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Mouse Anti-COX6B1 Recombinant Antibody (2D3) (CBMAB-C4886-LY)

This product is antibody recognizes COX6B1. The antibody 2D3 immunoassay techniques such as: ELISA, WB.
See all COX6B1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2D3
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
COX6B1 (AAH01015, 1 a.a. ~ 86 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: MAEDMETKIK NYKTAPFDSR FPNQNQTRNC WQNYLDFHRC QKAMTAKGGD ISVCEWYQRV YQSLCPTSWV TDWDEQRAEG TFPGKI
Specificity
Human
Antibody Isotype
IgG2a, κ
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
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
cytochrome c oxidase subunit Vib polypeptide 1 (ubiquitous)
Introduction
COX6B1 (Cytochrome C Oxidase Subunit 6B1) is a Protein Coding gene. Diseases associated with COX6B1 include Mitochondrial Complex Iv Deficiency and Encephalomyopathy. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Gene Expression.
Gene Ontology (GO) annotations related to this gene include cytochrome-c oxidase activity.
An important paralog of this gene is COX6B2.
Entrez Gene ID
1340
UniProt ID
P14854
Alternative Names
Cytochrome C Oxidase Subunit 6B1; Cytochrome C Oxidase Subunit Vib Polypeptide 1 (Ubiquitous); COX VIb-1; COX6B; Cytochrome C Oxidase Subunit VIb Isoform 1; Cytochrome C Oxidase Subunit Vib; COXVIb1; COXG;
Function
Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.
Biological Process
Mitochondrial electron transport, cytochrome c to oxygen Source: Reactome
Substantia nigra development Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex IV deficiency, nuclear type 7 (MC4DN7):
An autosomal recessive mitochondrial disorder characterized by encephalomyopathy resulting in variable clinical manifestations. Features include muscle weakness, gait disturbances, neurodegeneration, cognitive decline, metabolic acidosis, feeding difficulties, poor overall growth, cortical visual impairment, and hypertrophic cardiomyopathy. Serum lactate levels are increased. Patient tissues show decreased levels and activity of mitochondrial respiratory complex IV.
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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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