A muscle-enriched mitochondrial protein regulates postnatal heart growth through mitonuclear ubiquinone-PPAR signaling.

Loss of Coq10a in mice promotes postnatal cardiac hypertrophy through altered ubiquinone partitioning and PPAR signaling without impairing mitochondrial respiration.

• Why it matters: Understanding tissue-specific mitochondrial proteins like Coq10a is crucial because their roles beyond energy production remain unclear, especially in organ development and adaptation.
• What they did: Researchers generated three Coq10a-deficient mouse models and analyzed their cardiac growth, mitochondrial function, and gene expression, focusing on ubiquinone distribution and PPAR pathway involvement.
• The result: Coq10a deficiency increased ubiquinone in microsomal membranes, activated PPAR-associated genes, and caused hypertrophy, suggesting Coq10a regulates organ-specific mitochondrial lipid distribution and signaling during postnatal heart growth.