Okazawa H

doi

10.1016/S0006-291X(02)00211-5

subject

Has Abstract

pub_date

2002-04-26 00:00:00

pages

307-13

issue

1

eissn

0006-291X

issn

1090-2104

pii

S0006-291X(02)00211-5

journal_volume

293

pub_type

杂志文章

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