catalog number :
MBS8245218
products full name :
Anti-AGAT Antibody
products short name :
[AGAT]
products name syn :
[AGAT; Glycine amidinotransferase, mitochondrial; L-arginine:glycine amidinotransferase; Transamidinase]
other names :
[glycine amidinotransferase, mitochondrial isoform 1; Glycine amidinotransferase, mitochondrial; glycine amidinotransferase, mitochondrial; glycine amidinotransferase; L-arginine:glycine amidinotransferase; Transamidinase]
products gene name :
[GATM]
other gene names :
[GATM; GATM; AT; AGAT; CCDS3; AGAT]
uniprot entry name :
GATM_HUMAN
reactivity :
Human, Mouse, Rat
specificity :
Recognizes endogenous levels of AGAT protein.
purity :
The antibody was purified by immunogen affinity chromatography.
form :
Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
storage stability :
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC)
app notes :
WB: 1/500 - 1/2000; IHC: 1/50 - 1/200; IF/ICC: 1/50 - 1/200
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
image3 heading :
Immunofluorescence (IF)
other info1 :
Source: Rabbit. Immunogen: Recombinant full length protein of human AGAT
products description :
Rabbit polyclonal antibody to AGAT
ncbi acc num :
NP_001473.1
ncbi gb acc num :
NM_001482.2
ncbi mol weight :
44,943 Da
ncbi pathways :
Arginine And Proline Metabolism Pathway (82957); Arginine And Proline Metabolism Pathway (323); Creatine Metabolism Pathway (1270169); Creatine Pathway (413359); Creatine Pathway (468234); Glycine, Serine And Threonine Metabolism Pathway (82949); Glycine, Serine And Threonine Metabolism Pathway (313); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Amino Acids And Derivatives Pathway (1270158)
ncbi summary :
This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by mental retardation, language impairment, and behavioral disorders. [provided by RefSeq, Jul 2008]
uniprot summary :
GATM: 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. Defects in GATM are the cause of arginine:glycine amidinotransferase deficiency (AGAT deficiency). 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. Belongs to the amidinotransferase family. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Transferase; Amino Acid Metabolism - arginine and proline; Mitochondrial; EC 2.1.4.1; Amino Acid Metabolism - glycine, serine and threonine. Chromosomal Location of Human Ortholog: 15q21.1. Cellular Component: mitochondrial inner membrane; mitochondrial intermembrane space. Molecular Function: glycine amidinotransferase activity. Biological Process: creatine biosynthetic process; creatine metabolic process. Disease: Cerebral Creatine Deficiency Syndrome 3