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Mouse Anti-SCO2 Recombinant Antibody (6D337) (CBMAB-C0319-CN)

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Datasheet
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Basic Information

Host Animal
Mouse
Clone
6D337
Application
WB
Immunogen
Equine Cytochrome C
Specificity
Horse, Goat, Human, Monkey, Mouse, Rat, Sheep
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS
Preservative
0.02% Sodium azide
Concentration
1 mg/mL
Purity
> 95% as determined by SDS-PAGE
More Infomation

Target

Full Name
SCO2, Cytochrome C Oxidase Assembly Protein
Introduction
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]
Entrez Gene ID
Human9997
Mouse100126824
Horse100056712
UniProt ID
HumanO43819
MouseQ8VCL2
HorseF6UXT1
Alternative Names
SCO2, Cytochrome C Oxidase Assembly Protein; SCO (Cytochrome Oxidase Deficient, Yeast) Homolog 2; SCO Cytochrome Oxidase Deficient Homolog 2 (Yeast); SCO Cytochrome Oxidase Deficient Homolog 2; Protein SCO2 Homolog, Mitochondrial; Myopia 6; CEMCOX1; SCO1L; MYP6;
Function
Copper metallochaperone essential for the synthesis and maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Involved in transporting copper to the Cu(A) site on MT-CO2/COX2 (PubMed:15229189, PubMed:17189203).
Also acts as a thiol-disulfide oxidoreductase to regulate the redox state of the cysteines in SCO1 during maturation of MT-CO2/COX2 (PubMed:19336478).
Biological Process
Biological Process cellular copper ion homeostasisIEA:InterPro
Biological Process eye developmentManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process in utero embryonic developmentIEA:Ensembl
Biological Process mitochondrial cytochrome c oxidase assemblyManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process muscle system processIEA:Ensembl
Biological Process respiratory electron transport chainIEA:Ensembl
Biological Process response to activityIEA:Ensembl
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex IV deficiency, nuclear type 2 (MC4DN2):
An autosomal recessive, severe mitochondrial disorder characterized by hypotonia, global developmental delay, hypertrophic cardiomyopathy, lactic acidosis, gliosis, and neuronal loss in basal ganglia, brainstem and spinal cord. Serum lactate is increased, and laboratory studies show decreased mitochondrial complex IV protein and activity levels in various tissues, including heart and skeletal muscle. Most patients die in infancy of cardiorespiratory failure.
Myopia 6 (MYP6):
A refractive error of the eye, in which parallel rays from a distant object come to focus in front of the retina, vision being better for near objects than for far.
Topology
Mitochondrial matrix: 42-60
Helical: 61-78
Mitochondrial intermembrane: 79-266
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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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