Recombinant Human PHYH Protein
Size: 100μg
Catalogue Numbers: PKSH030645-100
Citations, Manuals and MSDS Available upon request.
Abbreviation: PHYH
Target Synonym: LN1; LNAP1; PAHX; PHYH1; RD
UNIProt ID: O14832
Research Areas: Signal Transduction; Neuroscience; Cancer; metabolism;
Target Species: Human
Expression Host: E.coli
Fusion Tag: None
Accession: O14832
Background: PHYH belongs to the family of iron(II)-dependent oxygenases, which typically incorporate one atom of dioxygen into the substrate and one atom into the succinate carboxylate group. PHYH is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. It converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Defects in PHYH can cause Refsum disease (RD). RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues.
Sequence: Ser31-Leu338
Purity: > 80 % as determined by reducing SDS-PAGE.
Formulation: Lyophilized from sterile 20mM mops, 10% glycerol, 2mM DDT, 1mM EDTA, 0.2mM PMSF, 0.2M NaCl, pH 7.2
Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.
Reconstitution: Please refer to the printed manual for detailed information.
Endotoxin: Please contact us for more information.
Calculated MW: 35.6 kDa
Observed MW: 26-32 kDa
Storage: Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
Shipping: This product is provided as lyophilized powder which is shipped with ice packs.
Research Use Only