PAH / PH Protein (His Tag)

28.03.2012

Produkt-Information von PAH / PH Protein

Synonym	PH, PKU, PKU1
Protein Construction	A DNA sequence encoding the human PAH (P00439) (Met 1- Lys 452)（415 Asn/Asp）was expressed, with a polyhistidine tag at the N-terminus.
Source	Human
Expression Host	Baculovirus-Insect cells

PAH / PH Protein QC Testing

Purity	> 90 % as determined by SDS-PAGE	SDS-PAGE: PAH / PH protein
Endotoxin	< 1.0 EU per μg of the protein as determined by the LAL method
Stability	Samples are stable for up to twelve months from date of receipt at -70℃
Predicted N terminal	Met
Molecular Mass	The recombinant human PAH consists of 471 amino acids and predicts a molecular mass of 54 kDa. It migrates as an approximately 50 KDa band in SDS-PAGE under reducing conditions.
Formulation	Lyophilized from 0.2μm filtered solution of 20mM Tris,500mM NaCl, pH 8.0, 10%glycerol Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA..

PAH / PH Protein Usage Guide

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 PAH / PH Protein

PAH (phenylalanine hydroxylase,,N-Histidine-tagged), also known as PH, is an enzyme which belongs to the biopterin-dependent aromatic amino acid hydroxylase family. It consists of three distinct domains: a regulatory N-terminal domain, the catalytic domain, and a C-terminal domain responsible for oligomerization of identical monomers. PAH is expressed both in the liver and the kidney, and there is some indication that it may be differentially regulated in these tissues. It catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PAH is a critical enzyme in phenylalanine metabolism and catalyzes the rate-limiting step in its complete catabolism to carbon dioxide and water. Deficiency of PAH results in autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol which usually causes mental retardation. They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.

References

Fitzpatrick PF. et al., 1999, Annu Rev Biochem. 68: 355-81.
Panay AJ. et al., 2011, Biochemistry. 50(11): 1928-33.
Bassan A. et al., 2003, Chemistry. 9 (1): 106-15.
Olsson E. et al., 2011, Chemistry. 17 (13): 3746-58.
Bassan A. et al., 2003, Chemistry. 9 (17): 4055-67.

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