Nieoullon A
Université de la Méditerranée, Laboratoire de Neurobiologie Cellulaire et Fonctionnelle, UPR 9013 du CNRS, Marseille, France.
Therapie. 1998 Jan-Feb;53(1):21-9.
Neurodegenerative processes are generally characterized by the long-lasting course of neuronal death and the selectivity of the neuronal population or brain structure involved in the lesion. This is the case for Alzheimer's, Parkinson's or Huntington's diseases, or amyotrophic lateral sclerosis (ALS). The reasons for such a specificity are largely unknown as are generally the mechanisms of the diseases. One common feature of these diseases, however, is that the neuronal death is thought to involve apoptosis, at least partly. Interestingly, apoptosis in the brain would involve specific gene products similar to that identified in the nematode c. elegans, partly corresponding in mammals to ICE-related compounds and Bcl2 protein. The involvement of calcium as well as of oxydative stress mechanisms in such neuronal death is to be fully proved but putative modulation by external signals (such as those provided through trophic factors or even neurotransmitters) represents an interesting way to validate the current hypothesis of neuronal death in neurodegenerative diseases in humans.
神经退行性过程通常具有神经元死亡病程持久以及病变所涉及的神经元群体或脑结构具有选择性的特点。阿尔茨海默病、帕金森病、亨廷顿病或肌萎缩侧索硬化症（ALS）都是如此。这种特异性的原因在很大程度上尚不清楚，这些疾病的一般机制也是如此。然而，这些疾病的一个共同特征是，人们认为神经元死亡至少部分涉及细胞凋亡。有趣的是，大脑中的细胞凋亡会涉及与线虫秀丽隐杆线虫中鉴定出的类似的特定基因产物，在哺乳动物中部分对应于ICE相关化合物和Bcl2蛋白。钙以及氧化应激机制在这种神经元死亡中的作用有待充分证实，但外部信号（如通过神经营养因子甚至神经递质提供的信号）的假定调节是验证当前关于人类神经退行性疾病中神经元死亡假说的一种有趣方式。
