SCO2; SCOD1; Protein SCO2 homolog; mitochondrial; SCO cytochrome oxidase deficient homolog 1 (yeast)

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

SCO2 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

SCO2 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to 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.

Small volumes of anti-SCO1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

SCO2 Antibody: Synthesis of cytochrome c oxidase 2 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 SCO2 and SCO1 are dependent on copper ion binding. Recent studies suggest that SCO2 expression is regulated by p53, so that a decrease in p53 expression, such as in numerous tumors and cells lines, the drop in SCO2 expression leads to a shift from normal aerobic respiration towards the production of glycolytic ATP. Defects in the SCO2 protein are also associated with fatal infantile cardioencephalomyopathy and COX deficiency.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

SCO2 antibody can be used for detection of SCO2 by Western blot at 0.5 - 2 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.

Western blot analysis of SCO2 in HL60 cell lysate lysate with SCO2 antibody at (A) 1 and (B) 2 μg/mL.

Immunohistochemistry of SCO2 in human liver tissue with SCO2 antibody at 2.5 μg/mL.

Immunofluorescence of SCO2 in Human Liver tissue with SCO2 antibody at 20 μg/mL.

29,810 Da

SCO2 cytochrome c oxidase assembly protein

protein SCO2 homolog, mitochondrial; SCO cytochrome oxidase deficient homolog 2

Protein SCO2 homolog, mitochondrial

SCO2_HUMAN

Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps to maintain the proton gradient across the inner mitochondrial membrane that is necessary for aerobic ATP production. Human COX is a multimeric protein complex that requires several assembly factors; this gene encodes one of the COX assembly factors. The encoded protein is a metallochaperone that is involved in the biogenesis of cytochrome c oxidase subunit II. Mutations in this gene are associated with fatal infantile encephalocardiomyopathy and myopia 6. [provided by RefSeq, Oct 2014]

SCO2: Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2). Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC). This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies. Belongs to the SCO1/2 family.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: nucleoplasm; myofibril; mitochondrion; mitochondrial inner membrane

Molecular Function: copper ion binding

Biological Process: cellular copper ion homeostasis; eye development; in utero embryonic development; respiratory chain complex IV assembly; response to activity; muscle system process; copper ion transport

Disease: Cardioencephalomyopathy, Fatal Infantile, Due To Cytochrome C Oxidase Deficiency 1; Myopia 6

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