MCCC2, Polyclonal Antibody

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

Polyclonal

IgG

Rabbit

Affinity Purification

Store at -20 degree C. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

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.

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.

Western Blot (WB), Immunohistochemistry (IHC)

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

Western blot analysis of extracts of various cell lines, using MCCC2 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 30s.

Immunohistochemistry of paraffin-embedded human prostate using MCCC2 antibody at dilution of 1:100 (40x lens).

Immunohistochemistry of paraffin-embedded rat brain using MCCC2 antibody at dilution of 1:100 (40x lens).

Immunohistochemistry of paraffin-embedded mouse stomach using MCCC2 antibody at dilution of 1:100 (40x lens).

61kDa

methylcrotonoyl-CoA carboxylase 2 (beta)

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

Chromosomal Location of Human Ortholog: 5q12-q13

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

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

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

Disease: 3-methylcrotonyl-coa Carboxylase 2 Deficiency

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