Product Synonym Names
MCCB
Product Gene Name
anti-MCCC2 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9HCC0
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Affinity purification
Immunogen
Recombinant protein of human MCCC2
Storage Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Preparation and Storage
Aliquot and store at-20 degree C. Avoid repeated freeze/thaw cycles.
Other Notes
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.
Related Product Information for
anti-MCCC2 antibody
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.
Applications Tested/Suitable for anti-MCCC2 antibody
Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-MCCC2 antibody
WB: 1:500-1:2000
IHC: 1:50-1:200
NCBI/Uniprot data below describe general gene information for MCCC2. It may not necessarily be applicable to this product.
NCBI Accession #
NP_071415.1
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NCBI GenBank Nucleotide #
NM_022132.4
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UniProt Primary Accession #
Q9HCC0
[Other Products]
UniProt Secondary Accession #
Q96C27; Q9Y4L7; A6NIY9[Other Products]
UniProt Related Accession #
Q9HCC0[Other Products]
NCBI Official Full Name
methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform 1
NCBI Official Synonym Full Names
methylcrotonoyl-CoA carboxylase 2
NCBI Official Symbol
MCCC2 [Similar Products]
NCBI Official Synonym Symbols
MCCB
[Similar Products]
NCBI Protein Information
methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
UniProt Protein Name
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
UniProt Synonym Protein Names
3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
Protein Family
Methylcrotonoyl-CoA carboxylase
UniProt Gene Name
MCCC2 [Similar Products]
UniProt Synonym Gene Names
MCCB; MCCase subunit beta [Similar Products]
NCBI Summary for MCCC2
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. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, May 2018]
UniProt Comments for MCCC2
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.
Research Articles on MCCC2
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
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Disclaimer
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