bifunctional AAA family ATPase chaperone/translocase BCS1, L000000164, YDR375C

Protein translocase and chaperone required for Complex III assembly; member of the AAA ATPase family; forms a homo-oligomeric complex in the mitochondrial inner membrane that translocates the C-terminal domain of Rip1p from the matrix across the inner membrane, and then delivers it to an assembly intermediate of respiratory Complex III in an ATP-dependent reaction; also required for assembly of the Qcr10p subunit; mutations in human homolog BCS1L linked to neonatal diseases

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