Reactome | PITRM1 proteolyzes mitochondrial targeting peptides (presequences)

PITRM1 proteolyzes mitochondrial targeting peptides (presequences)

Stable Identifier

R-HSA-8986181

Type

Reaction [omitted]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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PITRM1 proteolyzes mitochondrial targeting peptides (presequences)

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After MPP cleaves off targeting peptides (also called presequences) of proteins imported into the mitochondrial matrix, PITRM1 (also called PreP) proteolyzes the cleaved targeting peptides (Pinho et al. 2010, Alikhani et al. 2011, Teixeira et al. 2012). PITRM1 also cleaves amyloid beta peptide that is imported into the matrix (Falkevall et al. 2006). Missense mutations in PITRM1 are associated with a neurological syndrome comprising mental retardation, spinocerebellar ataxia, cognitive decline, psychosis and possible epilepsy (Brunetti et al. 2016).

Literature References

PubMed ID	Title	Journal	Year
16849325	Degradation of the amyloid beta-protein by the novel mitochondrial peptidasome, PreP Falkevall, A, Alikhani, N, Bhushan, S, Pavlov, PF, Busch, K, Johnson, KA, Eneqvist, T, Tjernberg, L, Ankarcrona, M, Glaser, E	J. Biol. Chem.	2006
23041349	In vitro oxidative inactivation of human presequence protease (hPreP) Teixeira, PF, Pinho, CM, Branca, RM, Lehtiö, J, Levine, RL, Glaser, E	Free Radic. Biol. Med.	2012
21750375	Decreased proteolytic activity of the mitochondrial amyloid-β degrading enzyme, PreP peptidasome, in Alzheimer's disease brain mitochondria Alikhani, N, Guo, L, Yan, S, Du, H, Pinho, CM, Chen, JX, Glaser, E, Yan, SS	J. Alzheimers Dis.	2011
26697887	Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration Brunetti, D, Torsvik, J, Dallabona, C, Teixeira, P, Sztromwasser, P, Fernandez-Vizarra, E, Cerutti, R, Reyes, A, Preziuso, C, D'Amati, G, Baruffini, E, Goffrini, P, Viscomi, C, Ferrero, I, Boman, H, Telstad, W, Johansson, S, Glaser, E, Knappskog, PM, Zeviani, M, Bindoff, LA	EMBO Mol Med	2016
19962426	Genetic and biochemical studies of SNPs of the mitochondrial A beta-degrading protease, hPreP Pinho, CM, Björk, BF, Alikhani, N, Bäckman, HG, Eneqvist, T, Fratiglioni, L, Glaser, E, Graff, C	Neurosci. Lett.	2010