Hpd, also known as 4-hydroxyphenylpyruvate dioxygenase, is a 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. Source: Recombinant protein corresponding to aa1-393 from mouse Hpd, fused to His-Tag at N-terminal, expressed in E. coli. Molecular Weight: ~47.4kD (416aa) (confirmed by MALDI-TOF) Enzyme Activity: Not determined. This product is recommended for use in applications that do not require a catalytically active form of the protein. AA Sequence: MGSSHHHHHH SSGLVPRGSH MGSMTTYNNK GPKPERGRFL HFHSVTFWVG NAKQAASFYC NKMGFEPLAY RGLETGSREV VSHVIKQGKI VFVLCSALNP WNKEMGDHLV KHGDGVKDIA FEVEDCDHIV QKARERGAKI VREPWVEQDK FGKVKFAVLQ TYGDTTHTLV EKINYTGRFL PGFEAPTYKD TLLPKLPRCN LEIIDHIVGN QPDQEMQSAS EWYLKNLQFH RFWSVDDTQV HTEYSSLRSI VVTNYEESIK MPINEPAPGR KKSQIQEYVD YNGGAGVQHI ALKTEDIITA IRHLRERGTE FLAAPSSYYK LLRENLKSAK IQVKESMDVL EELHILVDYD EKGYLLQIFT KPMQDRPTLF LEVIQRHNHQ GFGAGNFNSL FKAFEEEQAL RGNLTDLEPN GVRSGM Endotoxin: <1EU/1ug (LAL) Storage and Stability: May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
