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    SPR/Sepiapterin reductase Antibody, Biotin Conjugated

    Catalog # bs-11784R-Biotin
    Product Name SPR/Sepiapterin reductase Antibody, Biotin Conjugated
    Applications WB, IHC-P
    Reactivity Human, Mouse, Rat
    Conjugation Biotin
    Host Rabbit
    Source KLH conjugated synthetic peptide derived from human Sepiapterin reductase
    Clonality Polyclonal
    Isotype IgG
    Concentration 1ug/ul
    Purification Purified by Protein A.
    Storage Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for 12 months.
    Synonyms SDR38C1; Sepiapterin reductase 7,8 dihydrobiopterin:NADP+ oxidoreductase; Sepiapterin reductase; Short chain dehydrogenase/reductase family 38C, member 1; SPR; SPRE_HUMAN.
    Background SPR, also known as sepiapterin reductase, is a homodimeric cytoplasmic protein that belongs to the sepiapterin reductase family. SPR functions as an NADH-dependent aldo-keto reductase and specifically catalyzes the reduction of pteridine derivatives. In addition, SPR plays an important role in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the final reduction step of the synthesis pathway. BH4 is an essential cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine) and is required for proper dopamine synthesis. Mutations in the gene encoding SPR can cause sepiapterin reductase deficiency, a monoamine neurotransmitter deficiency without hyperphenylalaninemia. Sepiapterin reductase deficiency interferes with BH4 synthesis, resulting in DOPA-responsive dystonia and a variety of other human diseases. In addition, SPR mRNA expression is increased in the brain of Parkinson’s Disease (PD) patients, suggesting that SPR may play a role in PD.