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

doi:10.1074/jbc.M113.523654

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

Resultado de la investigación: Article › revisión exhaustiva

69 Citas (Scopus)

Resumen

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).

Idioma original	English (US)
Páginas (desde-hasta)	2250-2259
Número de páginas	10
Publicación	Journal of Biological Chemistry
Volumen	289
N.º	4
DOI	https://doi.org/10.1074/jbc.M113.523654
Estado	Published - ene. 24 2014
Publicado de forma externa	Sí

ASJC Scopus subject areas

Biochemistry
Molecular Biology
Cell Biology

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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).",

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AU - Wang, Miao

AU - Martin, Ola J.

AU - Leone, Teresa C.

AU - Vega, Rick B.

AU - Han, Xianlin

AU - Kelly, Daniel P.

PY - 2014/1/24

Y1 - 2014/1/24

N2 - 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).

AB - 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).

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

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