The cytoplasmic dynein motor complex transports essential signals and organelles from the cell periphery to the perinuclear region, hence is critical for the survival and function of highly polarized cells such as neurons. Dynein Light Chain Roadblock-Type 1 (DYNLRB1) is thought to be an accessory subunit required for specific cargos, but here we show that it is essential for general dynein-mediated transport and sensory neuron survival. Homozygous Dynlrb1 null mice are not viable and die during early embryonic development. Furthermore, heterozygous or adult knockdown animals display reduced neuronal growth, and selective depletion of Dynlrb1 in proprioceptive neurons compromises their survival. Conditional depletion of Dynlrb1 in sensory neurons causes deficits in several signaling pathways, including β-catenin subcellular localization, and a severe impairment in the axonal transport of both lysosomes and retrograde signaling endosomes. Hence, DYNLRB1 is an essential component of the dynein complex, and given dynein's critical functions in neuronal physiology, DYNLRB1 could have a prominent role in the etiology of human neurodegenerative diseases.

中文翻译：

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DYNLRB1 对于动力蛋白介导的转运和神经元存活至关重要。

细胞质动力蛋白运动复合物将重要的信号和细胞器从细胞外围运输到核周区域，因此对于神经元等高度极化细胞的存活和功能至关重要。动力蛋白轻链路障型 1 (DYNLRB1) 被认为是特定货物所需的辅助亚基，但在这里我们表明它对于一般动力蛋白介导的运输和感觉神经元存活至关重要。纯合 Dynlrb1 缺失小鼠在早期胚胎发育过程中无法存活并死亡。此外，杂合子或成年敲除动物表现出神经元生长减少，本体感受神经元中 Dynlrb1 的选择性消耗会危及它们的生存。感觉神经元中 Dynlrb1 的条件性消耗导致几种信号通路的缺陷，包括 β-连环蛋白亚细胞定位，溶酶体和逆行信号内体的轴突运输严重受损。因此，DYNLRB1 是动力蛋白复合物的重要组成部分，鉴于动力蛋白在神经元生理学中的关键功能，DYNLRB1 可能在人类神经退行性疾病的病因学中发挥重要作用。