Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the loss of upper and lower motor neurons that results in progressive paralysis and muscular atrophy. There are many molecules and genes involved in neuromuscular degeneration in ALS; among these, matrix metalloproteinases (MMPs). MMPs play an important role in the pathology of ALS, and MMP-1, 2, 3, and 9 might serve as disease progression markers. Tissue inhibitors of metalloproteinases (TIMPS) might also function as progression markers in ALS because they participate in regulating the proteolytic activity of MMPs. Moreover, a diversity of genes also plays a role in the pathogenesis of ALS; most MMPs-coding genes present variants related to the pathological proteolytic activity. This short review, however, will focus on the role of matrix metalloproteinases in ALS.

中文翻译：

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肌萎缩侧索硬化中基质金属蛋白酶的失调

肌萎缩侧索硬化 (ALS) 是一种神经退行性疾病，其特征是上、下运动神经元的丧失，导致进行性瘫痪和肌肉萎缩。ALS 的神经肌肉变性涉及许多分子和基因；其中，基质金属蛋白酶（MMPs）。MMPs 在 ALS 的病理学中起重要作用，MMP-1、2、3 和 9 可能作为疾病进展的标志物。金属蛋白酶组织抑制剂 (TIMPS) 也可能作为 ALS 的进展标志物，因为它们参与调节 MMP 的蛋白水解活性。此外，多种基因也在 ALS 的发病机制中发挥作用。大多数 MMPs 编码基因存在与病理蛋白水解活性相关的变异。然而，这篇简短的综述将重点关注基质金属蛋白酶在 ALS 中的作用。