IMMP2L
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.
Involvement in disease
Gilles de la Tourette syndrome
GTS
Neurologic disorder manifested particularly by motor and vocal tics and associated with behavioral abnormalities.
None
The disease may be caused by variants affecting the gene represented in this entry.
Sequence Similarities
Belongs to the peptidase S26 family. IMP2 subfamily.
Tissue Specificity
Expressed in all tissues tested except adult liver and lung.
Cellular localization
- Mitochondrion inner membrane
- Single-pass membrane protein
Alternative names
Mitochondrial inner membrane protease subunit 2, IMP2-like protein, IMMP2L