Enhanced aggregation of androgen receptor in induced pluripotent stem cell-derived neurons from spinal and bulbar muscular atrophy.

doi:10.1074/jbc.M112.408211

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Article

Enhanced aggregation of androgen receptor in induced pluripotent stem cell-derived neurons from spinal and bulbar muscular atrophy.

The Journal of biological chemistry - 22 Mar 2013

Nihei Yoshihiro, Ito Daisuke, Okada Yohei, Akamatsu Wado, Yagi Takuya, Yoshizaki Takahito, Okano Hideyuki, Suzuki Norihiro

Abstract excerpt

Spinal and bulbar muscular atrophy (SBMA) is an X-linked motor neuron disease caused by a CAG repeat expansion in the androgen receptor (AR) gene. Ligand-dependent nuclear accumulation of mutant AR protein is a critical characteristic of the pathogenesis of SBMA. SBMA has been modeled in AR-overexpressing animals, but precisely how the polyglutamine (polyQ) expansion leads to neurodegeneration is unclear. Induced...

Topics

Aged, 80 and over
Androgens
Base Sequence
Benzoquinones
Cell Differentiation
Cells, Cultured
Dihydrotestosterone
Gene Expression
Gene Expression Regulation
HSP90 Heat-Shock Proteins

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Humans

Induced Pluripotent Stem Cells

Lactams, Macrocyclic

Male

Muscular Disorders, Atrophic

Mutation

Nerve Tissue Proteins

Neurons

Source

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Identifiers

PMID: 23364790
DOI: 10.1074/jbc.M112.408211
PMCID: PMC3605623

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