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

Research output: Contribution to journalArticle

9 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)644-653
Number of pages10
JournalMitochondrion
Volume12
Issue number6
DOIs
Publication statusPublished - 2012 Nov

Fingerprint

Lactic Acidosis
Metabolomics
Brain Diseases
Muscular Diseases
Pyruvic Acid
Mitochondrial DNA
Stroke
Mutation
MELAS Syndrome
Mitochondrial Diseases
Capillary Electrophoresis
Osteosarcoma
Therapeutics
Energy Metabolism
Mass Spectrometry
Mitochondria

Keywords

  • CE-MS
  • Cybrid
  • Energy metabolism
  • MELAS
  • Metabolome
  • Pyruvate

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology
  • Molecular Medicine

Cite this

Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations. / Kami, Kenjiro; Fujita, Yasunori; Igarashi, Saori; Koike, Sayaka; Sugawara, Shoko; Ikeda, Satsuki; Sato, Naomi; Ito, Masafumi; Tanaka, Masashi; Tomita, Masaru; Soga, Tomoyoshi.

In: Mitochondrion, Vol. 12, No. 6, 11.2012, p. 644-653.

Research output: Contribution to journalArticle

Kami, K, Fujita, Y, Igarashi, S, Koike, S, Sugawara, S, Ikeda, S, Sato, N, Ito, M, Tanaka, M, Tomita, M & Soga, T 2012, 'Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations', Mitochondrion, vol. 12, no. 6, pp. 644-653. https://doi.org/10.1016/j.mito.2012.07.113
Kami, Kenjiro ; Fujita, Yasunori ; Igarashi, Saori ; Koike, Sayaka ; Sugawara, Shoko ; Ikeda, Satsuki ; Sato, Naomi ; Ito, Masafumi ; Tanaka, Masashi ; Tomita, Masaru ; Soga, Tomoyoshi. / Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations. In: Mitochondrion. 2012 ; Vol. 12, No. 6. pp. 644-653.
@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",
year = "2012",
month = "11",
doi = "10.1016/j.mito.2012.07.113",
language = "English",
volume = "12",
pages = "644--653",
journal = "Mitochondrion",
issn = "1567-7249",
publisher = "Elsevier",
number = "6",

}

TY - JOUR

T1 - Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations

AU - Kami, Kenjiro

AU - Fujita, Yasunori

AU - Igarashi, Saori

AU - Koike, Sayaka

AU - Sugawara, Shoko

AU - Ikeda, Satsuki

AU - Sato, Naomi

AU - Ito, Masafumi

AU - Tanaka, Masashi

AU - Tomita, Masaru

AU - Soga, Tomoyoshi

PY - 2012/11

Y1 - 2012/11

N2 - 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.

AB - 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.

KW - CE-MS

KW - Cybrid

KW - Energy metabolism

KW - MELAS

KW - Metabolome

KW - Pyruvate

UR - http://www.scopus.com/inward/record.url?scp=84869093640&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84869093640&partnerID=8YFLogxK

U2 - 10.1016/j.mito.2012.07.113

DO - 10.1016/j.mito.2012.07.113

M3 - Article

VL - 12

SP - 644

EP - 653

JO - Mitochondrion

JF - Mitochondrion

SN - 1567-7249

IS - 6

ER -