Inner mitochondrial membrane peptidase subunit 2 (IMMP2L)

Inner mitochondrial membrane peptidase subunit 2 (IMMP2L) is a mitochondrial serine-type peptidase encoded by the IMMP2L gene on chromosome 7q31 in humans[2][4][5]. This protein is part of the mitochondrial inner membrane peptidase complex and is responsible for cleaving signal peptide sequences from select imported mitochondrial proteins, such as cytochrome c1 and glycerol phosphate dehydrogenase (GPD2), thus allowing their correct maturation and integration into the mitochondrial inner membrane or intermembrane space[2][3][4]. IMMP2L plays a crucial role in maintaining mitochondrial functional integrity and redox homeostasis. Disruptions or mutations in IMMP2L have been associated with several neurodevelopmental and neuropsychiatric disorders, including Tourette syndrome and autism spectrum disorder, and experimental models show increased oxidative stress and neurodegeneration when IMMP2L function is compromised[3][4][5]. There is currently no evidence it is a direct therapeutic target or that drugs are known to interact with it.

Other names

IMP2Mitochondrial inner membrane protease subunit 2IMP2 inner mitochondrial membrane peptidase-like (S. cerevisiae)IMP2 inner mitochondrial membrane protease-like (S. cerevisiae)IMMP2L-IT1