Presenilins-associated rhomboid-like protein, mitochondrial

Definition:

Required for the control of apoptosis during postnatal growth. Essential for proteolytic processing of an antiapoptotic form of OPA1 which prevents the release of mitochondrial cytochrome c in response to intrinsic apoptotic signals (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP). Promotes changes in mitochondria morphology regulated by phosphorylation of P-beta domain. Required for processing of CLPB into a form with higher protein disaggregase activity by removing an autoinhibitory N-terminal peptide.

References:

[1]. Andrew W Greene, et al. Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment. EMBO Rep. 2012 Apr;13(4):378-85. [Content Brief]

[2]. Ryan R Cupo, et al. Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations. Elife. 2020 Jun 23;9:e55279. [Content Brief]

[3]. Attila Sík, et al. Self-regulated cleavage of the mitochondrial intramembrane-cleaving protease PARL yields Pbeta, a nuclear-targeted peptide. J Biol Chem. 2004 Apr 9;279(15):15323-9. [Content Brief]

[4]. Danny V Jeyaraju, et al. Phosphorylation and cleavage of presenilin-associated rhomboid-like protein (PARL) promotes changes in mitochondrial morphology. Proc Natl Acad Sci U S A. 2006 Dec 5;103(49):18562-7. [Content Brief]

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