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AMPD3 Rabbit pAb (bs-9517R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
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產品編號 bs-9517R
英文名稱 AMPD3 Rabbit pAb
中文名稱 紅細胞腺苷脫氨酶3抗體
別    名 Adenosine monophosphate deaminase(isoform E); Adenosine monophosphate deaminase 3; AMP aminohydrolase; AMP deaminase 3; AMP deaminase isoform E; Ampd3; AMPD3_HUMAN; Erythrocyte AMP deaminase; Erythrocyte specific AMP deaminase; Erythrocyte type AMP deamin  
研究領域 心血管  細胞生物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Chicken,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:50-200,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 89 kDa
檢測分子量
細胞定位 細胞漿 細胞外基質 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human AMPD3: 21-120/767 
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 AMP deaminase plays a critical role in energy metabolism.
Involvement in disease
Defects in AMPD3 are the cause of adenosine monophosphate deaminase deficiency erythrocyte type (AMPDDE); also known as erythrocyte AMP deaminase deficiency. AMPDDE is a metabolic disorder due to lack of activity of the erythrocyte isoform of AMP deaminase. It is a clinically asymptomatic condition characterized by a 50% increase in steady-state levels of ATP in affected cells. Individuals with complete deficiency of erythrocyte AMP deaminase are healthy and have no hematologic disorders.

Function:
AMP deaminase plays a critical role in energy metabolism.

Subunit:
Homotetramer.

Tissue Specificity:
Three isoforms are present in mammals: AMP deaminase 1 is the predominant form in skeletal muscle; AMP deaminase 2 predominates in smooth muscle, non-muscle tissue, embryonic muscle and undifferentiated myoblasts; AMP deaminase 3 is found in erythrocytes.

DISEASE:
Defects in AMPD3 are the cause of adenosine monophosphate deaminase deficiency erythrocyte type (AMPDDE) [MIM:612874]; also known as erythrocyte AMP deaminase deficiency. AMPDDE is a metabolic disorder due to lack of activity of the erythrocyte isoform of AMP deaminase. It is a clinically asymptomatic condition characterized by a 50% increase in steady-state levels of ATP in affected cells. Individuals with complete deficiency of erythrocyte AMP deaminase are healthy and have no hematologic disorders.

Similarity:
Belongs to the adenosine and AMP deaminases family.

SWISS:
Q01432

Gene ID:
272

Database links:

Entrez Gene: 272 Human

Entrez Gene: 11717 Mouse

Entrez Gene: 25095 Rat

NCBI: NP_001020560 Human

Omim: 102772 Human

SwissProt: Q01432 Human

SwissProt: O08739 Mouse

SwissProt: O09178 Rat

Unigene: 501890 Human

Unigene: 3238 Mouse

Unigene: 11106 Rat



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