BCS1L Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a partial recombinant BCS1L.
|Calculated MW||47534 Da|
|Other Names||Mitochondrial chaperone BCS1, h-BCS1, BCS1-like protein, BCS1L, BCS1|
|Target/Specificity||BCS1L (NP_004319, 320 a.a. ~ 418 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.|
|Format||Clear, colorless solution in phosphate buffered saline, pH 7.2 .|
|Storage||Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.|
|Precautions||BCS1L Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.|
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This gene encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Two alternatively spliced transcripts encoding the same protein have been described.
1.Characterization of complex III deficiency and liver dysfunction in GRACILE syndrome caused by a BCS1L mutation.Kotarsky H, Karikoski R, Morgelin M, Marjavaara S, Bergman P, Zhang DL, Smet J, van Coster R, Fellman F.Mitochondrion (2010), doi:10.1016/ j.mito.2010.05.0092.Pathogenic mutations in the 5' untranslated region of BCS1L mRNA in mitochondrial complex III deficiency.Gil-Borlado MC, Gonzalez-Hoyuela M, Blazquez A, Garcia-Silva MT, Gabaldon T, Manzanares J, Vara J, Martin MA, Seneca S, Arenas J, Ugalde C.Mitochondrion. 2009 Sep;9(5):299-305. Epub 2009 Apr 21.
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