Full Product Name
MCCC1 Antibody, HRP conjugated
Product Synonym Names
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
Product Gene Name
anti-MCCC1 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q96RQ3
Purity/Purification
>95%, Protein G purified
Immunogen
Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (1-200AA)
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Preparation and Storage
Upon receipt, store at -20 degree C or -80 degree C. Avoid repeated freeze.
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.
Related Product Information for
anti-MCCC1 antibody
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.
Product Categories/Family for anti-MCCC1 antibody
Tags & Cell Markers; Metabolism; Signal transduction
Applications Tested/Suitable for anti-MCCC1 antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for MCCC1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_001280202.1
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NCBI GenBank Nucleotide #
NM_001293273.1
[Other Products]
UniProt Primary Accession #
Q96RQ3
[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 2
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]
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
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.
Research Articles on MCCC1
1. This study reports data mainly obtained from the Portuguese newborn screening program collected over a ten-year period. Analysis of the MCCC1 and MCCC2 genes yielded 26 previously unreported mutations and a variant of clinically unknown significance.
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
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.
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