GTPase, IMAP Family, Member 3 (GIMAP3)

Gimap3, an outer mitochondrial membrane GTPase, is a critical regulator of this process in leukocytes. Gimap3 is important for T cell development and survival, suggesting that leukocyte survival may be a key factor in the genetic regulation of mtDNA sequence variants and in modulating human mitochondrial diseases.

Mitochondria are essential cellular organelles, which contain their own small circular genome in mammals. Gimap3, can regulate mtDNA segregation in mouse white blood cells. This is the first gene identified to regulate mtDNA segregation in mammals, which is also an important factor in cell survival. Future work on this protein will provide critical insight into the pathways that regulate mitochondrial DNA in white blood cells, an important factor in the pathogenesis of human mitochondrial DNA mutations.