SCO1 Antibody

SCO1 Antibody

Catalog# :4043

Synthesis of cytochrome c oxidase 1 was initially identified in yeast as one of two cytochrome c oxidase (COX) assembly proteins that enable the assembly of cytochrome c holoenzyme, a complex that catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. Like their yeast homologs, the function of both SCO1 and SCO2 are dependent on copper ion binding. Mutations in either gene can lead to cytochrome c oxidase respiratory chain defects, with a missense mutation in human SCO1 (P174L) associated with a fatal neonatal hepatopathy when the second allele is also non-functional, suggesting the pathology is due to loss of function. It has been suggested that this mutation alters the SCO1 affinity for the copper (I) ion, thus impairing the efficiency of copper transfer to the cytochrome c oxidase. At least two isoforms of SCO1 are known to exist and both are recognized by the SCO1 antibody. This SCO1 antibody has no cross-reactivity to SCO2.

Additional Names : SCO1, Synthesis of cytochrome c oxidase 1, cytochrome oxidase deficient homolog 1


Description
Left: Western blot analysis of SCO1 in human brain tissue lysate with SCO1 antibody at (A) 0.5 and (B) 1 µg/ml.

Below: Immunohistochemistry of SCO1 in human brain tissue with SCO1 antibody at 2.5 µg/ml.

Other Product Images
Source :SCO1 antibody was raised against a 14 amino acid peptide from near the center of human SCO1.
Purification : Affinity chromatography purified via peptide column
Clonality and Clone : This is a polyclonal antibody.
Host : SCO1 antibody was raised in rabbit. Please use anti-rabbit secondary antibodies.
Application : SCO1 antibody can be used for detection of SCO1 by Western blot at 0.5 – 1 µg/ml.
Tested Application(s) : E, WB, IHC
Buffer : Antibody is supplied in PBS containing 0.02% sodium azide.
Blocking Peptide :Cat.No. 4043P - SCO1 Peptide
Long-Term Storage : SCO1 antibody can be stored at 4ºC, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Positive Control :
1. Cat. No. 1303 - Human Brain Tissue Lysate
Species Reactivity : H, M, R
GI Number : 8134663
Accession Number : O75880
Short Description : Synthesis of cytochrome c oxidase 2
References
1. Glerum DM, Shtanko A, and Tzagoloff A. SCO1 and SCO2 act as high copy suppressors of a mitochondrial copper recruitment defect in Saccharomyces cerevisiae. J. Biol. Chem. 1996; 271:20531-5.
2. Horng Y-C, Leary SC, Cobine PA, et al. Human Sco1 and Sco2 function as copper-binding proteins. J. Biol. Chem. 2005; 280:34113-22.
3. Valnot I, Osmond S, Gigarel N. Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy. Am. J. Hum. Genet. 2000; 67:1104-9.
4. Banci L, Bertini I, Ciofi-Baffoni S, et al. Human Sco1 functional studies and pathological implications of the P174L mutant. Proc. Natl. Acad. Sci. USA 2007; 104:15-20.