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 language | English (US) |
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Pages (from-to) | 2250-2259 |
Number of pages | 10 |
Journal | Journal of Biological Chemistry |
Volume | 289 |
Issue number | 4 |
DOIs | |
State | Published - Jan 24 2014 |
Externally published | Yes |
ASJC Scopus subject areas
- Molecular Biology
- Biochemistry
- Cell Biology