Size: 100μg

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.

Exp date: 12 months

Category ID_II: Recombinant Proteins

Category ID_III: Others

Abbreviation:

Target Synonym: LN1;LNAP1;PAHX;PHYH1;RD

Research Areas: Signal Transduction;Neuroscience;Cancer;metabolism;

Conjugation:

Target Species: Human

Expression Host: E.coli

Application:

Fusion tag: None

UNIProt ID: O14832

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.

Concentration:

Activity: Not validated for activity

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% Tween 80 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

ObservedMW: 26-32 kDa