This domain corresponds to the C-terminal of mitochondrial acylglycerol kinase (AGK, also known as MuLK) [, , ]. AGK is a metazoan-specific protein integrated into the mitochondrial inner membrane through a short N-terminal transmembrane domain. This large C-terminal domain is adjacent the kinase domain and it is oriented to the intermembrane space. AGK is a subunit of the human TIM22 complex which stabilises the complex and regulates the import and assembly of mitochondrial carrier proteins, a function independent of its kinase activity [, ]. Disturbances in both functions of AGK (phospholipid metabolism and mitochondrial protein biogenesis) contribute to the pathogenesis of Sengers syndrome, which is characterised by cataracts, hypertrophic cardiomyopathy, and skeletal myopathy [, ].
