PHYH Protein (Native)

10.12.2012

Sino Biological Inc. liefert PHYH Protein (Native) mit höherer Qualität und günstiger Kosten im Vergleich mit anderen globalen Lieferanten.

Weitere Informationen über PHYH Protein (Native) lesen Sie bitte: http://www.sinobiological.com/PHYH-Protein-g-9976.html

Produkt-Information von PHYH Protein

Synonym	PHYH, PAHX
Protein Construction	A DNA sequence encoding the human PHYH (O14832) (Ser31-Leu338) was expressed, with a N-terminal Met.
Source	Human
Expression Host	E.coli

QC Testing von PHYH Protein

Purity	> 80 % as determined by SDS-PAGE	SDS-PAGE: PHYH protein
Endotoxin	Please contact us for more information.
Stability	Samples are stable for up to twelve months from date of receipt at -70℃
Predicted N terminal	Met
Molecular Mass	The recombinant human PHYH consists of 309 amino acids and predicts a molecular mass of 35.6 KDa. It migrates as an approximately 26-32 KDa band in SDS-PAGE under reducing conditions.
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 and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.

Usage Guide von PHYH Protein

Storage	Store it under sterile conditions at -70℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
Reconstitution	A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.

Beschreibung von PHYH Protein

PHYH is a member of 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. It is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. PHYH has three substrates which are known as phytanoyl-CoA, 2-oxoglutarate, and O2, while its three products are 2-hydroxyphytanoyl-CoA, succinate, and CO2. PHYH 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.

References

Mihalik SJ. et al., 1997, Nat Genet. 17 (2): 185-9.
McDonough MA. et al., 2005, J Biol Chem. 280 (49): 41101-10.
Jansen GA. et al., 1998, Clin Chim Acta. 271 (2): 203-11.