MCCB, Polyclonal Antibody

MCCB; Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; MCCase subunit beta; 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Recognizes endogenous levels of MCCB protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-MCCC2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to MCCB

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1/500 - 1/2000; IHC: 1/50 - 1/200

Western blot analysis of MCCB expression in Hela (A), HepG2 (B), mouse liver (C), mouse heart (D), rat brain (E) whole cell lysates.

Immunohistochemical analysis of MCCB staining in human prostate formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

57,519 Da

methylcrotonoyl-CoA carboxylase 2

methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

MCCB; MCCase subunit beta  [Similar Products]

MCCB_HUMAN

This gene encodes the small 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. [provided by RefSeq, Jul 2008]

MCCC2: Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D). An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic *****s. 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. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; EC 6.4.1.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Ligase

Chromosomal Location of Human Ortholog: 5q12-q13

Cellular Component: cytosol; mitochondrial matrix; mitochondrion

Molecular Function: ATP binding; methylcrotonoyl-CoA carboxylase activity; protein binding

Biological Process: biotin metabolic process; branched chain family amino acid catabolic process; coenzyme A metabolic process; leucine catabolic process

Disease: 3-methylcrotonyl-coa Carboxylase 2 Deficiency

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