The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
生物活性Specific activity: >0.1 unit/mg. One unit will form 1.0 umol of phospho(enol)pyruvate to pyruvate per minute at pH 7.5 at 37°C. Activity Assay:
Prepare a 1.45 ml reaction mixture into a suitable container.
- Reaction mixture: 100mM Tris-HCl pH 7.5, 7.6 mM ADP, 15 mM MgCl2, 74 mM KCl, 0.2 mM Beta-NADH, 5.2 mM PEP, 0.025 units Recombinant LDHA protein (ab93699)
Add 50ul of recombinant PKLR protein solution with various concentrations (0.5ug, 1ug)
Read the decrease in A340nm in kinetic mode for 10 minutes.
% SDS-PAGE. ab100984 is purified using conventional chromatography techniques.
Concentration information loading...
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
This product is an active protein and may elicit a biological response in vivo, handle with caution.
Pyruvate kinase 1
Pyruvate kinase isozymes R/L
Pyruvate kinase liver and blood cell
Pyruvate kinase liver and RBC
Pyruvate kinase liver and red blood cell
Pyruvate kinase liver type
Pyruvate kinase type L
Pyruvate kinase, red cell type
R type/L type pyruvate kinase
R-type/L-type pyruvate kinase
Red cell/liver pyruvate kinase
功能Plays a key role in glycolysis.
通路Carbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 5/5.
疾病相关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.