Bousquet O, Portier M M
Laboratoire de Biochimie Cellulaire, Collège de France, Paris, France.
C R Seances Soc Biol Fil. 1996;190(2-3):269-87.
The characteristics of the seven intermediate filament proteins expressed during the development of neurons are reviewed. The most extensively studied have been neurofilament proteins (NFP) and peripherin. The relative plasticity of the peripherin network can account for its possible role during development when the axons of the neurons in which this protein is expressed have to find their targets crossing a non-neural environment probably in answer to signals from this environment and from their respective targets. Peripherin may assume a similar role when axons regenerate. NFP are considered as maintaining the axonal caliber, thus ensuring a normal axonal transport. Their network is highly disrupted in several diseases, particularly in motor neuron diseases. Mice transgenic for human NFP or in which mouse NFP are overexpressed have been obtained and are considered as animal models for these diseases.
本文综述了神经元发育过程中表达的七种中间丝蛋白的特性。研究最为广泛的是神经丝蛋白（NFP）和外周蛋白。外周蛋白网络的相对可塑性可以解释其在发育过程中可能发挥的作用，此时表达这种蛋白的神经元轴突必须穿越非神经环境以找到它们的靶标，这可能是对来自该环境及其各自靶标的信号的响应。当轴突再生时，外周蛋白可能发挥类似的作用。NFP被认为可维持轴突管径，从而确保正常的轴突运输。它们的网络在几种疾病中高度紊乱，尤其是在运动神经元疾病中。已经获得了转人类NFP基因的小鼠或过表达小鼠NFP的小鼠，并将其视为这些疾病的动物模型。
