Lysosomal physiology.
Lysosomal physiology.
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DOI：
10.1146/annurev-physiol-021014-071649
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发表时间：
2015
影响因子：
18.2
通讯作者：
Ren D
中科院分区：
文献类型：
--
作者：
Xu H;Ren D
溶酶体是充满60多种不同类型的水解酶的酸性隔间。它们调节内吞作用产生的胞外颗粒和自噬作用产生的细胞内成分的降解。消化后的产物通过特定的分解代谢物输出体或囊泡膜转运出溶酶体。溶酶体还含有50多种膜蛋白，并配备了检测营养可获得性的机制，该机制决定了溶酶体的分布、数量、大小和活性，以控制货物流量的特异性和降解的时间(开始和结束)。降解、输出或运输方面的缺陷会导致溶酶体功能障碍和溶酶体储存病(LSD)。溶酶体通道和转运体通过调节周膜离子流量来调节溶酶体离子动态平衡、膜电位、分解代谢产物输出、膜转运和营养感知。溶酶体通道的失调是许多LSD的基础，也可能是代谢性和常见的神经退行性疾病的基础。
Lysosomes are acidic compartments filled with more than 60 different types of hydrolases. They mediate the degradation of extracellular particles from endocytosis and of intracellular components from autophagy. The digested products are transported out of the lysosome via specific catabolite exporters or via vesicular membrane trafficking. Lysosomes also contain more than 50 membrane proteins and are equipped with the machinery to sense nutrient availability, which determines the distribution, number, size, and activity of lysosomes to control the specificity of cargo flux and timing (the initiation and termination) of degradation. Defects in degradation, export, or trafficking result in lysosomal dysfunction and lysosomal storage diseases (LSDs). Lysosomal channels and transporters mediate ion flux across perimeter membranes to regulate lysosomal ion homeostasis, membrane potential, catabolite export, membrane trafficking, and nutrient sensing. Dysregulation of lysosomal channels underlies the pathogenesis of many LSDs and possibly that of metabolic and common neurodegenerative diseases.