MCCC2, Polyclonal Antibody

Anti -MCCC2, CT (MCCC2, MCCB, Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial, 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.

Chromosome: 5; NC_000005.9 (70883115..70954533). Location: 5q12-q13

Polyclonal

IgG

Rabbit

Human

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

Antibodies; Abs to Mitochondrial Proteins

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500

61,333 Da[Similar Products]

methylcrotonoyl-CoA carboxylase 2 (beta)

methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; biotin carboxylase; MCCase subunit beta; 3-methylcrotonyl-CoA carboxylase 2; methylcrotonoyl-Coenzyme A carboxylase 2 (beta); 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase

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; EC 6.4.1.4; Ligase; Mitochondrial

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

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.