ACO2

The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification. [provided by RefSeq]
Full Name
aconitase 2, mitochondrial
Function
Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.
Biological Process
Citrate metabolic process
Generation of precursor metabolites and energy
Tricarboxylic acid cycle
Cellular Location
Mitochondrion
Involvement in disease
Infantile cerebellar-retinal degeneration (ICRD): A severe autosomal recessive neurodegenerative disorder characterized by onset between ages 2 and 6 months of truncal hypotonia, athetosis, seizures, and ophthalmologic abnormalities, particularly optic atrophy and retinal degeneration. Affected individuals show profound psychomotor retardation, with only some achieving rolling, sitting, or recognition of family. Brain MRI shows progressive cerebral and cerebellar degeneration.
Optic atrophy 9 (OPA9): A condition that features progressive visual loss in association with optic atrophy. Atrophy of the optic disk indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm and optic tracts.
PTM
Forms covalent cross-links mediated by transglutaminase TGM2, between a glutamine and the epsilon-amino group of a lysine residue, forming homopolymers and heteropolymers.
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Anti-ACO2 antibodies

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Mouse Anti-ACO2 Recombinant Antibody (V2-179329) (CBMAB-A0627-YC)

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Target: ACO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat
Clone: V2-179329
Application*: WB, IP, P, C, F, IC, IF, E
Datasheet SDS

Rabbit Anti-ACO2 Recombinant Antibody (V2-179331) (CBMAB-A0630-YC)

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Target: ACO2
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat, Hamster, Monkey
Clone: V2-179331
Application*: WB, IP, IF
Datasheet SDS

Mouse Anti-ACO2 Recombinant Antibody (V2-179330) (CBMAB-A0629-YC)

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Target: ACO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat
Clone: V2-179330
Application*: IC, IF
Datasheet SDS

Mouse Anti-ACO2 Recombinant Antibody (V2-179328) (CBMAB-A0626-YC)

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Target: ACO2
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human, Mouse, Rat
Clone: V2-179328
Application*: E, IH, IP
Datasheet SDS

Mouse Anti-ACO2 Recombinant Antibody (V2-179327) (CBMAB-A0624-YC)

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Target: ACO2
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: V2-179327
Application*: F, IC, IF
Datasheet SDS

Mouse Anti-ACO2 Monoclonal Antibody (V2-5178) (CBMAB-0926-YC)

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Target: ACO2
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: V2-5178
Application*: E
Datasheet SDS

Mouse Recombinant ACO2 protein, GST Tag (V2LY-0526-LY7769)

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Target: ACO2
Expressed Host: Baculovirus-Insect Cells
Sequence: Amino Acid: 28-780
Tag: GST Tag
Datasheet SDS

Human Recombinant ACO2 protein, GST Tag (V2LY-0526-LY1901)

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Target: ACO2
Expressed Host: Baculovirus-Insect Cells
Sequence: Amino Acid: 28-780
Tag: GST Tag
Datasheet SDS

Rabbit Anti-ACO2 Recombinant Antibody (
8H4) (V2LY-0725-LY1062)

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Target: ACO2
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 8H4
Application*: E, IH
Datasheet SDS

Mouse Anti-ACO2 Recombinant Antibody (V2-6068) (CBMAB-0013CQ)

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Target: ACO2
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: V2-6068
Application*: WB, IF, IH
Datasheet SDS
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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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