IVD
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2017]
Full Name
Isovaleryl-CoA Dehydrogenase
Function
Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268).
To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA (PubMed:7640268).
To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA (PubMed:7640268).
Biological Process
Branched-chain amino acid catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Fatty acid beta-oxidation using acyl-CoA dehydrogenaseManual Assertion Based On ExperimentIDA:BHF-UCL
Leucine catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Fatty acid beta-oxidation using acyl-CoA dehydrogenaseManual Assertion Based On ExperimentIDA:BHF-UCL
Leucine catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Isovaleric acidemia (IVA):
A metabolic disorder characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.
A metabolic disorder characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.
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Anti-IVD antibodies
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Target: IVD
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: A704
Application*: ELISA, IF, IHC, WB
Target: IVD
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CF251
Application*: ELISA, WB, IHC, IF
Target: IVD
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Rat, Monkey
Clone: CBYY-I2094
Application*: WB, IF, F
Target: IVD
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBYY-I1933
Application*: WB, F
Target: IVD
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Dog, Rat, Monkey
Clone: CBYY-I1898
Application*: WB, IH, IF, F
Target: IVD
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Monkey, Rat
Clone: CBYY-I0989
Application*: F, IF, WB
Target: IVD
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 1F1
Application*: F, WB
Target: IVD
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Dog, Monkey, Rat
Clone: 1B10
Application*: F, IF, IH, P, WB
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For Research Use Only. Not For Clinical Use.
(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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