DATASHEET
Host:
Rabbit
Target Protein:
SPR/Sepiapterin reductase
Immunogen Range:
101-200/261
Clonality:
Polyclonal
Isotype:
IgG
Source:
KLH conjugated synthetic peptide derived from human Sepiapterin reductase
Purification:
Purified by Protein A.
Storage Buffer:
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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.
Conjugation:
PE
Excitation/ Emission:
496,564nm/578nm
Size:
100ul
Concentration:
1ug/ul
Applications:
WB(1:300-5000)
28
Human
Mouse
Rat
Predicted Cross Reactive Species:
Pig
Horse
Rabbit
For research use only. Not intended for diagnostic or therapeutic use.