ATP-dependent metalloprotease YME1L1 (YME1L1)

ATP-dependent metalloprotease YME1L1 is an enzyme embedded in the inner mitochondrial membrane and belongs to the AAA family of ATPases. It is crucial for the maintenance of mitochondrial morphology, mitochondrial protein quality control, and regulation of protein import and turnover within mitochondria. By degrading unfolded, misfolded, or otherwise regulatory proteins—including those involved in protein import, respiratory chain complexes, and mitochondrial dynamics—it preserves mitochondrial function and structure. YME1L1 also regulates the balance between mitochondrial fusion and fission through proteolytic processing of OPA1. Deficiency or dysfunction in YME1L1 leads to perturbations in mitochondrial dynamics, increased susceptibility to apoptosis, and contributes to disease phenotypes including infantile-onset mitochondriopathies, optic atrophy, and may play a role in certain cancers and neurodegenerative conditions[1][2][3][4][5][6][7].

Other names

YME1 like 1 ATPaseYME1L1YME1-like protein 1i-AAA protease (inner mitochondrial membrane AAA protease)YM EL1_HUMAN

ATP-dependent degradation of unfolded, misfolded, or regulatory mitochondrial proteins; Maintenance of mitochondrial morphology via OPA1 cleavage and turnover; Regulation of mitochondrial protein import machinery (TIM22, TIM23); Turnover of electron transport chain proteins and mitochondrial lipid biosynthetic enzymes