SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models

SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models

Perturbation of protein homeostasis and aggregation of misfolded proteins is a major cause of many human diseases. A hallmark of the neurodegenerative disease spinocerebellar ataxia type 7 (SCA7) is the intranuclear accumulation of mutant, misfolded ataxin-7 (polyQ-ATXN7). Here, we show that endogen...

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Bibliographic Details
Main Authors: Martina Marinello, Andreas Werner, Mariagiovanna Giannone, Khadija Tahiri, Sandro Alves, Christelle Tesson, Wilfred den Dunnen, Jacob-S. Seeler, Alexis Brice, Annie Sittler
Format: Article
Language:English
Published: The Company of Biologists 2019-01-01
Series:Disease Models & Mechanisms
Subjects:
Polyglutamine disease
SCA7
SUMO
Proteasome
RNF4
Online Access:http://dmm.biologists.org/content/12/1/dmm036145

Internet

http://dmm.biologists.org/content/12/1/dmm036145

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