製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号P30613
    • 生物種Human
    • 分子量41 kDa

特性

Our Abpromise guarantee covers the use of ab112726 in the following tested applications.

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

  • アプリケーション

    Western blot

  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

    pH: 6.80
    Constituents: 1.58% Tris HCl, 4% SDS, 3.09% DTT, 0.2% Bromophenol blue, 20% Sucrose

関連情報

  • 別名
    • 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
  • 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.
  • 配列類似性Belongs to the pyruvate kinase family.
  • Information by UniProt

Recombinant Human PKLR protein (ab112726) 使用論文

ab112726 has not yet been referenced specifically in any publications.

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