A role for peroxisome proliferator-activated receptor γ coactivator 1 (PGC-1) in the regulation of cardiac mitochondrial phospholipid biosynthesis

Ling Lai, Miao Wang, Ola J. Martin, Teresa C. Leone, Rick B. Vega, Xianlin Han, Daniel P. Kelly

Research output: Contribution to journalArticlepeer-review

59 Scopus citations

Abstract

Background: Peroxisome proliferator-activated receptor γ coactivator 1 (PGC-1) γ and γ are transcriptional regulators of mitochondrial metabolism. Results: Loss of PGC-1 coactivators in mouse heart causes a defect in phospholipid biosynthesis resulting in mitochondrial ultrastructural abnormalities. Conclusion: PGC-1 coactivators coordinately regulate mitochondrial metabolism and structure. Significance: The mitochondrial phenotype of PGC-1-deficient mice resembles that of humans with genetic defects in mitochondrial phospholipid biosynthesis (Barth syndrome).

Original languageEnglish (US)
Pages (from-to)2250-2259
Number of pages10
JournalJournal of Biological Chemistry
Volume289
Issue number4
DOIs
StatePublished - Jan 24 2014
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

Fingerprint Dive into the research topics of 'A role for peroxisome proliferator-activated receptor γ coactivator 1 (PGC-1) in the regulation of cardiac mitochondrial phospholipid biosynthesis'. Together they form a unique fingerprint.

Cite this