Recombinant人GATM protein (ab116925)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP50440
    • 种属Human
    • 序列MLRVRCLRGGSRGAEAVHYIGSRLGRTLTGWVQRTFQSTQAATASSRNSC AADDKATEPLPKDCPVSSYNEWDPLEEVIVGRAENACVPPFTIEVKANTY
    • 分子量37 kDa including tags
    • 氨基酸1 to 100

技术指标

Our Abpromise guarantee covers the use of ab116925 in the following tested applications.

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

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • AGAT
    • AT
    • GATM
    • GATM_HUMAN
    • Glycine amidinotransferase
    • Glycine amidinotransferase, mitochondrial
    • L-arginine:glycine amidinotransferase
    • mitochondrial
    • Transamidinase
    see all
  • 功能Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
  • 组织特异性Expressed in brain, heart, kidney, liver, lung, salivary gland and skeletal muscle tissue, with the highest expression in kidney. Biallelically expressed in placenta and fetal tissues.
  • 通路Amine and polyamine biosynthesis; creatine biosynthesis; creatine from L-arginine and glycine: step 1/2.
  • 疾病相关Defects in GATM are the cause of arginine:glycine amidinotransferase deficiency (AGAT deficiency) [MIM:612718]. AGAT deficiency is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, and severe depletion of creatine/phosphocreatine in the brain.
  • 序列相似性Belongs to the amidinotransferase family.
  • 结构域One chain folds into a compact single domain composed of repeating units, five beta-beta-alpha-beta modules, which surround the central active site.
  • 细胞定位Mitochondrion inner membrane. Cytoplasm. The mitochondrial form is found in the intermembrane space probably attached to the outer side of the inner membrane.
  • Information by UniProt

Recombinant Human GATM protein 图像

  • 12.5% SDS-PAGE image showing ab116925 at approx 36.63kDa. Stained with Coomassie Blue.

Recombinant Human GATM protein (ab116925)参考文献

ab116925 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab116925.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"