TFR2,转铁蛋白受体2抗体

产品编号HZ-9894R
英文名称TFR2
中文名称转铁蛋白受体2抗体
别 名HFE 3; HFE3; HFE-3; MGC126368; TFR 2; TFR2; TFR-2; TFR2_HUMAN; TFRC 2; TFRC2; Transferrin receptor protein 2; Transferrin Receptor 2.
说 明 书0.1ml 0.2ml
研究领域细胞生物 信号转导 通道蛋白
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Cow, Sheep,
TFR2,转铁蛋白受体2抗体产品应用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
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human TFR2/Transferrin Receptor 2
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.
TFR2,转铁蛋白受体2抗体PubMedPubMed
产品介绍background:
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.

TFR2,转铁蛋白受体2抗体Similarity:
Belongs to the peptidase M28 family. M28B subfamily.

Gene ID:
7036

Database links:
Entrez Gene: 7036 Human
Entrez Gene: 50765 Mouse
Entrez Gene: 288562 Rat
Omim: 604720 Human
SwissProt: Q9UP52 Human
SwissProt: Q9JKX3 Mouse
SwissProt: B2GUY2 Rat
Unigene: 544932 Human
Unigene: 21757 Mouse
Unigene: 59926 Rat

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

TFR2,转铁蛋白受体2抗体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.