Citrate synthase-lysine N-methyltransferase, mitochondrial (CSKMT)

Citrate synthase-lysine N-methyltransferase, mitochondrial (CSKMT), also known as METTL12, is a protein-lysine methyltransferase located in the mitochondrial matrix that selectively trimethylates lysine-368 on citrate synthase, a key enzyme in the tricarboxylic acid (TCA) cycle[1][2][3][4][5][7]. CSKMT catalyzes protein-lysine N-methylation, including mono-, di-, and trimethylation states, likely in a distributive (non-processive) manner[1][2][7]. This methylation modifies citrate synthase and may have regulatory functions for mitochondrial metabolism and energy production. Mutations or dysfunction in CSKMT have been associated with microvillus inclusion disease[1]. No drugs are currently known to directly target this enzyme, and its precise roles in disease and therapeutic modulation remain under exploration.

Other names

METTL12Methyltransferase-like protein 12CS-KMTU99HG