Pitrilysin metallopeptidase 1 (PITRM1)

Pitrilysin metallopeptidase 1 (PITRM1) is a zinc-dependent, ATP-independent mitochondrial matrix enzyme of the metalloendopeptidase M16C family. It functions primarily in hydrolyzing and degrading short peptides including mitochondrial presequence fragments and amyloid beta peptides, which are implicated in neurodegenerative diseases. PITRM1 contributes to mitochondrial proteostasis and cell viability by preventing the accumulation of toxic peptides that can disrupt mitochondrial membrane potential. Deficiency, genetic mutations, or reduced activity of PITRM1 is associated with syndromes such as autosomal recessive spinocerebellar ataxia 30, mental retardation, cognitive dysfunction, and accumulation of amyloid beta aggregates, linking it with Alzheimer’s disease and other neurodegenerative conditions[1][3][4][5][7].

Other names

Presequence protease, mitochondrial (PreP)Metalloprotease 1 (MP1 or MTP-1)SCAR30PITRM1

Cleavage or degradation of amyloid beta peptides within mitochondria; Hydrolysis of cleaved mitochondrial transit peptides; Modulation of mitochondrial homeostasis by regulating toxic peptide levels.