TIMM22 is an essential core component of the TIM22 protein insertion complex, a complex that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. In the TIM22 complex, it constitutes the voltage-activated, signal-gated hydrophilic high-conductance channel. It forms a twin-pore translocase that uses the membrane potential as an external driving force in 2 voltage-dependent steps.
Thompson K et al. Recurrent De Novo Dominant Mutations in SLC25A4 Cause Severe Early-Onset Mitochondrial Disease and Loss of Mitochondrial DNA Copy Number. Am J Hum Genet99:860-876 (2016).
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