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Recombinant Human PAH

Cat.No.: PAH-28935TH
Product Overview: Recombinant Full Length Human PAH expressed in Saccharomyces cerevisiae; amino acids 1-452; 452 amino acids, 51.9kDa.
Description: PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.
Form: Liquid
Purity: >90% by SDS-PAGE
Storage buffer: Preservative: NoneConstituents: 30% Glycerol, 0.5% Triton-X-100, 50mM HEPES, 30mM Glutathione, 100mM Sodium chloride, 1mM DTT, pH 7.5
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Sequences of amino acids: MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFS LKEEVGALAKVLRLFEENDVNLTHIESRPSRLKKDEYEFF THLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPW FPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQ FADIAYNYRHGQPIPRVEYMEEEKKTWGTVFKTLKSLYKT HACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTCTGF RLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPE PDICHELLGHVPLFSDRSFAQFSQEIGLASLGAPDEYIEK LATIYWFTVEFGLCKQGDSIKAYGAGLLSSFGELQYCLSE KPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVR NFAATIPRPFSVRYDPYTQRIEVLDNTQQLKILADSINSE IGILCSALQKIK
Sequence Similarities: Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.Contains 1 ACT domain.
Gene Name: PAH phenylalanine hydroxylase [ Homo sapiens ]
Official Symbol: PAH
Synonyms: PAH; phenylalanine hydroxylase; phenylalanine-4-hydroxylase; PH; phenylalanine 4 monooxygenase;
Gene ID: 5053
mRNA Refseq: NM_000277
Protein Refseq: NP_000268
MIM: 612349
Uniprot ID: P00439
Chromosome Location: 12q22-q24.2
Pathway: Biogenic Amine Synthesis, organism-specific biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of amino acids and derivatives, organism-specific biosystem; Phenylalanine and tyrosine catabolism, organism-specific biosystem;
Function: amino acid binding; iron ion binding; metal ion binding; phenylalanine 4-monooxygenase activity;

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