Mitochondrial contact site and cristae organizing system subunit 13 (MICOS13)

Mitochondrial contact site and cristae organizing system subunit 13 (MICOS13), also known as MIC13, QIL1, or C19orf70, is a small (9.7 kDa, 88 amino acids) protein located in the inner membrane of mitochondria[2][3]. MICOS13 is a crucial component of the MICOS complex, which is responsible for maintaining the integrity and shape of mitochondrial cristae junctions, structures essential for mitochondrial respiration and function[1][3][7]. It facilitates the incorporation of other MICOS subunits and is necessary for the proper formation of cristae junctions and mitochondrial architecture[2][3][7]. Mutations in MICOS13 lead to disruption of mitochondrial structure and severe mitochondrial diseases, including combined oxidative phosphorylation deficiency and mitochondrial DNA depletion syndrome, especially hepatocerebral forms with early-onset encephalopathy and liver dysfunction[4]. MICOS13 is not a classic pharmacological target (e.g., enzyme, receptor), but rather a mitochondrial structural protein critical for organelle integrity and bioenergetics.

Other names

MICOS13MIC13C19orf70QIL1P117MIC12chromosome 19 open reading frame 70protein QIL1protein P117