Synthetic peptide, corresponding to residues in Human SPR (UniProt: P35270).
A549, MCF7, HepG2 and HeLa cell lysates; Human thyroid gland carcinoma tissue; HeLa and MCF7 cells.
This product is a recombinant rabbit monoclonal antibody.
We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated "PUR" on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.
Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents
1/100 - 1/250. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
1/100 - 1/250.
1/10 - 1/100.
1/100 - 1/500.
ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.
Involvement in disease
Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD) [MIM:612716]. 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. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures.
Belongs to the sepiapterin reductase family.
In vitro phosphorylation of Ser-213 by CaMK2 does not change kinetic parameters.