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GATM Rabbit pAb (bs-13296R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-13296R
英文名称 GATM Rabbit pAb
中文名称 GATM蛋白抗体
别    名 AGAT; AT; GATM; GATM_HUMAN; Glycine amidinotransferase; Glycine amidinotransferase, mitochondrial; L-arginine:glycine amidinotransferase; mitochondrial; Transamidinase.  
研究领域 肿瘤  细胞生物  发育生物学  神经生物学  信号转导  新陈代谢  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Pig,Sheep,Zebrafish,Dog,Cat)
产品应用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 44 kDa
检测分子量
细胞定位 细胞浆 细胞膜 线粒体
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GATM: 141-240/423 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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: 81660 Rat

Entrez Gene: 266799 Zebrafish

Omim: 602360 Human

SwissProt: P50440 Human

SwissProt: Q9D964 Mouse

SwissProt: P50442 Rat

SwissProt: Q6PH19 Zebrafish



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