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MCCC1 Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a partial recombinant MCCC1.
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, E |
|---|---|
| Primary Accession | Q96RQ3 |
| Other Accession | NM_020166 |
| Reactivity | Human |
| Host | mouse |
| Clonality | Monoclonal |
| Isotype | IgG2a Kappa |
| Clone Names | 2G8 |
| Calculated MW | 80473 Da |
| Gene ID | 56922 |
|---|---|
| Other 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 |
| Target/Specificity | MCCC1 (NP_064551, 526 a.a. ~ 625 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. |
| Dilution | WB~~1:500~1000 |
| Format | Clear, colorless solution in phosphate buffered saline, pH 7.2 . |
| Storage | Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing. |
| Precautions | MCCC1 Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures. |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score. Rose JE, et al. Mol Med, 2010 Jul-Aug. PMID 20379614.Cryptic exon activation by disruption of exon splice enhancer: novel mechanism causing 3-methylcrotonyl-CoA carboxylase deficiency. Stucki M, et al. J Biol Chem, 2009 Oct 16. PMID 19706617.3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family. Eminoglu FT, et al. J Child Neurol, 2009 Apr. PMID 19339287.Toward a confocal subcellular atlas of the human proteome. Barbe L, et al. Mol Cell Proteomics, 2008 Mar. PMID 18029348.Novel mutations in five Japanese patients with 3-methylcrotonyl-CoA carboxylase deficiency. Uematsu M, et al. J Hum Genet, 2007. PMID 17968484.
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