Article
p62/SQSTM1 differentially removes the toxic mutant androgen receptor via autophagy and inclusion formation in a spinal and bulbar muscular atrophy mouse model.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 1 May 2013
Doi Hideki, Adachi Hiroaki, Katsuno Masahisa, Minamiyama Makoto, Matsumoto Shinjiro, Kondo Naohide, Miyazaki Yu, Iida Madoka, Tohnai Genki, Qiang Qiang, Tanaka Fumiaki, Yanagawa Toru, Warabi Eiji, Ishii Tetsuro, Sobue Gen
Abstract excerpt
Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders that are caused by the expansion of trinucleotide CAG repeats in the causative genes. Spinal and bulbar muscular atrophy (SBMA) is an inherited motor neuron disease that is caused by the expansion of a polyQ tract within the androgen receptor (AR). p62 is a ubiquitin- and light-chain 3-binding protein that is known to regulate the...
Topics
- Aged
- Animals
- Autophagy
- Disease Models, Animal
- Female
- Gene Expression Regulation
- Humans
- Inclusion Bodies
- Male
- Mice
