Portier M M, Escurat M, Landon F, Djabali K, Bousquet O
Collège de France, Laboratoire de Biochimie Cellulaire, Paris, France.
C R Acad Sci III. 1993 Sep;316(9):1124-40.
This review focuses attention on the expression of peripherin and the low-molecular mass neurofilament protein during development, as well as on recent results concerning the roles of these neuronal proteins. Peripherin is the only type III intermediate filament that has been shown to be expressed in neurons but exclusively in motor, sensory and sympathetic neurons; moreover, it is co-expressed with neurofilament proteins (NFP). Clearly, peripherin is expressed concomitantly with axonal growth during development, and its synthesis appears necessary to axonal regeneration in the adult. As to NFP, they are presumed to maintain the axonal diameter and thereby ensure a normal conduction velocity. In many neuropathies, either occurring in man or provoked by different means in animals, the neurofilament network is disrupted thus giving rise to bundles of filaments in perikarya or along axons; consequently, the axonal transport is impaired. The possible significance of the overexpression of NFP is discussed.
本综述重点关注外周蛋白和低分子量神经丝蛋白在发育过程中的表达，以及关于这些神经元蛋白作用的最新研究结果。外周蛋白是唯一已被证明在神经元中表达但仅在运动、感觉和交感神经元中表达的III型中间丝；此外，它与神经丝蛋白（NFP）共同表达。显然，外周蛋白在发育过程中与轴突生长同时表达，其合成似乎是成年后轴突再生所必需的。至于神经丝蛋白，它们被认为可维持轴突直径，从而确保正常的传导速度。在许多人类发生的或通过不同方式在动物中诱发的神经病变中，神经丝网络遭到破坏，从而导致核周体或沿轴突出现丝束；因此，轴突运输受损。文中讨论了神经丝蛋白过度表达的可能意义。
