Recombinant Human Presequence protease, mitochondrial (PITRM1), Truncated

Uniprot Id

Q5JRX3

Target Species

Human

Target Name

PITRM1

Target Full Name

Presequence protease, mitochondrial

Target Function

Metalloendopeptidase of the mitochondrial matrix that functions in peptide cleavage and degradation rather than in protein processing. Has an ATP-independent activity. Specifically cleaves peptides in the range of 5 to 65 residues. Shows a preference for cleavage after small polar residues and before basic residues, but without any positional preference. Degrades the transit peptides of mitochondrial proteins after their cleavage. Also degrades other unstructured peptides. It is also able to degrade amyloid-beta protein 40, one of the peptides produced by APP processing, when it accumulates in mitochondrion. It is a highly efficient protease, at least toward amyloid-beta protein 40. Cleaves that peptide at a specific position and is probably not processive, releasing digested peptides intermediates that can be further cleaved subsequently.

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Peptidase M16 family, PreP subfamily

Target Tissue Specificity

Widely expressed. Expressed at higher level in muscle and heart compared to brain, pancreas, liver, lung and placenta.

Target Synonyms

hMP1; hPreP; KIAA1104; Metalloprotease 1; MGC138192; MGC141929 ; mitochondrial; MP1; Pitrilysin metallopeptidase 1; Pitrilysin metalloproteinase 1; PITRM 1; PITRM1; PreP; PreP peptidasome; PREP_HUMAN; Presequence protease; Presequence protease mitochondrial

Target Background

The protein encoded by this gene is an ATP-dependent metalloprotease that degrades post-cleavage mitochondrial transit peptides. The encoded protein binds zinc and can also degrade amyloid beta A4 protein, suggesting a possible role in Alzheimer's disease.