SCO2, Polyclonal Antibody

Protein SCO2 homolog; mitochondrial; SCO2

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

Polyclonal

Rabbit Ig

Rabbit

This SCO2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 196-224 amino acids from the C-terminal region of human SCO2.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

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 asembly 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.

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

SCO2 Antibody (C-term) western blot analysis in ZR-75-1 cell line lysates (35ug/lane).This demonstrates the SCO2 antibody detected the SCO2 protein (arrow).

29810

SCO2 cytochrome c oxidase assembly protein

protein SCO2 homolog, mitochondrial

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; mitochondrion; myofibril; 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

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Foltopoulou, P.F., et al. Biochim. Biophys. Acta 1802(6):497-508(2010)
Pronicki, M., et al. Eur. J. Paediatr. Neurol. 14(3):253-260(2010)
Kamatani, Y., et al. Nat. Genet. 42(3):210-215(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)

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