Cy5.5 Rabbit anti-Human, Mouse, Rat SPR/Sepiapterin reductase Antibody, Cy5.5 Conjugated [IgG]

SDR38C1; Sepiapterin reductase 7,8 dihydrobiopterin:NADP+ oxidoreductase; Sepiapterin reductase; Short chain dehydrogenase/reductase family 38C, member 1; SPR; SPRE_HUMAN.

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.