Recombinant SPR Monoclonal Antibody
Product Specifications
Background
SPR can catalyze the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD) . In the majority of cases, patients manifest progressive psychomotor retardation, dystonia and spasticity. Cognitive anomalies are also often present. The disease is due to severe dopamine and serotonin deficiencies in the central nervous system caused by a defect in BH4 synthesis.
Abbreviation
SPR
UniProt
P35270
Host
Rabbit
Reactivity
Human
Immunogen
Peptide. This information is proprietary to PTMab.
Target
SDR38C; member; OTTHUMP; SPR; SDR38C1;8 dihydrobiopterin:NADP+ oxidoreductase) ; member 1; OTTHUMP00000160199; Sepiapterin reductase; Sepiapterin reductase (7; Short chain dehydrogenase/reductase family 38C; SPRE
Clonality
Monoclonal,Recombinant
Clone
A748
Conjugation
Unconjugated
Applications
WB; IHC
Field of Research
Neuroscience; Signal Transduction; Metabolism
Purification
Protein A purified
Concentration
1 mg/mL
Dilution
WB 1:1000; IHC 1:100
Buffer
PBS, 50% glycerol, 0.05% Proclin 300, 0.05% protein protectant.
Shipping Conditions
Ice bag
Storage Conditions
Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.
Calculated Molecular Weight
28 kDa
Observed Molecular Weight
27 kDa
Isotype
IgG, κ
Available Sizes
Curated Selection
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