The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
追加情報IHC-P: Use at a concentration of 3 µg/ml. Antigen retrieval is recommended: Place sample in 1X citrate buffer (pH 6.0) and microwave at 750W for 20 minutes, cool sample
WB: Use at a concentration of 1 µg/ml. Predicted molecular weight: 62 kDa.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
機能Plays a key role in glycolysis.
パスウェイCarbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 5/5.
関連疾患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 kinase-deficient 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.