SCO1 (Protein SCO1 Homolog, Mitochondrial, SCOD1)

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.\n\nApplications:\nSuitable for use in ELISA, Western Blot, Immunofluorescence and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 0.5-1ug/ml\nImmunofluorescence: 20ug/ml\nImmunohistochemistry (Formalin fixed paraffin embedded): 2.5ug/ml\nOptimal dilutions to be determined by the researcher.\n\nPositive Control:\nHuman Brain Tissue Lysate\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

146157

Size

100ug

Applications

ELISA, IF, IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

Supplied as a liquid in PBS, 0.02% sodium azide.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

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.

Additional Info

Recognizes human SCO1. Species Crossreactivity: mouse and rat.