{"product_id":"recombinant-human-phyh-protein-pksh030645","title":"Recombinant Human PHYH Protein - PKSH030645","description":"\u003cp\u003eRecombinant Human PHYH Protein\u003c\/p\u003e\n\n\u003cp\u003eSize: 100μg\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: PKSH030645-100\u003c\/p\u003e\n\n\u003cp\u003eCitations, Manuals and MSDS Available upon request.\u003c\/p\u003e\n\n\u003cp\u003eAbbreviation: PHYH\u003c\/p\u003e\n\n\u003cp\u003eTarget Synonym: LN1; LNAP1; PAHX; PHYH1; RD\u003c\/p\u003e\n\n\u003cp\u003eUNIProt ID: O14832\u003c\/p\u003e\n\n\u003cp\u003eResearch Areas: Signal Transduction; Neuroscience; Cancer; metabolism;\u003c\/p\u003e \n\n\u003cp\u003eTarget Species: Human\u003c\/p\u003e\n\n\u003cp\u003eExpression Host: E.coli\u003c\/p\u003e\n\n\u003cp\u003eFusion Tag: None\u003c\/p\u003e\n\n\u003cp\u003eAccession: O14832\u003c\/p\u003e\n\n\u003cp\u003eBackground: 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.\u003c\/p\u003e\n\n\u003cp\u003eSequence: Ser31-Leu338\u003c\/p\u003e\n\n\u003cp\u003ePurity: \u0026gt; 80 % as determined by reducing SDS-PAGE.\u003c\/p\u003e\n\n\u003cp\u003eFormulation: Lyophilized from sterile 20mM mops, 10% glycerol, 2mM DDT, 1mM EDTA, 0.2mM PMSF, 0.2M NaCl, pH 7.2\u003cbr\u003e\nNormally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.\u003cbr\u003e\nPlease refer to the specific buffer information in the printed manual.\u003c\/p\u003e\n\n\u003cp\u003eReconstitution: Please refer to the printed manual for detailed information.\u003c\/p\u003e\n\n\u003cp\u003eEndotoxin: Please contact us for more information.\u003c\/p\u003e\n\n\u003cp\u003eCalculated MW: 35.6 kDa\u003c\/p\u003e\n\n\u003cp\u003eObserved MW: 26-32 kDa\u003c\/p\u003e\n\n\u003cp\u003eStorage: 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 \u0026lt; -20℃ for 3 months.\u003c\/p\u003e\n\n\u003cp\u003eShipping: This product is provided as lyophilized powder which is shipped with ice packs.\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"Elabscience","offers":[{"title":"100μg","offer_id":43083641258163,"sku":"PKSH030645-100","price":1197.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/products\/logo_new_644a3059-d0f6-4b9d-9f90-362bf8e1fa36.png?v=1708945879","url":"https:\/\/afsbio.com\/products\/recombinant-human-phyh-protein-pksh030645","provider":"AFSBio Inc.","version":"1.0","type":"link"}