Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations

Kenjiro Kami, Yasunori Fujita, Saori Igarashi, Sayaka Koike, Shoko Sugawara, Satsuki Ikeda, Naomi Sato, Masafumi Ito, Masashi Tanaka, Masaru Tomita, Tomoyoshi Soga

研究成果: Article査読

14 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)644-653
ページ数10
ジャーナルMitochondrion
12
6
DOI
出版ステータスPublished - 2012 11

ASJC Scopus subject areas

  • 分子医療
  • 分子生物学
  • 細胞生物学

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