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货号： bs-9517R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9517R-AF350
- 英文名称
- Anti-AMPD3/AF350
- 中文名称
- AF350标记的红细胞腺苷脱氨酶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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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
- IF=1:50-200
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/ml
- 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.
- 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.
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
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.