Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1) (AA 526-626) antibody

Details for Product No. ABIN395693
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Synonyms MCC-B, MCCA, 1810045E08Rik, 2310058B18Rik, MCCalpha, Mcca, R75106
AA 526-626
(15), (13), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1)
(30), (5), (2)
(24), (6)
Clonality (Clone)
Monoclonal ()
(2), (2), (2), (2), (2), (2)
ELISA, Western Blotting (WB)
(29), (19), (4), (3)
Pubmed 5 references available
Quantity 0.1 mg
Shipping to United States ( )
Availability Will be delivered in 8 to 11 Business Days
Immunogen MCCC1 (NP_064551, 526 a.a. ~ 626 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 K
Clone 2G8
Isotype IgG2a kappa
Specificity MCCC1 (NP_064551, 526 a.a. ~ 626 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Alternative Name MCCC1 (MCCC1 Antibody Abstract)
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 80473 DA
Gene ID 12406
NCBI Accession NP_064551
Research Area Metabolism, Cell Structure
Application Notes ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000

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.

Restrictions For Research Use only
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Supplier Images
ELISA image for anti-Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1) (AA 526-626) antibody (ABIN395693) Detection limit for recombinant GST tagged MCCC1 is approximately 0.1 ng/mL as a capt...
Background publications Rose, Behm, Drgon et al.: "Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score." in: Molecular medicine (Cambridge, Mass.), Vol. 16, Issue 7-8, pp. 247-53, 2010 (PubMed).

Stucki, Suormala, Fowler et al.: "Cryptic exon activation by disruption of exon splice enhancer: novel mechanism causing 3-methylcrotonyl-CoA carboxylase deficiency." in: The Journal of biological chemistry, Vol. 284, Issue 42, pp. 28953-7, 2009 (PubMed).

Eminoglu, Ozcelik, Okur et al.: "3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family." in: Journal of child neurology, Vol. 24, Issue 4, pp. 478-81, 2009 (PubMed).

Barbe, Lundberg, Oksvold et al.: "Toward a confocal subcellular atlas of the human proteome." in: Molecular & cellular proteomics : MCP, Vol. 7, Issue 3, pp. 499-508, 2008 (PubMed).

Uematsu, Sakamoto, Sugawara et al.: "Novel mutations in five Japanese patients with 3-methylcrotonyl-CoA carboxylase deficiency." in: Journal of human genetics, Vol. 52, Issue 12, pp. 1040-3, 2007 (PubMed).

Catalog No. ABIN395693
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