期刊
MITOCHONDRION
卷 12, 期 6, 页码 644-653出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2012.07.113
关键词
MELAS; Pyruvate; Cybrid; Energy metabolism; Metabolome; CE-MS
资金
- Ministry of Education, Culture, Sports, Science, and Technology (MEXT) of Japan [A-22240072, B-21390459, 180 73004, 18GS0314, F-3]
- Ministry of Health, Labour and Welfare (MHLW) of Japan [H23-016, H23-119, H24-005]
- Takeda Science Foundation
- Gifu Prefecture, Japan
- Government of Yamagata Prefecture
- Tsuruoka City, Japan
- Grants-in-Aid for Scientific Research [22300244] Funding Source: KAKEN
Pyruvate treatment was found to alleviate clinical symptoms of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and is highly promising therapeutic. Using capillary electrophoresis time-of-flight mass spectrometry (CE-TOFMS), we measured time-changes of 161 intracellular and 85 medium metabolites to elucidate metabolic effects of pyruvate treatment on cybrid human 143B osteosarcoma cells harboring normal (2SA) and MELAS mutant (2SD) mitochondria. The results demonstrated dramatic and sustainable effects of pyruvate administration on the energy metabolism of 2SD cells, corroborating pyruvate as a metabolically rational treatment regimen for improving symptoms associated with MELAS and possibly other mitochondrial diseases. (C) 2012 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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