RHOT Proteins Link Mitochondrial Motility to Cardiomyocyte Sarcomere Maturation - PubMed
5 hours ago
- #sarcomere
- #mitochondria
- #cardiomyocyte
- RHOT proteins (RHOT1 and RHOT2) regulate mitochondrial motility by linking mitochondria to kinesin and dynein motors.
- Cardiomyocyte-selective deletion of Rhot1 and Rhot2 in embryos (cRhot1/2-KO) leads to fatal cardiomyopathy, sarcomere disarray, and perinuclear mitochondrial accumulation.
- Mitochondria from cRhot1/2-KO hearts show impaired motility but preserved respiratory capacity.
- Proteome analysis reveals RHOT proteins bind mitochondria to contractile muscle fiber proteins.
- Inducible deletion of Rhot1 and Rhot2 in adult mice (iRhot1/2-KO) does not cause heart failure, with preserved mitochondrial localization, ATP production, and sarcomere structure.
- RHOT proteins are essential for mitochondrial positioning during cardiomyocyte development but not after maturation.