Article
The TIM22 complex mediates the import of sideroflexins and is required for efficient mitochondrial one-carbon metabolism.
Molecular biology of the cell - 15 Mar 2021
Jackson Thomas D, Hock Daniella H, Fujihara Kenji M, Palmer Catherine S, Frazier Ann E, Low Yau C, Kang Yilin, Ang Ching-Seng, Clemons Nicholas J, Thorburn David R, Stroud David A, Stojanovski Diana
Abstract excerpt
Acylglycerol kinase (AGK) is a mitochondrial lipid kinase that contributes to protein biogenesis as a subunit of the TIM22 complex at the inner mitochondrial membrane. Mutations in AGK cause Sengers syndrome, an autosomal recessive condition characterized by congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, and lactic acidosis. We mapped the proteomic changes in Sengers patient fibroblasts and...
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