Full Product Name
MCCC1 Rabbit Polyclonal
Product Gene Name
anti-MCCC1 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
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.
Applications Tested/Suitable for anti-MCCC1 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for MCCC1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_064551.3
[Other Products]
NCBI GenBank Nucleotide #
NM_020166.4
[Other Products]
UniProt Secondary Accession #
Q59ES4; Q9H959; Q9NS97[Other Products]
UniProt Related Accession #
Q96RQ3[Other Products]
Molecular Weight
80,473 Da
NCBI Official Full Name
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1
NCBI Official Synonym Full Names
methylcrotonoyl-CoA carboxylase 1
NCBI Official Symbol
MCCC1??[Similar Products]
NCBI Official Synonym Symbols
MCCA; MCC-B
??[Similar Products]
NCBI Protein Information
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
UniProt Protein Name
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
UniProt Synonym Protein Names
3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
UniProt Gene Name
MCCC1??[Similar Products]
UniProt Synonym Gene Names
MCCA; MCCase subunit alpha??[Similar Products]
UniProt Entry Name
MCCA_HUMAN
NCBI Summary for MCCC1
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]
UniProt Comments for MCCC1
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: Ligase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.4; Mitochondrial
Chromosomal Location of Human Ortholog: 3q27
Cellular Component: cytosol; mitochondrial inner membrane; mitochondrial matrix; mitochondrion
Molecular Function: ATP binding; biotin binding; biotin carboxylase activity; metal ion binding; methylcrotonoyl-CoA carboxylase activity; protein binding
Biological Process: biotin metabolic process; branched chain family amino acid catabolic process; leucine catabolic process
Disease: 3-methylcrotonyl-coa Carboxylase 1 Deficiency
Research Articles on MCCC1
1. Four new point mutations were detected in the MCCC1 gene in patients with maternal 3-methylcrotonyl coenzyme deficiency.
Precautions
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.
Disclaimer
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