YDR375C
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| Systematic name | YDR375C |
| Gene name | BCS1 |
| Aliases | |
| Feature type | ORF, Verified |
| Coordinates | Chr IV:1226536..1225166 |
| Primary SGDID | S000002783 |
Description of 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[1][2][3]
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References
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- Jump up ↑ Cruciat CM, et al. (1999) Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex. EMBO J 18(19):5226-33 SGD PMID 10508156
- Jump up ↑ Tuppen HA, et al. (2010) Long-term survival of neonatal mitochondrial complex III deficiency associated with a novel BCS1L gene mutation. Mol Genet Metab 100(4):345-8 SGD PMID 20472482
- Jump up ↑ Wagener N, et al. (2011) A pathway of protein translocation in mitochondria mediated by the AAA-ATPase Bcs1. Mol Cell 44(2):191-202 SGD PMID 22017868
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