Temperature-sensitive mitochondrial outer membrane proteins used as novel quality control substrates reveal a unique mitochondria-associated degradation pathway consisting of both cytosolic and mitochondrial ubiquitin-proteasome system machinery.

During times of mitochondrial impairment, non-imported mitochondrial precursor proteins are targeted to the nucleus for proteasome-dependent degradation.

Mitophagy regulates mitochondrial quality and mediates extensive mitochondrial degradation in (patho-)physiological settings and is one of the key components of hypoxic preconditioning which protects the heart from ischemia/reperfusion injury.

The endoplasmic reticulum E3 ubiquitin ligase Doa10 and the mitochondrial AAA-ATPase Msp1 govern targeting fidelity of outer mitochondrial tail-anchored proteins by controlling cytoplasmic concentration and extracting mistargeted and orphan species.

Mitochondrial quality control in the germline is mediated by regulators of both programmed cell death and the aging program.

Caenorhabditis elegans utilize multiple strategies to optimize germline mitochondrial genome integrity at the foundational stage of gonad development.

Removal of spurious poly(A) tails by the PDE12 exoribonuclease is required to maintain the integrity of human mitochondrial tRNAs and rRNAs and their availability for efficient mitochondrial translation.

Msp1 recruits substrates at the open seam of the spiral oligomer and extracts them with functionally adapted elements.

PINK1 deficiency in Parkinson's disease models causes toxic oxidized and iron-depleted CISD1 yet CISD1 removal benefits PINK1 mutant Drosophila indicating its potential as a therapeutic target for mitochondrial dysfunction and neurodegeneration.

Fundc1-mediated mitophagy protects renal EPO-producing cells from myofibroblastic conversion under stresses.