HPD, aa1- 393, Recombinant, Human (4-hydroxyphenylpyruvate Dioxygenase, 4-hydroxyphenylpyruvic Acid Oxidase, 4HPPD, HPPDase, PPD)

Supplier:

United States Biological

Cat no: H7033-02

HPD, aa1- 393, Recombinant, Human (4-hydroxyphenylpyruvate Dioxygenase, 4-hydroxyphenylpyruvic Acid Oxidase, 4HPPD, HPPDase, PPD)

4-hydroxyphenylpyruvate dioxygenase isoform 1, also known as HPD, is an Fe-containing enzyme, that catalyzes the second reaction in the catabolism of tyrosine the conversion of 4-hydroxyphenylpyruvate to homogentisate. Existing 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. Defects in the gene encoding HPD are the cause of tyrosinemia type 3 and hawkinsinuria, both of which are inborn errors of metabolism that are associated with a variety of symptoms, including mental retardation and seizures and hair and urine abnormalities.\n\nSource:\nRecombinant corresponding to aa1-393 of human HPD protein, fused to His-tag at N-terminus, expressed in E.coli.\n\nAA Sequence:\nMGSSHHHHHH SSGLVPRGSH MTTYSDKGAK PERGRFLHFH SVTFWVGNAK QAASFYCSKM GFEPLAYRGL ETGSREVVSH VIKQGKIVFV LSSALNPWNK EMGDHLVKHG DGVKDIAFEV EDCDYIVQKA RERGAKIMRE PWVEQDKFGK VKFAVLQTYG DTTHTLVEKM NYIGQFLPGY EAPAFMDPLL PKLPKCSLEM IDHIVGNQPD QEMVSASEWY LKNLQFHRFW SVDDTQVHTE YSSLRSIVVA NYEESIKMPI NEPAPGKKKS QIQEYVDYNG GAGVQHIALK TEDIITAIRH LRERGLEFLS VPSTYYKQLR EKLKTAKIKV KENIDALEEL KILVDYDEKG YLLQIFTKPV QDRPTLFLEV IQRHNHQGFG AGNFNSLFKA FEEEQNLRGN LTNMETNGVV PGM\n\nMolecular Weight: 47kD (413aa) confirmed by MALDI-TOF\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 6 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

H7033-02

Size

50ug

Form

Supplied as a liquid in 20mM Tris-HCl, pH 8.0, 1mM DTT, 20% glycerol, 50mM sodium chloride.

Purity

~90% (SDS-PAGE).

References

1. Wada GH., et al. (1975) J Biol Chem J. 250(17):6720-6. 2. Uetschi U., et al. (1997) Genomics. 44:292-299.

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SUPPLIER INFO

United States Biological

Address:

4 Technology Way, Salem, MA 01970, US

Phone:

+1 800 520 3011

Website:

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