Spr also known as Sepiapterin reductase, belongs to the Short-chain dehydrogenase/reductase (Sdr) family and also reduces various exogenous carbonyl compounds including phenylpropanedione. Spr is an essential enzyme for the biosynthesis of tetrahydrobiopterin, an essential cofactor for aromatic amino acid hydrolases including tyrosine hydroxylase, the rate-limiting enzyme in dopamine synthesis. Defects in Spr cause DOPA- responsive dystonia defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Source: Recombinant protein corresponding to aa1-262 from mouse Spr, fused to His-tag at N-terminal, expressed in E. coli. Molecular Weight: ~30.3kD (285aa), confirmed by MALDI-TOF Amino Acid Sequence: MGSSHHHHHH SSGLVPRGSH MGSMEAGGLG CAVCVLTGAS RGFGRALAPQ LARLLSPGSV MLVSARSESM LRQLKEELGA QQPDLKVVLA AADLGTEAGV QRLLSAVREL PRPEGLQRLL LINNAATLGD VSKGFLNVND LAEVNNYWAL NLTSMLCLTS GTLNAFQDSP GLSKTVVNIS SLCALQPYKG WGLYCAGKAA RDMLYQVLAA EEPSVRVLSY APGPLDNDMQ QLARETSKDP ELRSKLQKLK SDGALVDCGT SAQKLLGLLQ KDTFQSGAHV DFYDC 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 6 months after receipt at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
