ACADSB Antibody (C-9): sc-398773
- ACADSB Antibody (C-9) is a mouse monoclonal IgG1 κ ACADSB antibody provided at 200 µg/ml
- raised against amino acids 368-411 mapping near the C-terminus of ACADSB of human origin
- ACADSB Antibody (C-9) is recommended for detection of ACADSB of human origin by WB, IP, IF and ELISA
- Anti-ACADSB Antibody (C-9) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG Fc BP-HRP, m-IgG1 BP-HRP and m-IgGκ BP-HRP are the preferred secondary detection reagents for ACADSB Antibody (C-9) for WB applications. These reagents are now offered in bundles with ACADSB Antibody (C-9) (see ordering information below).
ACADSB Antibody (C-9) is a mouse monoclonal IgG1 kappa light chain antibody that detects ACADSB protein of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-ACADSB antibody (C-9) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. The Acyl-CoA dehydrogenase (ACAD) family of enzymes plays a crucial role in the catabolism of fatty acids and amino acids, serving as a significant energy source for the heart and skeletal muscle. Specifically, short/branched chain acyl-CoA dehydrogenase (ACADSB), also known as 2-methylbutyryl-coenzyme A dehydrogenase, is a 432 amino acid protein that is ubiquitously expressed and forms a homotetramer within the mitochondrial matrix. ACADSB is essential for the degradation of L-isoleucine and exhibits the highest affinity for substrates such as (S)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA. Notably, post-translational modifications of ACADSB, including phosphorylation and acetylation, can influence enzymatic activity and stability, which is vital for maintaining metabolic homeostasis. Mutations in the gene encoding ACADSB lead to short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), an autosomal recessive disorder characterized by elevated levels of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine, with patients often presenting seizures and psychomotor delays as primary clinical features.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
ACADSB Antibody (C-9) References:
- 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. | Korman, SH., et al. 2005. Clin Chem. 51: 610-7. PMID: 15615815
- Inborn errors of isoleucine degradation: a review. | Korman, SH. 2006. Mol Genet Metab. 89: 289-99. PMID: 16950638
- Association of genetic polymorphisms of ACADSB and COMT with human hypertension. | Kamide, K., et al. 2007. J Hypertens. 25: 103-10. PMID: 17143180
- 2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report. | Kanavin, OJ., et al. 2007. J Med Case Rep. 1: 98. PMID: 17883863
- 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism. | Sass, JO., et al. 2008. Mol Genet Metab. 93: 30-5. PMID: 17945527
- Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family. | Rozen, R., et al. 1994. Genomics. 24: 280-7. PMID: 7698750
- Localization of short/branched chain acyl-CoA dehydrogenase (ACADSB) to human chromosome 10. | Arden, KC., et al. 1995. Genomics. 25: 743-5. PMID: 7759115
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
ACADSB Antibody (C-9) | sc-398773 | 200 µg/ml | $322.00 | |||
ACADSB Antibody (C-9): m-IgG Fc BP-HRP Bundle | sc-530923 | 200 µg Ab; 10 µg BP | $361.00 | |||
ACADSB Antibody (C-9): m-IgGκ BP-HRP Bundle | sc-524478 | 200 µg Ab, 40 µg BP | $361.00 | |||
ACADSB Antibody (C-9): m-IgG1 BP-HRP Bundle | sc-544320 | 200 µg Ab; 20 µg BP | $361.00 | |||
ACADSB Antibody (C-9) AC | sc-398773 AC | 500 µg/ml, 25% agarose | $424.00 | |||
ACADSB Antibody (C-9) HRP | sc-398773 HRP | 200 µg/ml | $322.00 | |||
ACADSB Antibody (C-9) FITC | sc-398773 FITC | 200 µg/ml | $336.00 | |||
ACADSB Antibody (C-9) PE | sc-398773 PE | 200 µg/ml | $349.00 | |||
ACADSB Antibody (C-9) Alexa Fluor® 488 | sc-398773 AF488 | 200 µg/ml | $364.00 | |||
ACADSB Antibody (C-9) Alexa Fluor® 546 | sc-398773 AF546 | 200 µg/ml | $364.00 | |||
ACADSB Antibody (C-9) Alexa Fluor® 594 | sc-398773 AF594 | 200 µg/ml | $364.00 | |||
ACADSB Antibody (C-9) Alexa Fluor® 647 | sc-398773 AF647 | 200 µg/ml | $364.00 | |||
ACADSB Antibody (C-9) Alexa Fluor® 680 | sc-398773 AF680 | 200 µg/ml | $364.00 | |||
ACADSB Antibody (C-9) Alexa Fluor® 790 | sc-398773 AF790 | 200 µg/ml | $364.00 |