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).

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

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.

Anti-IVD antibodies

Mouse Anti-IVD Recombinant Antibody (CBYY-I2094) (CBMAB-I3264-YY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human, Dog, Rat, Monkey

Clone: CBYY-I2094

Application*: WB, IF, F

Mouse Anti-IVD Recombinant Antibody (CBYY-I1933) (CBMAB-I3103-YY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG2b

Specificity: Human

Clone: CBYY-I1933

Application*: WB, F

Mouse Anti-IVD Recombinant Antibody (CBYY-I1898) (CBMAB-I3068-YY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG2b

Specificity: Human, Dog, Rat, Monkey

Clone: CBYY-I1898

Application*: WB, IH, IF, F

Mouse Anti-IVD Recombinant Antibody (CBYY-I0989) (CBMAB-I2157-YY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human, Dog, Monkey, Rat

Clone: CBYY-I0989

Application*: F, IF, WB

Mouse Anti-IVD Recombinant Antibody (1F1) (CBMAB-I0116-YY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG2b

Specificity: Human

Clone: 1F1

Application*: F, WB

Mouse Anti-IVD Recombinant Antibody (1B10) (CBMAB-I0115-YY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG2b

Specificity: Human, Dog, Monkey, Rat

Clone: 1B10

Application*: F, IF, IH, P, WB

Mouse Anti-IVD Recombinant Antibody (A704) (CBMAB-AP10927LY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human

Clone: A704

Application*: ELISA, IF, IHC, WB

Mouse Anti-IVD Recombinant Antibody (CF251) (CBMAB-FT251LY)

Datasheet

SDS

Target: IVD

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human

Clone: CF251

Application*: ELISA, WB, IHC, IF

For Research Use Only. Not For Clinical Use.

(P): Predicted

* Abbreviations

IFImmunofluorescence

IHImmunohistochemistry

IPImmunoprecipitation

WBWestern Blot

EELISA

MMicroarray

CIChromatin Immunoprecipitation

FFlow Cytometry

FNFunction Assay

IDImmunodiffusion

RRadioimmunoassay

TCTissue Culture

GSGel Supershift

NNeutralization

BBlocking

AActivation

IInhibition

DDepletion

ESELISpot

DBDot Blot

MCMass Cytometry/CyTOF

CTCytotoxicity

SStimulation

AGAgonist

APApoptosis

IMImmunomicroscopy

BABioassay

CSCostimulation

EMElectron Microscopy

IEImmunoelectrophoresis

PAPeptide Array

ICImmunocytochemistry

PEPeptide ELISA

MDMeDIP

SHIn situ hybridization

IAEnzyme Immunoassay

SEsandwich ELISA

PLProximity Ligation Assay

ECELISA(Cap)

EDELISA(Det)

BIBioimaging

IOImmunoassay

LFLateral Flow Immunoassay

LALuminex Assay

CImmunohistochemistry-Frozen Sections

PImmunohistologyp-Paraffin Sections

ISIntracellular Staining for Flow Cytometry

MSElectrophoretic Mobility Shift Assay

RIRNA Binding Protein Immunoprecipitation (RIP)