Human HPD Recombinant Protein (RPPB1823)
- SKU:
- RPPB1823
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P32754
- Research Area:
- Enzymes
Description
| Product Name: | Human HPD Recombinant Protein |
| Product Code: | RPPB1823 |
| Size: | 10µg |
| Species: | Human |
| Target: | HPD |
| Synonyms: | 4HPPD, GLOD3, 4-HPPD, PPD, HPPDase, Glyoxalase Domain Containing 3, 4-HydroxyphenylpYruvate Dioxygenase. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | The HPD protein solution (1mg/1ml) is formulated in 20mM Tris-HCl buffer (pH 8.0) 1mM DTT, 50mM NaCl and 20% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°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. |
| Purity: | Greater than 90% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH 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 |
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.
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.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:EC 1.13.11.27; Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; Amino Acid Metabolism - phenylalanine; Oxidoreductase; Amino Acid Metabolism - tyrosine 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 |
| NCBI Summary: | 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] |
| UniProt Code: | P32754 |
| NCBI GenInfo Identifier: | 417144 |
| NCBI Gene ID: | 3242 |
| NCBI Accession: | P32754.2 |
| UniProt Secondary Accession: | P32754,Q13234, A8K461, B3KQ63, |
| UniProt Related Accession: | P32754 |
| Molecular Weight: | 393 |
| NCBI Full Name: | 4-hydroxyphenylpyruvate dioxygenase |
| NCBI Synonym Full Names: | 4-hydroxyphenylpyruvate dioxygenase |
| NCBI Official Symbol: | HPD�� |
| NCBI Official Synonym Symbols: | PPD; 4HPPD; GLOD3; 4-HPPD; HPPDASE�� |
| NCBI Protein Information: | 4-hydroxyphenylpyruvate dioxygenase; glyoxalase domain containing 3; 4-hydroxyphenylpyruvic acid oxidase |
| UniProt Protein Name: | 4-hydroxyphenylpyruvate dioxygenase |
| UniProt Synonym Protein Names: | 4-hydroxyphenylpyruvic acid oxidase; 4HPPD; HPD; HPPDase |
| UniProt Gene Name: | HPD�� |
| UniProt Entry Name: | HPPD_HUMAN |
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