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

Mitochondrion. 2012 Nov;12(6):644-53. doi: 10.1016/j.mito.2012.07.113. Epub 2012 Aug 4.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Line
  • DNA, Mitochondrial / genetics
  • Electrophoresis, Capillary
  • Energy Metabolism
  • Humans
  • MELAS Syndrome / physiopathology*
  • Metabolome*
  • Mutation
  • Pyruvic Acid / metabolism*
  • Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization


  • DNA, Mitochondrial
  • Pyruvic Acid