PMID- 27430889
OWN - NLM
STAT- MEDLINE
DCOM- 20170707
LR  - 20181113
IS  - 1869-1889 (Electronic)
IS  - 1674-7305 (Print)
IS  - 1674-7305 (Linking)
VI  - 59
IP  - 8
DP  - 2016 Aug
TI  - Regulation of lysosomal ion homeostasis by channels and transporters.
PG  - 777-91
LID - 10.1007/s11427-016-5090-x [doi]
AB  - Lysosomes are the major organelles that carry out degradation functions. They 
      integrate and digest materials compartmentalized by endocytosis, phagocytosis or 
      autophagy. In addition to more than 60 hydrolases residing in the lysosomes, 
      there are also ion channels and transporters that mediate the flux or transport 
      of H(+), Ca(2+), Na(+), K(+), and Cl(-) across the lysosomal membranes. Defects 
      in ionic exchange can lead to abnormal lysosome morphology, defective vesicle 
      trafficking, impaired autophagy, and diseases such as neurodegeneration and 
      lysosomal storage disorders. The latter are characterized by incomplete lysosomal 
      digestion and accumulation of toxic materials inside enlarged intracellular 
      vacuoles. In addition to degradation, recent studies have revealed the roles of 
      lysosomes in metabolic pathways through kinases such as mechanistic target of 
      rapamycin (mTOR) and transcriptional regulation through calcium signaling 
      molecules such as transcription factor EB (TFEB) and calcineurin. Owing to the 
      development of new approaches including genetically encoded fluorescence probes 
      and whole endolysosomal patch clamp recording techniques, studies on lysosomal 
      ion channels have made remarkable progress in recent years. In this review, we 
      will focus on the current knowledge of lysosome-resident ion channels and 
      transporters, discuss their roles in maintaining lysosomal function, and evaluate 
      how their dysfunction can result in disease.
FAU - Xiong, Jian
AU  - Xiong J
AD  - Department of Integrative Biology and Pharmacology, McGovern Medical School, 
      Program of Cell and Regulatory Biology, Graduate School of Biomedical Sciences, 
      The University of Texas Health Science Center at Houston, Houston, 77030, USA.
FAU - Zhu, Michael X
AU  - Zhu MX
AD  - Department of Integrative Biology and Pharmacology, McGovern Medical School, 
      Program of Cell and Regulatory Biology, Graduate School of Biomedical Sciences, 
      The University of Texas Health Science Center at Houston, Houston, 77030, USA. 
      Michael.x.zhu@uth.tmc.edu.
LA  - eng
GR  - R01 NS092377/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Review
DEP - 20160719
PL  - China
TA  - Sci China Life Sci
JT  - Science China. Life sciences
JID - 101529880
RN  - 0 (Ion Channels)
RN  - 0 (Ions)
RN  - 0 (Membrane Transport Proteins)
SB  - IM
MH  - *Homeostasis
MH  - Humans
MH  - Hydrogen-Ion Concentration
MH  - Intracellular Membranes/metabolism
MH  - Ion Channels/*metabolism
MH  - Ion Transport
MH  - Ions/*metabolism
MH  - Lysosomes/chemistry/*metabolism
MH  - Membrane Transport Proteins/*metabolism
MH  - Models, Biological
PMC - PMC5147046
MID - NIHMS833672
OTO - NOTNLM
OT  - calcium
OT  - ion homeostasis
OT  - lysosomal storage disease (LSD)
OT  - lysosome acidification
COIS- Compliance and ethics The author(s) declare that they have no conflict of 
      interest.
EDAT- 2016/07/20 06:00
MHDA- 2017/07/08 06:00
PMCR- 2016/12/09
CRDT- 2016/07/20 06:00
PHST- 2016/05/06 00:00 [received]
PHST- 2016/06/02 00:00 [accepted]
PHST- 2016/07/20 06:00 [entrez]
PHST- 2016/07/20 06:00 [pubmed]
PHST- 2017/07/08 06:00 [medline]
PHST- 2016/12/09 00:00 [pmc-release]
AID - 10.1007/s11427-016-5090-x [pii]
AID - 10.1007/s11427-016-5090-x [doi]
PST - ppublish
SO  - Sci China Life Sci. 2016 Aug;59(8):777-91. doi: 10.1007/s11427-016-5090-x. Epub 
      2016 Jul 19.