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Neuropathology and therapeutic intervention in spinal and bulbar muscular atrophy.

Authors
  • Banno, Haruhiko
  • Katsuno, Masahisa
  • Suzuki, Keisuke
  • Tanaka, Fumiaki
  • Sobue, Gen
Type
Published Article
Journal
International Journal of Molecular Sciences
Publisher
MDPI AG
Publication Date
Mar 01, 2009
Volume
10
Issue
3
Pages
1000–1012
Identifiers
PMID: 19399234
Source
Medline
Keywords
License
Unknown

Abstract

Spinal and bulbar muscular atrophy (SBMA) is a hereditary motor neuron disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR). The histopathological finding in SBMA is loss of lower motor neurons in the anterior horn of the spinal cord as well as in the brainstem motor nuclei. Animal studies have revealed that the pathogenesis of SBMA depends on the level of serum testosterone, and that androgen deprivation mitigates neurodegeneration through inhibition of nuclear accumulation of the pathogenic AR. Heat shock proteins, ubiquitin-proteasome system and transcriptional regulation are also potential targets of therapy development for SBMA.

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