Human voluntary movement is controlled by the pyramidal motor system, a long CNS pathway comprising corticospinal and lower motor neurons. Hereditary spastic paraplegias (HSPs) are a large, genetically diverse group of inherited neurologic disorders characterized by a length-dependent distal axonopathy of the corticospinal tracts, resulting in lower limb spasticity and weakness. A range of studies are converging on alterations in the shaping of organelles, particularly the endoplasmic reticulum, as well as intracellular membrane trafficking and distribution as primary defects underlying the HSPs, with clear relevance for other long axonopathies affecting peripheral nerves and lower motor neurons.

中文翻译：

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遗传性痉挛性截瘫的细胞通路。

人的随意运动由锥体运动系统控制，锥体运动系统是一种长的 CNS 通路，包括皮质脊髓和下运动神经元。遗传性痉挛性截瘫 (HSP) 是一大类遗传性神经系统疾病，其特征是长度依赖的皮质脊髓束远端轴突病，导致下肢痉挛和无力。一系列研究集中在细胞器的形状改变上，特别是内质网，以及作为 HSP 基础缺陷的细胞内膜运输和分布，与影响周围神经和下运动神经元的其他长轴突病明显相关。