HPD Human; 4-Hydroxyphenylpyruvate Dioxygenase Human Recombinant; 4HPPD; GLOD3; 4-HPPD; PPD; HPPDase; Glyoxalase Domain Containing 3; 4-HydroxyphenylpYruvate Dioxygenase

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 90% as determined by SDS-PAGE.

The HPD protein solution (1mg/1ml) is formulated in 20mM Tris-HCl buffer (pH 8.0) 1mM DTT, 50mM NaCl and 20% glycerol.
Sterile Filtered clear solution.

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.

Small volumes of HPD recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: HPD produced in E Coli is a single, non-glycosylated polypeptide chain containing 413 amino acids (1-393a.a.) and having a molecular mass of 47kDa.HPD is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Introduction: 4-Hydroxyphenylpyruvate Dioxygenase Isoform-1 is an Fe-containing enzyme, which catalyzes the second reaction in the catabolism of tyrosine the conversion of 4-hydroxyphenylpyruvate to homogentisate. Present as a homodimer, HPD uses zinc as a cofactor to catalyze the third step in the conversion of L-phenylalanine to fumarate and acetoacetic acid. Flaws in the gene encoding HPD result in tyrosinemia type 3 and hawkinsinuria, two inborn defects of metabolism which are related to a number of symptoms, like mental retardation and seizures and hair and urine abnormalities.

ENZYMES; Enzymes; Oxygenase

40,497 Da

4-hydroxyphenylpyruvate dioxygenase

4-hydroxyphenylpyruvate dioxygenase; 4-hydroxyphenylpyruvic acid oxidase; glyoxalase domain containing 3

4-hydroxyphenylpyruvate dioxygenase

PPD; 4HPPD; HPD; HPPDase??[Similar Products]

HPPD_HUMAN

The protein encoded by this gene is an enzyme in the catabolic pathway of tyrosine. The encoded protein catalyzes the conversion of 4-hydroxyphenylpyruvate to homogentisate. Defects in this gene are a cause of tyrosinemia type 3 (TYRO3) and hawkinsinuria (HAWK). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2010]

HPD: Key enzyme in the degradation of tyrosine. Defects in HPD are the cause of tyrosinemia type 3 (TYRO3). TYRO3 is an inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, seizures and mild mental retardation. Defects in HPD are a cause of hawkinsinuria (HAWK). HAWK is an inborn error of tyrosine metabolism characterized by failure to thrive, persistent metabolic acidosis, fine and sparse hair, and excretion of the unusual cyclic amino acid metabolite, hawkinsin, in the urine. Belongs to the 4HPPD family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Amino Acid Metabolism - tyrosine; Oxidoreductase; Amino Acid Metabolism - phenylalanine; Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; EC 1.13.11.27

Chromosomal Location of Human Ortholog: 12q24.31

Cellular Component: cytosol

Molecular Function: 4-hydroxyphenylpyruvate dioxygenase activity; metal ion binding

Biological Process: L-phenylalanine catabolic process; tyrosine catabolic process

Disease: Hawkinsinuria; Tyrosinemia, Type Iii

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