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Mouse Anti-COX6A1 Recombinant Antibody (CBFYC-2126) (CBMAB-C2194-FY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYC-2126
Antibody Isotype
IgG1
Application
IF, FC

Basic Information

Immunogen
Full length human recombinant protein of human COX6A1.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal Antibody
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IF(ICC)1:100
FC1:100

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

Format
Liquid
Buffer
PBS, pH 7.3, 1% BSA, 50% Glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Cytochrome C Oxidase Subunit 6A1
Introduction
COX6A1 (Cytochrome C Oxidase Subunit 6A1) is a Protein Coding gene. Diseases associated with COX6A1 include Charcot-Marie-Tooth Disease, Recessive Intermediate D and Charcot-Marie-Tooth Disease, Axonal, Type 2R. 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 COX6A2.
Entrez Gene ID
Human1337
Mouse12861
Rat25282
UniProt ID
HumanP12074
MouseP43024
RatP10818
Alternative Names
Cytochrome C Oxidase Subunit 6A1; Cytochrome C Oxidase Subunit VIa Polypeptide 1; Cytochrome C Oxidase Polypeptide VIa-Liver; Cytochrome C Oxidase Subunit VIA-Liver; COX VIa-L; COX6AL
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 unsing 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.
Biological Process
Generation of precursor metabolites and energy Source: ProtInc
Mitochondrial electron transport, cytochrome c to oxygen Source: GO_Central
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Charcot-Marie-Tooth disease, recessive, intermediate type, D (CMTRID):
A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Recessive intermediate forms of Charcot-Marie-Tooth disease are characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec.
Topology
Mitochondrial matrix: 25-34
Helical: 35-59
Mitochondrial intermembrane: 60-109
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For research use only. Not intended for any clinical use.

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