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TFR2 Rabbit pAb (bs-9894R)  
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产品编号 bs-9894R
英文名称 TFR2 Rabbit pAb
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.  
Specific References  (1)     |     bs-9894R has been referenced in 1 publications.
[IF=3.7] Zhang, Ying, et al. "Calcium channel blockers ameliorate iron overload-associated hepatic fibrosis by altering iron transport and stellate cell apoptosis." Toxicology and Applied Pharmacology (2016).  WB ;  Mouse.  
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 (predicted: Mouse,Rat,Sheep,Cow)
产品应用 Flow-Cyt=1μg/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 89 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TFR2: 121-220/801 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
Q9UP52

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



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.
产品图片
Blank control (blue line): HL60(fixed with 70% ethanol Overnight at 4℃. Cells stained with Primary Antibody for 30 min at room temperature). Primary Antibody (green line): Rabbit Anti-TFR2 antibody (bs-9894R),Dilution: 1μg /10^6 cells; Isotype Control
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