Neuropathology and Therapeutic Intervention in Spinal and Bulbar Muscular Atrophy

被引：5

作者：

Banno, Haruhiko ^{[1
]}

Katsuno, Masahisa ^{[1
,2
]}

Suzuki, Keisuke ^{[1
]}

Tanaka, Fumiaki ^{[1
]}

Sobue, Gen ^{[1
]}

机构：

[1] Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

[2] Nagoya Univ, Inst Adv Res, Chikusa Ku, Nagoya, Aichi 4648601, Japan

来源：

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2009年 / 10卷 / 03期

关键词：

Spinal and bulbar muscular atrophy (SBMA); polyglutamine; androgen receptor (AR); leuprorelin acetate; TRANSGENIC MOUSE MODEL; ANDROGEN RECEPTOR PROTEIN; MOTOR-NEURON DEGENERATION; RECESSIVE BULBOSPINAL NEURONOPATHY; INCLUSION-BODY FORMATION; CREB-BINDING PROTEIN; HUNTINGTONS-DISEASE; PHENOTYPIC-EXPRESSION; AXONAL-TRANSPORT; CAG REPEAT;

D O I：

10.3390/ijms10031000

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：1000 / 1012

页数：13

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