MT-CO2
MT-CO2 (Mitochondrially Encoded Cytochrome C Oxidase II) is a Protein Coding gene. Diseases associated with MT-CO2 include Mitochondrial Complex Iv Deficiency and Familial Colorectal Cancer. Among its related pathways are Development EGFR signaling via small GTPases and Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. Gene Ontology (GO) annotations related to this gene include oxidoreductase activity and cytochrome-c oxidase activity.
Full Name
Mitochondrially Encoded Cytochrome C Oxidase II
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
ATP synthesis coupled electron transport Source: GO_Central
Cellular respiration Source: ComplexPortal
Lactation Source: Ensembl
Mitochondrial electron transport, cytochrome c to oxygen Source: FlyBase
Positive regulation of ATP biosynthetic process Source: Ensembl
Positive regulation of hydrogen peroxide biosynthetic process Source: Ensembl
Positive regulation of necrotic cell death Source: Ensembl
Response to cold Source: Ensembl
Cellular respiration Source: ComplexPortal
Lactation Source: Ensembl
Mitochondrial electron transport, cytochrome c to oxygen Source: FlyBase
Positive regulation of ATP biosynthetic process Source: Ensembl
Positive regulation of hydrogen peroxide biosynthetic process Source: Ensembl
Positive regulation of necrotic cell death Source: Ensembl
Response to cold Source: Ensembl
Cellular Location
Mitochondrion inner membrane
Involvement in disease
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.
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.
Topology
Mitochondrial intermembrane: 1-14
Helical: 15-45
Mitochondrial matrix: 46-59
Helical: 60-87
Mitochondrial intermembrane: 88-227
Helical: 15-45
Mitochondrial matrix: 46-59
Helical: 60-87
Mitochondrial intermembrane: 88-227
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Anti-MT-CO2 antibodies
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Target: MT-CO2
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: A1199
Application*: FC, ICC, IF, IHC, IP, WB
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Rat
Clone: CBWJC-4190
Application*: WB
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBWJC-4189
Application*: WB
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBWJC-4184
Application*: E, IH, WB
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Rat
Clone: CBWJC-4183
Application*: WB
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Rat
Clone: CBWJC-4181
Application*: E
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBWJC-4180
Application*: F
Target: MT-CO2
Host: Rabbit
Antibody Isotype: IgG
Specificity: Mouse, Human
Clone: CBWJC-2337
Application*: WB, IC, IC
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: AS66
Application*: WB, E, F, SE
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBXC-1763
Application*: WB, IP, IF, P, E
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Rat, Mouse, Chicken, Human
Clone: 33/Cox-2
Application*: WB, IF, IH
Target: MT-CO2
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYM-0309
Application*: IF
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 12C4F12
Application*: WB, P, C, F, IF
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Rat, Human
Clone: CBWJC-2972
Application*: WB
Target: MT-CO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBWJC-2971
Application*: WB
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(P): Predicted
* Abbreviations
- AActivation
- AGAgonist
- APApoptosis
- BBlocking
- BABioassay
- BIBioimaging
- CImmunohistochemistry-Frozen Sections
- CIChromatin Immunoprecipitation
- CTCytotoxicity
- CSCostimulation
- DDepletion
- DBDot Blot
- EELISA
- ECELISA(Cap)
- EDELISA(Det)
- ESELISpot
- EMElectron Microscopy
- FFlow Cytometry
- FNFunction Assay
- GSGel Supershift
- IInhibition
- IAEnzyme Immunoassay
- ICImmunocytochemistry
- IDImmunodiffusion
- IEImmunoelectrophoresis
- IFImmunofluorescence
- IGImmunochromatography
- IHImmunohistochemistry
- IMImmunomicroscopy
- IOImmunoassay
- IPImmunoprecipitation
- ISIntracellular Staining for Flow Cytometry
- LALuminex Assay
- LFLateral Flow Immunoassay
- MMicroarray
- MCMass Cytometry/CyTOF
- MDMeDIP
- MSElectrophoretic Mobility Shift Assay
- NNeutralization
- PImmunohistologyp-Paraffin Sections
- PAPeptide Array
- PEPeptide ELISA
- PLProximity Ligation Assay
- RRadioimmunoassay
- SStimulation
- SESandwich ELISA
- SHIn situ hybridization
- TCTissue Culture
- WBWestern Blot
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