Flow Cytometry (FC/FACS)
(Flow Cytometric analysis of HEK293T cells transfected with either recombinant SPR protein (red) or empty vector (blue) stained using SPR antibody)
Mouse SPR Monoclonal Antibody | anti-SPR antibody
SPR antibody
IF: 1:100
IHC: 1:150
WB: 1:500-2000
Flow Cytometry (FC/FACS)
(Flow Cytometric analysis of HEK293T cells transfected with either recombinant SPR protein (red) or empty vector (blue) stained using SPR antibody)
Flow Cytometry (FC/FACS)
(Flow Cytometric analysis of HEK293T cells transfected with either recombinant SPR protein (red) or empty vector (blue) stained using SPR antibody)
Western Blot (WB)
(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant SPR protein (Right) or empty vector (Left) detected with SPR antibody)
Western Blot (WB)
(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant SPR protein (Right) or empty vector (Left) detected with SPR antibody)
Immunohistochemistry (IHC)
(Immunohistochemical analysis of SPR protein in paraffin embedded Adenocarcinoma of Human colon tissue using SPR antibody)
Immunohistochemistry (IHC)
(Immunohistochemical analysis of SPR protein in paraffin embedded Adenocarcinoma of Human colon tissue using SPR antibody)
Immunofluorescence (IF)
(Immunofluorescent staining of COS7 cells transiently transfected with recombinant SPR protein using SPR antibody)
Immunofluorescence (IF)
(Immunofluorescent staining of COS7 cells transiently transfected with recombinant SPR protein using SPR antibody)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an aldo-keto reductase that catalyzes the NADPH-dependent reduction of pteridine derivatives and is important in the biosynthesis of tetrahydrobiopterin (BH4). Mutations in this gene result in DOPA-responsive dystonia due to sepiaterin reductase deficiency. A pseudogene has been identified on chromosome 1. [provided by RefSeq, Jul 2008]
Uniprot Description
SPR: Catalyzes 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. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Belongs to the sepiapterin reductase family.
Protein type: EC 1.1.1.153; Oxidoreductase; Cofactor and Vitamin Metabolism - folate biosynthesis
Chromosomal Location of Human Ortholog: 2p14-p12
Cellular Component: nucleoplasm; mitochondrion; cytoplasm; cytosol
Molecular Function: aldo-keto reductase activity; sepiapterin reductase activity; NADP binding
Biological Process: voluntary musculoskeletal movement; tetrahydrobiopterin biosynthetic process; death; pteridine metabolic process; regulation of multicellular organism growth; norepinephrine metabolic process; nitric oxide metabolic process; dopamine metabolic process; L-phenylalanine metabolic process; neuron morphogenesis during differentiation; regulation of nitric-oxide synthase activity; nitric oxide biosynthetic process; serotonin metabolic process
Disease: Dystonia, Dopa-responsive, Due To Sepiapterin Reductase Deficiency
Research Articles on SPR
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Product Notes
The SPR spr (Catalog #AAA833589) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's SPR can be used in a range of immunoassay formats including, but not limited to, Flow Cytometry (FC/FACS), Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB). FC: 1:100 IF: 1:100 IHC: 1:150 WB: 1:500-2000. Researchers should empirically determine the suitability of the SPR spr for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "SPR, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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