Product datasheet Anti-PKLR antibody [AT1E3] ab119500 1 Image Overview Product name Anti-PKLR antibody [AT1E3] Description Mouse monoclonal [AT1E3] to PKLR Tested applications WB, ELISA Species reactivity Reacts with: Human Immunogen Recombinant fragment, corresponding to amino acids 47-574 of Human PKLR, purified from E. coli (NP_000289) Positive control 293T cell lysate Properties Form Liquid Storage instructions Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles. Storage buffer pH: 7.40 Preservative: 0.1% Sodium azide Constituent: 99% PBS Purity Protein G purified Clonality Monoclonal Clone number AT1E3 Isotype IgG1 Light chain type kappa Applications Our Abpromise guarantee covers the use of ab119500 in the following tested applications. The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user. Application Abreviews Notes WB 1/500. Predicted molecular weight: 62 kDa. ELISA Use at an assay dependent dilution. Target 1 Function Plays a key role in glycolysis. Pathway Carbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 5/5. Involvement in disease Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:102900]; also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP. Defects in PKLR are the cause of pyruvate kinase deficiency of red cells (PKRD) [MIM:266200]. A frequent cause of hereditary non-spherocytic hemolytic anemia. Clinically, pyruvate kinasedeficient patients suffer from a highly variable degree of chronic hemolysis, ranging from severe neonatal jaundice and fatal anemia at birth, severe transfusion-dependent chronic hemolysis, moderate hemolysis with exacerbation during infection, to a fully compensated hemolysis without apparent anemia. Sequence similarities Belongs to the pyruvate kinase family. Anti-PKLR antibody [AT1E3] images Anti-PKLR antibody [AT1E3] (ab119500) at 1/500 dilution + 293T cell lysate at 40 µg Secondary goat anti-mouse HRP developed using the ECL technique Predicted band size : 62 kDa Observed band size : 62 kDa Western blot - Anti-PKLR antibody [AT1E3] (ab119500) Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE" Our Abpromise to you: Quality guaranteed and expert technical support Replacement or refund for products not performing as stated on the datasheet Valid for 12 months from date of delivery Response to your inquiry within 24 hours We provide support in Chinese, English, French, German, Japanese and Spanish Extensive multi-media technical resources to help you We investigate all quality concerns to ensure our products perform to the highest standards If the product does not perform as described on this datasheet, we will offer a refund or replacement. For full details of the Abpromise, please visit http://www.abcam.com/abpromise or contact our technical team. Terms and conditions Guarantee only valid for products bought direct from Abcam or one of our authorized distributors 2 3