Cytoskeleton

Eduardo E. Benarroch
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Abstract

The cytoskeleton consists primarily of microfilaments, microtubules, and intermediate filaments. Actin microfilaments have major role in growth, maintenance, and dynamic changes of growth cones and dendrites; stabilization of proteins at specific membrane locations; and vesicle dynamics during endocytosis and exocytosis. Microtubules provide the major tracks for intracellular transport and local cues for positioning of mitochondria and other organelles. The intermediate filaments in neurons are the neurofilaments that have a major role in regulating axonal caliber and mechanical stability. Glial fibrillary acid protein is a primary component of intermediate filaments in astrocytes. Nuclear lamins participate in regulation of the chromatin organization, trafficking of transcription factors across the nuclear envelope, and transduction of mechanical signals. Mutations affecting these cytoskeletal proteins produce a wide range of neurologic disorders, including neurodevelopmental disorders, peripheral neuropathies, myopathies, and leukodystrophy. All components of the cytoskeleton are involved in adult-onset neurodegenerative disorders.
细胞骨架
细胞骨架主要由微丝、微管和中间丝组成。肌动蛋白微丝在生长球果和树突的生长、维持和动态变化中起重要作用;蛋白质在特定膜位置的稳定;以及胞吞和胞吐过程中的囊泡动力学。微管为细胞内运输提供了主要途径,并为线粒体和其他细胞器的定位提供了局部线索。神经元中的中间丝是在调节轴突直径和机械稳定性中起主要作用的神经丝。胶质原纤维酸蛋白是星形胶质细胞中间纤维的主要成分。核层蛋白参与染色质组织的调节,转录因子在核膜上的运输,以及机械信号的转导。影响这些细胞骨架蛋白的突变产生广泛的神经系统疾病,包括神经发育障碍、周围神经病变、肌病和白质营养不良。细胞骨架的所有组成部分都与成人发病的神经退行性疾病有关。
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