Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP50440
    • SpeciesHuman
    • SequenceMLRVRCLRGGSRGAEAVHYIGSRLGRTLTGWVQRTFQSTQAATASSRNSC AADDKATEPLPKDCPVSSYNEWDPLEEVIVGRAENACVPPFTIEVKANTY
    • Molecular weight37 kDa including tags
    • Amino acids1 to 100

Specifications

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.

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • FormLiquid
  • Additional notesProtein 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...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • AGAT
    • AT
    • GATM
    • GATM_HUMAN
    • Glycine amidinotransferase
    • Glycine amidinotransferase, mitochondrial
    • L-arginine:glycine amidinotransferase
    • mitochondrial
    • Transamidinase
    see all
  • FunctionCatalyzes 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.
  • Tissue specificityExpressed 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.
  • PathwayAmine and polyamine biosynthesis; creatine biosynthesis; creatine from L-arginine and glycine: step 1/2.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the amidinotransferase family.
  • DomainOne chain folds into a compact single domain composed of repeating units, five beta-beta-alpha-beta modules, which surround the central active site.
  • Cellular localizationMitochondrion 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 images

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

References for Recombinant Human GATM protein (ab116925)

ab116925 has not yet been referenced specifically in any publications.

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