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Methylcrotonoyl-CoA carboxylase

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Carboxyltransferase 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.

Below are the list of possible Methylcrotonoyl-CoA carboxylase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Methylcrotonoyl-CoA carboxylase beta chain

 Methylcrotonoyl-CoA carboxylase beta chain ELISA Kit
 Methylcrotonoyl-CoA carboxylase beta chain Recombinant
 Methylcrotonoyl-CoA carboxylase beta chain Antibody
Also known as 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).
MCCC2: Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D). An autosomal recessive disorder of leucine cata
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bolism. 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. 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

Cellular Component: mitochondrion

Molecular Function: methylcrotonoyl-CoA carboxylase activity

Biological Process: coenzyme A metabolic process
 Mccc2 ELISA Kit
 Mccc2 Recombinant
 Mccc2 Antibody
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Methylcrotonoyl-CoA carboxylase subunit alpha

 Methylcrotonoyl-CoA carboxylase subunit alpha ELISA Kit
 Methylcrotonoyl-CoA carboxylase subunit alpha Recombinant
 Methylcrotonoyl-CoA carboxylase subunit alpha Antibody
Also known as 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).
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.
 mccA ELISA Kit
 mccA Recombinant
 mccA Antibody
 mccc1 ELISA Kit
 mccc1 Recombinant
 mccc1 Antibody
 DDB_G0287377 ELISA Kit
 DDB_G0287377 Recombinant
 DDB_G0287377 Antibody
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