Overview

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionP30613
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMLTQELGTAFFQQQQLPAAMADTFLEHLCL LDIDSEPVAARSTSIIATIGPASRSVERLKEMIKAGMNIARLNFSHGSHE YHAESIANVREAVESFAGSPLSYRPVAIALDTKGPEIRTGILQGGPESEV ELVKGSQVLVTVDPAFRTRGNANTVWVDYPNIVRVVPVGGRIYIDDGLIS LVVQKIGPEGLVTQVENGGVLGSRKGVNLPGAQVDLPGLSEQDVRDLRFG VEHGVDIVFASFVRKASDVAAVRAALGPEGHGIKIISKIENHEGVKRFDE ILEVSDGIMVARGDLGIEIPAEKVFLAQKMMIGRCNLAGKPVVCATQMLE SMITKPRPTRAETSDVANAVLDGADCIMLSGETAKGNFPVEAVKMQHAIA REAEAAVYHRQLFEELRRAAPLSRDPTEVTAIGAVEAAFKCCAAAIIVLT TTGRSAQLLSRYRPRAAVIAVTRSAQAARQVHLCRGVFPLLYREPPEAIW ADDVDRRVQFGIESGKLRGFLRVGDLVIVVTGWRPGSGYTNIMRVLSIS
    • Molecular weight59 kDa including tags
    • Amino acids47 to 574
    • TagsHis tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab100984 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Biological activitySpecific 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:
    1. 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)
    2. Add 50ul of recombinant PKLR protein solution with various concentrations (0.5ug, 1ug)
    3. Read the decrease in A340nm in kinetic mode for 10 minutes.
  • Applications

    Functional Studies

    SDS-PAGE

  • Mass spectrometry
    MALDI-TOF
  • Purity> 90 % SDS-PAGE.
    ab100984 is purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 10% Glycerol, 0.2M Sodium chloride, 20mM Tris HCl, 1mM DTT, pH 8.0

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

General Info

  • Alternative names
    • EC 2.7.1.40
    • KPYR_HUMAN
    • L-PK
    • Pk-1
    • PK1
    • PKL
    • Pklg
    • Pklr
    • PKR
    • PKRL
    • 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-PK
    • R-type/L-type pyruvate kinase
    • Red cell/liver pyruvate kinase
    • RPK
    see all
  • FunctionPlays a key role in glycolysis.
  • PathwayCarbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 5/5.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the pyruvate kinase family.
  • Information by UniProt

Recombinant human PKLR protein images

  • 15% SDS-PAGE analysis of 3µg ab100984.

References for Recombinant human PKLR protein (ab100984)

ab100984 has not yet been referenced specifically in any publications.

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