Journal
ANNUAL REVIEW OF PHYSIOLOGY, VOL 77
Volume 77, Issue -, Pages 57-80Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-physiol-021014-071649
Keywords
TRPML1; TPC1; TPC2; mTOR; TFEB; lysosomal exocytosis; lysosomal storage disease
Categories
Funding
- NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES [R01AR060837] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF MENTAL HEALTH [R03MH096595] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS074257, R01NS055293, R01NS062792] Funding Source: NIH RePORTER
- NIAMS NIH HHS [R01 AR060837, AR060837] Funding Source: Medline
- NIMH NIH HHS [MH096595, R03 MH096595] Funding Source: Medline
- NINDS NIH HHS [NS074257, NS055293, R01 NS062792, R01 NS055293, R01 NS074257, NS062792] Funding Source: Medline
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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.
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