期刊
BIOCHEMICAL SOCIETY TRANSACTIONS
卷 38, 期 -, 页码 1458-1464出版社
PORTLAND PRESS LTD
DOI: 10.1042/BST0381458
关键词
calcium regulation; endolysosome lysosomal storage disease; nicotinic acid-adenine dinucleotide phosphate (NAADP); Niemann Pick type C disease (NPC disease); two pore channel (TPC)
资金
- Action Medical Research
- SPARKS
- Newlife UK
- Action Medical Research [1766] Funding Source: researchfish
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
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