轉鐵蛋白受體2抗體規(guī)格 英文名稱  Anti-TFR2/Transferrin Receptor 2

中文名稱  轉鐵蛋白受體2抗體規(guī)格 

別    名 HFE 3; HFE3; HFE-3; MGC126368; TFR 2; TFR2; TFR-2; TFR2_HUMAN; TFRC 2; TFRC2; Transferrin receptor protein 2.

產品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應   Human, Mouse, Rat, Cow, Sheep

產品類型   一抗  

研究領域    細胞生物 信號轉導 通道蛋白

蛋白分子量  predicted molecular weight: 89kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human TFR2/Transferrin Receptor 2

亞    型  IgG

純化方法   affinity purified by Protein A

儲 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

轉鐵蛋白受體2抗體規(guī)格 產品應用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200

（石蠟切片需做抗原修復） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

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

產品介紹 Mediates cellular uptake of transferrin-bound iron in a non-iron dependent manner. May be involved in iron metabolism, hepatocyte function and erythrocyte differentiation.

Function : Mediates cellular uptake of transferrin-bound iron in a non-iron dependent manner. May be involved in iron metabolism, hepatocyte function and erythrocyte differentiation.

Subunit : Homodimer.

Subcellular Location : Cell membrane and Cytoplasm. Lacks the transmembrane domain. Probably intracellular.

Tissue Specificity : Predominantly expressed in liver. While the alpha form is also expressed in spleen, lung, muscle, prostate and peripheral blood mononuclear cells, the beta form is expressed in all tissues tested, albeit weakly.

DISEASE : Defects in TFR2 are a cause of hemochromatosis type 3 (HFE3) [MIM:604250]. HFE3 is a disorder of iron hemostasis resulting in iron overload and has a phenotype indistinguishable from that of hereditary hemochromatosis (HH). HH is characterized by abnormal intestinal iron absorption and progressive increase of total body iron, which results in midlife in clinical complications including cirrhosis, cardiopathy, diabetes, endocrine dysfunctions, arthropathy, and susceptibility to liver cancer. Since the disease complications can be effectively prevented by regular phlebotomies, early diagnosis is most important to provide a normal life expectancy to the affected subjects.

Similarity : Belongs to the peptidase M28 family. M28B subfamily.

Database links : UniProtKB/Swiss-Prot: Q9UP52.1

Involvement in disease;Defects in TFR2 are a cause of hemochromatosis type 3 (HFE3) . HFE3 is a disorder of iron hemostasis resulting in iron overload and has a phenotype indistinguishable from that of hereditary hemochromatosis (HH). HH is characterized by abnormal intestinal iron absorption and progressive increase of total body iron, which results in midlife in clinical complications including cirrhosis, cardiopathy, diabetes, endocrine dysfunctions, arthropathy, and susceptibility to liver cancer. Since the disease complications can be effectively prevented by regular phlebotomies, early diagnosis is most important to provide a normal life expectancy to the affected subjects.

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