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Product Name GATM Chinese Name GATM蛋白抗体 Alias AGAT; AT; GATM; GATM_HUMAN; Glycine amidinotransferase; Glycine amidinotransferase, mitochondrial; L-arginine:glycine amidinotransferase; mitochondrial; Transamidinase. Research Area Tumour Cell biology Developmental biology Neurobiology Signal transduction The new supersedes the old Mitochondrion Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Zebrafish, Sheep, Cat, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 44kDa Cellular localization cytoplasmic The cell membrane Mitochondrion Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human GATM: 141-240/423 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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.
Function:
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.
Subunit:
Homodimer. There is an equilibrium between the monomeric and dimeric forms, shifted towards the side of the monomer.
Subcellular Location:
Mitochondrion inner membrane. Cytoplasm. The mitochondrial form is found in the intermembrane space probably attached to the outer side of the inner membrane.
Tissue Specificity:
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.
DISEASE:
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.
Similarity:
Belongs to the amidinotransferase family.
SWISS:
P50440
Gene ID:
2628
Database links:Entrez Gene: 2628 Human
Entrez Gene: 67092 Mouse
Entrez Gene: 266799 Zebrafish
Omim: 602360 Human
SwissProt: P50440 Human
SwissProt: Q9D964 Mouse
SwissProt: Q6PH19 Zebrafish
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