Recombinant Human Mitochondrial inner membrane protease subunit 2 (IMMP2L), Truncated

Uniprot Id

Q96T52

Target Species

Human

Target Name

IMMP2L

Target Full Name

Mitochondrial inner membrane protease subunit 2

Target 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.

Target Involvement

Gilles de la Tourette syndrome (GTS)

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein.

Target Protein Families

Peptidase S26 family, IMP2 subfamily

Target Tissue Specificity

Expressed in all tissues tested except adult liver and lung.

Target Synonyms

IMMP2L; Mitochondrial inner membrane protease subunit 2; IMP2-like protein

Target Background

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.