YDR375C

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Systematic name YDR375C
Gene name BCS1
Aliases
Feature type ORF, Verified
Coordinates Chr IV:1226529..1225159
Primary SGDID S000002783


Description of YDR375C: Mitochondrial protein of the AAA ATPase family; has ATP-dependent chaperone activity; required for assembly of Rip1p and Qcr10p into cytochrome bc(1) complex; mutations in human homolog BCS1L are linked to neonatal mitochondrial diseases[1][2]




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References

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  1. 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
  2. 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

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