Mitochondrial contact site and cristae organizing system subunit 10 (MICOS10)

Mitochondrial contact site and cristae organizing system subunit 10 (MICOS10), also known as MIC10, is a critical component of the MICOS complex, a multi-protein assembly located at the inner membrane of mitochondria where it orchestrates the formation and maintenance of crista junctions and overall mitochondrial architecture[1][5]. MICOS10 plays a vital role in enabling proper mitochondrial respiration by preserving the structural integrity of cristae, which are essential for efficient energy production[2][3][5]. Genetic variants in MICOS10 have been identified as a cause of hepatocerebral mitochondrial DNA depletion syndrome (MTDPS), a severe disorder featuring liver and central nervous system involvement due to impaired mitochondrial structure and function[1][2][3]. Current evidence implicates MICOS10 as necessary for normal mitochondrial DNA maintenance and suggests that deficiency disrupts mitochondrial dynamics, reduces ATP synthesis, and impairs organ function, but it is not a direct therapeutic target—no drugs are known to interact with or modulate MICOS10 directly[1][2][5].

Other names

MICOS complex subunit MIC10MIC10C1orf151MINOS1RP5-1056L3.2FLJ36999MIO10Mic10UPF0327 protein C1orf151mitochondrial inner membrane organizing system protein 1