3-硝基丙酸作为研究亨廷顿病发病机制的工具：过去、现在和未来。

Departamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Maimónides de Investigaciones Biomédicas de Córdoba, Universidad de Córdoba, Av. Menéndez Pidal s/n, 14004 Córdoba, Spain.

Molecules. 2010 Feb 10;15(2):878-916. doi: 10.3390/molecules15020878.

Huntington's disease (HD) is an inheritable autosomal-dominant disorder whose causal mechanisms remain unknown. Experimental models have begun to uncover these pathways, thus helping to understand the mechanisms implicated and allowing for the characterization of potential targets for new therapeutic strategies. 3-Nitropropionic acid is known to produce in animals behavioural, biochemical and morphologic changes similar to those occurring in HD. For this reason, this phenotypic model is gaining attention as a valuable tool to mimick this disorder and further developing new therapies. In this review, we will focus on the past and present research of this molecule, to finally bring a perspective on what will be next in this promising field of study.

亨廷顿病（HD）是一种可遗传的常染色体显性疾病，其病因机制尚不清楚。实验模型已开始揭示这些途径，从而有助于了解所涉及的机制，并为新的治疗策略的潜在靶点的特征提供依据。3-硝基丙酸已知会在动物中产生类似亨廷顿病的行为、生化和形态变化。出于这个原因，这种表型模型作为模拟这种疾病和进一步开发新疗法的有价值工具受到了关注。在这篇综述中，我们将重点介绍该分子过去和现在的研究，最后对这个充满希望的研究领域的未来发展提出看法。