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

Details for Product No. ABIN395693
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Antigen
Synonyms MCC-B, MCCA, 1810045E08Rik, 2310058B18Rik, MCCalpha, Mcca, R75106
Epitope
AA 526-626
(7), (6), (2), (1), (1), (1), (1), (1), (1)
Reactivity
Human
(18), (5), (2)
Host
Mouse
(16), (2)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1)
Application
ELISA, Western Blotting (WB)
(18), (11), (4), (1)
Pubmed 5 references available
Quantity 0.1 mg
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Catalog No. ABIN395693
450.00 $
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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
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
Comment

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
anti-Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1) (AA 526-626) antibody Detection limit for recombinant GST tagged MCCC1 is approximately 0.1 ng/mL as a capture antibody.
Background publications 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).

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).

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).

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).

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).

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