Anti-SCO2 Antibody (C-Terminus) IHC-plus; SCO2; SCO1L; Human SCO2

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

Polyclonal

Rabbit

19 amino acid peptide from near the carboxy terminus of human SCO2.

Purified

PBS, 0.02% sodium azide.

1 mg/ml (lot specific)

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles. Store undiluted.

Small volumes of anti-SCO2 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.

Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog of the yeast SCO2 gene.

Immunohistochemistry (IHC - Paraffin), Western Blot (WB)

IHC-P (2.5 ug/ml), WB
Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary antib...

Anti-SCO2 antibody IHC of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Anti-SCO2 antibody IHC of human lung, respiratory epithelium. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Immunofluorescence of SCO2 in Human Liver cells with SCO2 antibody at 20 ug/ml.

Western blot of SCO2 in human liver tissue lysate with SCO2 antibody at (A) 0.5, (B) 1 and (C) 2 ug/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; copper ion transport; muscle system process

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

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