胍基乙酸狈甲基转移酶抗体
规格：1尘驳/1尘濒
英文名： GAMT
别名： GAMT; GAMT_HUMAN; Guanidinoacetate N methyltransferase; Guanidinoacetate N-methyltransferase; PIG2; TP53I2.
分子量： 26kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human GAMT
交叉反应：Human, Mouse, Rat, Dog, Pig,
细胞定位：
胍基乙酸狈甲基转移酶抗体产物介绍：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.胍基乙酸狈甲基转移酶抗体 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.胍基乙酸狈甲基转移酶抗体 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.
胍基乙酸狈甲基转移酶抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid