The mitochondrial transporter SFXN1 is critical for Complex III integrity and cellular metabolism

Mutations in acylglycerol kinase (AGK) leads to Sengers syndrome, a rare disease characterized by skeletal myopathy, hypertrophic cardiomyopathy, and cataracts. AGK, in vitro, produces lysophosphatidic acid and phosphatidic acid via phosphorylation of monoacylglycerol and diacylglycerol, accordingly. AGK is also a component of the TIM22 import machinery in the inner mitochondrial membrane and, independent of its kinase activity, has been shown to mediate the import of mitochondrial carrier family (SLC25) members. We compared the proteomes of mitochondria from HEK293 WT and AGK KO cells in order to identify additional candidate substrates of the TIM22 translocase that relies on AGK.