Experimental therapeutics in transgenic mouse models of Huntington's disease

被引：169

作者：

Beal, MF

Ferrante, RJ

机构：

[1] Cornell Univ, Weill Med Coll, Dept Neurol & Neurosci, New York, NY 10021 USA

[2] Bedford VA Med Ctr, Ctr Geriatr Res Educ & Clin, Bedford, MA USA

[3] Boston Univ, Sch Med, Dept Neurol, Boston, MA 02118 USA

[4] Boston Univ, Sch Med, Dept Pathol, Boston, MA 02118 USA

[5] Boston Univ, Sch Med, Dept Psychiat, Boston, MA 02118 USA

来源：

NATURE REVIEWS NEUROSCIENCE | 2004年 / 5卷 / 05期

基金：

美国国家卫生研究院;

关键词：

D O I：

10.1038/nrn1386

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

Despite important advances in understanding and elucidating the molecular and mechanistic pathways that mediate progression in Huntington's disease (HD), effective pharmacotherapy remains elusive. Insights into disease pathogenesis have come from studies using tissue culture, yeast, Caenorhabditis elegans, Drosophila melanogaster and transgenic mouse models. Here, we present a brief overview of HD pathogenesis and discuss the efficacy of therapeutic agents in transgenic mouse models of HD. We conclude by considering issues that affect the translation of findings in transgenic mouse models of HD to human clinical trials.

引用

页码：373 / 384

页数：12

共 141 条

[21] Altered neuronal nitric oxide synthase expression contributes to disease progression in Huntington's disease transgenic mice [J].