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Mouse Anti-MT-CO3 Recombinant Antibody (CBFYM-2724) (CBMAB-M2917-FY)


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Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Cattle, Human, S. cerevisiae
Clone
CBFYM-2724
Antibody Isotype
IgG2a
Application
WB, IP

Basic Information

Specificity
Mouse, Rat, Cattle, Human, S. cerevisiae
Antibody Isotype
IgG2a
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
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
MITOCHONDRIALLY ENCODED CYTOCHROME C OXIDASE III
Introduction
MT-CO3 is a Protein Coding gene. Diseases associated with MT-CO3 include Leber Hereditary Optic Neuropathy and Genetic Recurrent Myoglobinuria. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Gene Expression. Gene Ontology annotations related to this gene include cytochrome-c oxidase activity and heme-copper terminal oxidase activity.
Entrez Gene ID
Human4514
Mouse17710
Rat26204
UniProt ID
HumanP00414
MouseP00416
RatP05505
CattleP00415
Alternative Names
Mitochondrially Encoded Cytochrome C Oxidase III; Cytochrome C Oxidase III; MTCO3; COIII; Cytochrome C Oxidase Polypeptide III; Cytochrome C Oxidase Subunit III; EC 1.9.3.1; COXIII; COX3
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
Aerobic respiration Source: GO_Central
Cellular respiration Source: ComplexPortal
Mitochondrial electron transport, cytochrome c to oxygen Source: GO_Central
Respiratory chain complex IV assembly Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Leber hereditary optic neuropathy (LHON):
A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
Mitochondrial complex IV deficiency (MT-C4D):
A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome.
Recurrent myoglobinuria mitochondrial (RM-MT):
Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine.
Topology
Mitochondrial matrix: 1-15
Helical: 16-34
Mitochondrial intermembrane: 35-40
Helical: 41-66
Mitochondrial matrix: 67-72
Helical: 73-105
Mitochondrial intermembrane: 106-128
Helical: 129-152
Mitochondrial matrix: 153-155
Helical: 156-183
Mitochondrial intermembrane: 184-190
Helical: 191-223
Mitochondrial matrix: 224-232
Helical: 233-256
Mitochondrial intermembrane: 257-261
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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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