Anti-AAMPD3抗体AMP deaminase plays a critical role in energy metabolism.
Involvement in disease

产品编号 yb-9517R
英文名称 Anti-AAMPD3抗体
中文名称 红细胞腺苷脱氨酶3抗体
别    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 deaminase; Myoadenylate deaminase.
Anti-AAMPD3抗体
说 明 书 0.1ml  0.2ml  
研究领域 心血管  细胞生物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （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.
分 子 量 89kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human AMPD3
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.

PubMed PubMed
产品介绍 background:
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.



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.