Product Name
MCCC1, Polyclonal Antibody
Popular Item
Full Product Name
MCCC1 Polyclonal Antibody
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
Species Reactivity
Human, Mouse
Purity/Purification
Affinity purification
Immunogen
Recombinant protein of human MCCC1
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Preparation and Storage
Store at -20 degree C. Avoid freeze / thaw cycles.
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.
Product Categories/Family for anti-MCCC1 antibody
Polyclonal
Applications Tested/Suitable for anti-MCCC1 antibody
Western Blot (WB)
Application Notes for anti-MCCC1 antibody
WB: 1:500 - 1:1000
Western Blot (WB) of anti-MCCC1 antibody
Western blot analysis of extracts of various cell lines, using MCCC1 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 90s.
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
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UniProt Primary Accession #
Q96RQ3
[Other Products]
UniProt Secondary Accession #
Q59ES4; Q9H959; Q9NS97[Other Products]
UniProt Related Accession #
Q96RQ3[Other Products]
Molecular Weight
Calculated MW: 80kDa
Molecular Weight: 725
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]
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: EC 6.4.1.4; Ligase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation
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; vitamin metabolic process; water-soluble vitamin metabolic 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
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Disclaimer
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