Endolysosomal calcium regulation and disease
Endolysosomal calcium regulation and disease
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DOI：
10.1042/bst0381458
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发表时间：
2010-12-01
影响因子：
3.9
通讯作者：
Platt, Frances M.
中科院分区：
文献类型：
--
作者：
Evans, Emyr Lloyd;Evans, Helen Waller;Platt, Frances M.
直到最近，人们对调节内溶酶体钙稳态的机制知之甚少。NAADP（烟酸-腺嘌呤二核苷酸磷酸）作为内溶酶体系统中存在的两个孔通道的分子靶标的发现，强调了该区室作为重要的钙储存库的作用。最近发现，NAADP 释放功能失调会导致内吞功能缺陷，进而导致内吞功能缺陷。 导致溶酶体贮积病中继发性脂质积累 Niemann-Pick C 型是人类疾病与溶酶体钙释放缺陷之间直接联系的第一个证据。在本综述中，我们总结了有关内溶酶体系统内钙稳态机制的最新知识，以及这些机制如何在人类代谢紊乱中受到影响
Until recently the mechanisms that regulate endolysosomal calcium homoeostasis were poorly understood The discovery of the molecular target of NAADP (nicotinic acid-adenine dinucleotide phosphate) as the two pore channels resident in the endolysosomal system has highlighted this compartment as an important calcium store The recent findings that dysfunctional NAADP release leads to defective endocytic function which in turn results in secondary lipid accumulation in the lysosomal storage disease Niemann-Pick type C is the first evidence of a direct connection between a human disease and defective lysosomal calcium release In the present review we provide a summary of the current knowledge on mechanisms of calcium homoeostasis within the endolysosomal system and how these mechanisms may be affected in human metabolic disorders