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MCCC1

This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.

Full Name

METHYLCROTONOYL-COA CARBOXYLASE 1

Function

Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.

Biological Process

Biotin metabolic process Source: UniProtKB
Branched-chain amino acid catabolic process Source: ComplexPortal
Leucine catabolic process Source: ParkinsonsUK-UCL

Cellular Location

Mitochondrion matrix

Involvement in disease

3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D):
An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.

PTM

Acetylated.

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Anti-MCCC1 antibodies

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Mouse Anti-MCCC1 Recombinant Antibody (CBFYM-1864) (CBMAB-M2035-FY)

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Target: MCCC1

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human

Clone: CBFYM-1864

Application*: WB

Mouse Anti-MCCC1 Recombinant Antibody (CBFYM-0602) (CBMAB-M0735-FY)

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Target: MCCC1

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: CBFYM-0602

Application*: E, WB

Human MCCC1 ELISA Kit (V2LY-0626-LY4886)

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Target: MCCC1

Sensitivity: 0.0074 ng/mL

Detection Range: 0.015-3 ng/mL

Sample Type: Serum, Plasma, cell culture supernates

Specificity: Human

Assay Type: Sandwich

Reactivity: Human

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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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