@article{7df2fed31e644d45ab6ba068a42d7af1,
title = "Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations",
abstract = "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.",
keywords = "CE-MS, Cybrid, Energy metabolism, MELAS, Metabolome, Pyruvate",
author = "Kenjiro Kami and Yasunori Fujita and Saori Igarashi and Sayaka Koike and Shoko Sugawara and Satsuki Ikeda and Naomi Sato and Masafumi Ito and Masashi Tanaka and Masaru Tomita and Tomoyoshi Soga",
note = "Funding Information: The authors thank Dr. Masahiro Sugimoto for software development and Dr. Maria R. Monton and Ms. Kaori Igarashi for conducting the metabolome analysis. This work was supported in part by the following grants: a grant of the Global COE Program entitled, “ Human Metabolomic Systems Biology ,” “Grant-in-Aid” research grants for the 21st Century Centre of Excellence (COE) Program (F-3); Scientific Research ( A-22240072 and B-21390459 ), Creative Scientific Research ( 180 73004 , 18GS0314 ), Targeted Proteins Research Program (TPRP) , and Scientific Research on Priority Areas “Systems Genomes” and “Lifesurveyor” from the Ministry of Education, Culture, Sports, Science, and Technology (MEXT) of Japan ; Grant-in-Aids ( H23-016 , H23-119 , and H24-005 ) for the Research on Intractable Diseases (Mitochondrial Disease) from the Ministry of Health, Labour and Welfare (MHLW) of Japan ; grants for scientific research from the Takeda Science Foundation ; a grant for biological research from Gifu Prefecture, Japan ; and research funds from the Government of Yamagata Prefecture and Tsuruoka City, Japan .",
year = "2012",
month = nov,
doi = "10.1016/j.mito.2012.07.113",
language = "English",
volume = "12",
pages = "644--653",
journal = "Mitochondrion",
issn = "1567-7249",
publisher = "Elsevier",
number = "6",
}
