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Abstract：
Neurons are long-lived and highly polarized cells that depend on autophagy to maintain cellular homeostasis. The robust, constitutive biogenesis of autophagosomes in the distal axon occurs via a conserved pathway that is required to maintain functional synapses and prevent axon degeneration. Autophagosomes are formed de novo at the axon terminal in a stepwise assembly process, engulfing mitochondrial fragments, aggregated proteins, and bulk cytosol in what appears to be a nonselective uptake mechanism. Following formation, autophagosomes fuse with late endosomes/lysosomes and then are rapidly and efficiently transported along the axon toward the soma, driven by the microtubule motor cytoplasmic dynein. Motile autophagosomes mature to autolysosomes in transit by fusing with additional late endosomes/lysosomes, arriving at the soma as fully competent degradative organelles. Misregulation of neuronal autophagy leads to axonal degeneration and synaptic destabilization, and has been implicated in neurodegenerative diseases including Alzheimer\'s disease, Parkinson\'s disease, Huntington\'s disease, and ALS.
摘要：
神经元是长寿命且高度极化的细胞，依赖于自噬来维持细胞稳态。健壮的，远端轴突中自噬体的组成型生物发生是通过保守途径发生的，该途径是维持功能性突触和防止轴突变性所必需的。自噬体在轴突末端以逐步组装过程从头形成，吞噬线粒体碎片，聚集的蛋白质，和大量的细胞质似乎是一种非选择性的摄取机制。编队之后,自噬体与晚期内体/溶酶体融合，然后沿着轴突快速有效地转运到胞体，由微管运动细胞质动力蛋白驱动。运动性自噬体通过与其他晚期内体/溶酶体融合而在运输中成熟为自溶酶体，作为完全胜任的降解细胞器到达索马。神经元自噬的失调导致轴突变性和突触不稳定,并与神经退行性疾病有关，包括阿尔茨海默病，帕金森病,亨廷顿病,和ALS。
