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Int. J. Mol. Sci. 2009, 10(3), 1000-1012; doi:10.3390/ijms10031000

Neuropathology and Therapeutic Intervention in Spinal and Bulbar Muscular Atrophy

1
Department of Neurology, Nagoya University Graduate School of Medicine / 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan
2
Institute for Advanced Research, Nagoya University / Furo-cho, Chikusa-ku, Nagoya 464-8601, Japan
*
Authors to whom correspondence should be addressed.
Received: 26 January 2009 / Revised: 6 March 2009 / Accepted: 9 March 2009 / Published: 10 March 2009
(This article belongs to the Special Issue Advances in Molecular Neuropathology)
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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. View Full-Text
Keywords: spinal and bulbar muscular atrophy (SBMA); polyglutamine; androgen receptor (AR); leuprorelin acetate spinal and bulbar muscular atrophy (SBMA); polyglutamine; androgen receptor (AR); leuprorelin acetate
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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MDPI and ACS Style

Banno, H.; Katsuno, M.; Suzuki, K.; Tanaka, F.; Sobue, G. Neuropathology and Therapeutic Intervention in Spinal and Bulbar Muscular Atrophy. Int. J. Mol. Sci. 2009, 10, 1000-1012.

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