Recombinatie SPR monoklonaal antilichaam

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.

SPR

P35270

Human

Peptide. This information is proprietary to PTMab.

SDR38C; member; OTTHUMP; SPR; SDR38C1;8 dihydrobiopterin:NADP+ oxidoreductase) ; member 1; OTTHUMP00000160199; Sepiapterin reductase; Sepiapterin reductase (7; Short chain dehydrogenase/reductase family 38C; SPRE

A748

Unconjugated

WB; IHC

Neuroscience; Signal Transduction; Metabolism

Protein A purified

1 mg/mL

WB 1:1000; IHC 1:100

PBS, 50% glycerol, 0.05% Proclin 300, 0.05% protein protectant.

Ice bag

Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.

https://789.bio/eb/qvYIuVOY

28 kDa

27 kDa

IgG, κ