Recombinant人PKLR protein (ab112726)

概述

描述

  • 性质
    Recombinant
  • 来源
    Escherichia coli
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 分子量
      41 kDa

技术指标

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.

  • 应用

    Western blot

  • 形式
    Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 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.
  • 序列相似性
    Belongs to the pyruvate kinase family.
  • Information by UniProt

Recombinant Human PKLR protein (ab112726)参考文献

ab112726 has not yet been referenced specifically in any publications.

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