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Rabbit Anti-AMPD3/Cy7 Conjugated antibody (bs-9517R-Cy7)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-9517R-Cy7
英文名稱 Rabbit Anti-AMPD3/Cy7 Conjugated antibody
中文名稱 Cy7標(biāo)記的紅細胞腺苷脫氨酶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 deaminase; Myoadenylate deaminase.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  細胞生物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 89kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human AMPD3
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 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.
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