Centre for Brain Repair, Dept. of Clinical Neurosciences, Cambridge, UK.
Exp Neurol. 2013 Oct;248:541-5. doi: 10.1016/j.expneurol.2013.07.015. Epub 2013 Aug 8.
The presynaptic protein α-synuclein is central to the pathogenesis of α-synucleinopathies. We show that the presence of endogenous mouse α-synuclein leads to higher number of dopaminergic neurons in the substantia nigra of wild-type C57Bl/6J mice compared with C57Bl/6S mice with a spontaneous deletion of the α-synuclein gene or C57Bl/6J mice with a targeted deletion of the α-synuclein gene. This effect of α-synuclein on dopaminergic neuron occurs during development between E10.5 and E13.5 and persists in adult life supporting the involvement of α-synuclein in the development of a subset of dopaminergic neurons.
突触前蛋白α-突触核蛋白是α-突触核蛋白病发病机制的核心。我们发现，与具有α-突触核蛋白基因自发缺失的 C57Bl/6S 小鼠或α-突触核蛋白基因靶向缺失的 C57Bl/6J 小鼠相比，内源性小鼠α-突触核蛋白的存在导致野生型 C57Bl/6J 小鼠黑质中的多巴胺能神经元数量增加。这种α-突触核蛋白对多巴胺能神经元的影响发生在 E10.5 和 E13.5 之间的发育过程中，并在成年期持续存在，支持α-突触核蛋白参与一部分多巴胺能神经元的发育。
