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|Product Name||SURF1, 80-273aa, Human|
|Background||SURF1, also known as surfeit 1, is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. This protein is localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. Defect in SURF1 is a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency. Recombinant SURF1 protein was expressed in E.coli and purified by using conventional chromatography techniques.|
|Synonyms||, Surfeit 1 SURF 1, SURF1, Surfeit locus protein 1,|
|AA Sequence||MGSSHHHHHH SSGLVPRGSH MQVQRRKWKL NLIAELESRV LAEPVPLPAD PMELKNLEYR PVKVRGCFDH SKELYMMPRT MVDPVREARE GGLISSSTQS GAYVVTPFHC TDLGVTILVN RGFVPRKKVN PETRQKGQIE GEVDLIGMVR LTETRQPFVP ENNPERNHWH YRDLEAMARI TGAEPIFIDA NFQSTVPGGP IGGQTRVTLR NEHLQ|
|Molecular Weight||37.8 kDa (333aa), confirmed by MALDI-TOF.|
|Form||Liquid, in 20mM Tris-HCl buffer (pH 8.0) containing 10% glycerol, 1mM DTT, 0.1 M NaCl.|
|Concentration||1 mg/ml (determined by Bradford assay)|
|Purity||> 95% by SDS-PAGE|
|Storage||Can be stored at +4C short term (1-2 weeks). For long term storage, aliquot and store at -20C or -70C. Avoid repeated freezing and thawing cycles.|
|Intended Use||For Research Use Only|
|References||Pequignot MO., et al. (2001) Hum Mutat. 17(5):374-81.
Zhu Z., et al. (1998) Nat Genet. 20(4):337-43.
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