Description
Aliases
L-arginine:glycine amidinotransferase, Transamidinase, GATM, AGAT
Antibody Type
Polyclonal Antibody
Species
Human
Uniprot ID
P50440
Immunogen
Recombinant human Glycine amidinotransferase, mitochondrial protein (124-423AA)
Raised In
Rabbit
Species Reactivity
Human,Mouse
Tested Applications
ELISA, WB, IHC;Recommended dilution:WB:1:500-5000, IHC:1:20-1:200
Background / 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.
Isotype
IgG
Conjugate
Unconjugated
Storage Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Form
Liquid
Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze.
Purity
Antigen Affinity Purified
Literature
[1]”Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency.”Nouioua S., Cheillan D., Zaouidi S., Salomons G.S., Amedjout N., Kessaci F., Boulahdour N., Hamadouche T., Tazir M.Neuromuscul. Disord. 23:670-674(2013). [2]”Biochemical, molecular, and clinical diagnoses of patients with cerebral creatine deficiency syndromes.”Comeaux M.S., Wang J., Wang G., Kleppe S., Zhang V.W., Schmitt E.S., Craigen W.J., Renaud D., Sun Q., Wong L.J.Mol. Genet. Metab. 109:260-268(2013). [3]”Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans.”Item C.B., Stockler-Ipsiroglu S., Stromberger C., Muhl A., Alessandri M.G., Bianchi M.C., Tosetti M., Fornai F., Cioni G.Am. J. Hum. Genet. 69:1127-1133(2001).
Additional information
| Size | 50μl, 100μl |
|---|
