PHYH Antibody (E-8): sc-376727
- PHYH Antibody (E-8) is a mouse monoclonal IgG1 κ PHYH antibody provided at 200 µg/ml
- specific for an epitope mapping between amino acids 145-181 within an internal region of PHYH of human origin
- PHYH Antibody (E-8) is recommended for detection of PHYH of mouse, rat and human origin by WB, IP, IF and ELISA; also reactive with additional species, including and equine, canine and bovine
- Anti-PHYH Antibody (E-8) 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 PHYH Antibody (E-8) for WB applications. These reagents are now offered in bundles with PHYH Antibody (E-8) (see ordering information below).
PHYH Antibody (E-8) is a mouse monoclonal IgG1 kappa light chain antibody that detects PHYH protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). anti-PHYH antibody (E-8) 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. PHYH protein, also known as phytanoyl-CoA 2-hydroxylase, RD, LN1, PAHX, or LNAP1, consists of 338 amino acids and is primarily localized in the peroxisome, where PHYH plays a crucial role in fatty acid metabolism. This enzyme is essential for the alpha-oxidation of 3-methyl branched fatty acids, catalyzing the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA using iron and ascorbate as cofactors. Proper functioning of PHYH is vital, as defects in the gene encoding PHYH are linked to Refsum disease, an autosomal recessive disorder that manifests with a range of symptoms including retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts, and cardiac impairment, typically emerging in the second or third decade of life. PHYH monoclonal antibody (E-8) in multiple formats enhances research applications, making PHYH (E-8) antibody an invaluable tool for studying PHYH implications in health and disease.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
PHYH Antibody (E-8) References:
- Phytanoyl-CoA hydroxylase deficiency. Enzymological and molecular basis of classical Refsum disease. | Jansen, GA., et al. 1999. Adv Exp Med Biol. 466: 371-6. PMID: 10709665
- Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease. | Mukherji, M., et al. 2001. Hum Mol Genet. 10: 1971-82. PMID: 11555634
- A novel murine long-chain acyl-CoA synthetase expressed in brain participates in neuronal cell proliferation. | Kee, HJ., et al. 2003. Biochem Biophys Res Commun. 305: 925-33. PMID: 12767919
- Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase: implications for Refsum disease? | Foulon, V., et al. 2003. J Lipid Res. 44: 2349-55. PMID: 12923223
- Molecular basis of Refsum disease: sequence variations in phytanoyl-CoA hydroxylase (PHYH) and the PTS2 receptor (PEX7). | Jansen, GA., et al. 2004. Hum Mutat. 23: 209-18. PMID: 14974078
- Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands. | Searls, T., et al. 2005. J Lipid Res. 46: 1660-7. PMID: 15930519
- Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease. | McDonough, MA., et al. 2005. J Biol Chem. 280: 41101-10. PMID: 16186124
- Phytanoyl-coenzyme A hydroxylase deficiency -- the enzyme defect in Refsum's disease. | Jansen, GA., et al. 1997. N Engl J Med. 337: 133-4. PMID: 9221344
- Identification of PAHX, a Refsum disease gene. | Mihalik, SJ., et al. 1997. Nat Genet. 17: 185-9. PMID: 9326939
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
PHYH Antibody (E-8) | sc-376727 | 200 µg/ml | $322.00 | |||
PHYH Antibody (E-8): m-IgG Fc BP-HRP Bundle | sc-529927 | 200 µg Ab; 10 µg BP | $361.00 | |||
PHYH Antibody (E-8): m-IgGκ BP-HRP Bundle | sc-523067 | 200 µg Ab, 40 µg BP | $361.00 | |||
PHYH Antibody (E-8): m-IgG1 BP-HRP Bundle | sc-543770 | 200 µg Ab; 20 µg BP | $361.00 | |||
PHYH Antibody (E-8) AC | sc-376727 AC | 500 µg/ml, 25% agarose | $424.00 | |||
PHYH Antibody (E-8) HRP | sc-376727 HRP | 200 µg/ml | $322.00 | |||
PHYH Antibody (E-8) FITC | sc-376727 FITC | 200 µg/ml | $336.00 | |||
PHYH Antibody (E-8) PE | sc-376727 PE | 200 µg/ml | $349.00 | |||
PHYH Antibody (E-8) Alexa Fluor® 488 | sc-376727 AF488 | 200 µg/ml | $364.00 | |||
PHYH Antibody (E-8) Alexa Fluor® 546 | sc-376727 AF546 | 200 µg/ml | $364.00 | |||
PHYH Antibody (E-8) Alexa Fluor® 594 | sc-376727 AF594 | 200 µg/ml | $364.00 | |||
PHYH Antibody (E-8) Alexa Fluor® 647 | sc-376727 AF647 | 200 µg/ml | $364.00 | |||
PHYH Antibody (E-8) Alexa Fluor® 680 | sc-376727 AF680 | 200 µg/ml | $364.00 | |||
PHYH Antibody (E-8) Alexa Fluor® 790 | sc-376727 AF790 | 200 µg/ml | $364.00 | |||
PHYH (E-8) Neutralizing Peptide | sc-376727 P | 100 µg/0.5 ml | $69.00 |