Product Name	Anti-GAMT/PE-Cy3
Chinese Name	PE-Cy3标记的胍基乙酸N甲基转移酶抗体
Alias	GAMT; GAMT_HUMAN; Guanidinoacetate N methyltransferase; Guanidinoacetate N-methyltransferase; PIG2; TP53I2.
Research Area	Tumour  Cell biology  Neurobiology  Signal transduction  The new supersedes the old
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human,  (predicted: Mouse, Rat, Dog, Pig, )
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	26kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human GAMT
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: In the creatine biosynthesis pathway, glycine is converted to guanidinoacetate by amidinotransferase, and guanidinoacetate is then converted to creatine by Guanidinoacetate N-methyltransferase (GAMT). GAMT, a methyltransferase, uses S-adenosylmethionine as the methyl donor for this reaction. Methyltransferases are a type of transferase enzyme which transfers a methyl group to nucleic bases in DNA or amino acids in protein. Encoding a 236 amino acid protein, the human GAMT gene maps to chromosome 19p13.3. Defects in the GAMT gene leads to GAMT deficiency, which is associated with guanidinoacetate accumulation and decreased levels of creatine excretion in brain. Such biochemical changes are thought to lead to various neurological syndromes and muscular hypotonia. Tissue Specificity: Expressed in liver. DISEASE: Defects in GAMT are the cause of guanidinoacetate methyltransferase deficiency (GAMT deficiency) [MIM:612736]. GAMT deficiency is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, intractable seizures and movement disturbances, severe depletion of creatine/phosphocreatine in the brain, and accumulation of guanidinoacetic acid (GAA) in brain and body fluids. Similarity: Belongs to the RMT2 methyltransferase family. Database links: Clone ID: NP_000147.1 Human Entrez Gene: 2593 Human Entrez Gene: 25257 Rat Omim: 601240 Human SwissProt: Q14353 Human SwissProt: P10868 Rat Unigene: 81131 Human Unigene: 33890 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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