IMMP2L

This gene encodes a protein involved in processing the signal peptide sequences used to direct mitochondrial proteins to the mitochondria. The encoded protein resides in the mitochondria and is one of the necessary proteins for the catalytic activity of the mitochondrial inner membrane peptidase (IMP) complex. Two variants that encode the same protein have been described for this gene. [provided by RefSeq, Sep 2011]

Full Name

Inner Mitochondrial Membrane Peptidase Subunit 2

Function

Catalyzes the removal of transit peptides required for the targeting of proteins from the mitochondrial matrix, across the inner membrane, into the inter-membrane space. Known to process the nuclear encoded protein DIABLO.

Biological Process

Blood circulationIEA:Ensembl
Brain developmentIEA:Ensembl
Cellular response to DNA damage stimulusIEA:Ensembl
Cerebellum vasculature developmentIEA:Ensembl
Mitochondrial respiratory chain complex assemblyManual Assertion Based On ExperimentIBA:GO_Central
Ovarian follicle developmentIEA:Ensembl
OvulationIEA:Ensembl
Protein processing involved in protein targeting to mitochondrionBy SimilarityISS:UniProtKB
Respiratory electron transport chainIEA:Ensembl
Signal peptide processingIEA:InterPro
SpermatogenesisIEA:Ensembl
Superoxide metabolic processIEA:Ensembl

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Gilles de la Tourette syndrome (GTS):
Neurologic disorder manifested particularly by motor and vocal tics and associated with behavioral abnormalities.