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Rabbit Anti-GATM  antibody (bs-13296R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-13296R
英文名稱 Rabbit Anti-GATM  antibody
中文名稱 GATM蛋白抗體
別    名 AGAT; AT; GATM; GATM_HUMAN; Glycine amidinotransferase; Glycine amidinotransferase, mitochondrial; L-arginine:glycine amidinotransferase; mitochondrial; Transamidinase.  
研究領域 腫瘤  細胞生物  發育生物學  神經生物學  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Pig,Sheep,Zebrafish,Dog,Cat)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 44kDa
細胞定位 細胞漿 細胞膜 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GATM: 141-240/423 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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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