Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; MCCase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; MCCC1; MCCA

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

Polyclonal

IgG

Rabbit

Antigen Affinity Purified

Liquid

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-MCCC1 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.

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.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Western blot
All lanes: MCCC1 antibody at 0.89 ug/ml
Lane 1: HepG-2 whole cell lysate
Lane 2: 293T whole cell lysate
Lane 3: Hela whole cell lysate
Lane 4: MCF7 whole cell lysate
Lane 5: A549 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 80 kDa
Observed band size: 80 kDa

Immunohistochemistry of paraffin-embedded human colon cancer using MBS7044109 at dilution of 1:100

80,473 Da

methylcrotonoyl-CoA carboxylase 1

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

MCCA; MCCase subunit alpha??[Similar Products]

MCCA_HUMAN

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. [provided by RefSeq, Jul 2008]

MCCC1: Defects in MCCC1 are the cause of 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 *****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.

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

Chromosomal Location of Human Ortholog: 3q27

Cellular Component: cytosol; mitochondrial matrix; mitochondrion

Molecular Function: methylcrotonoyl-CoA carboxylase activity; protein binding

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

Disease: 3-methylcrotonyl-coa Carboxylase 1 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.