TFR2 Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss

bsm-61886R

重组兔单抗、mIHC精品抗体

TFR2 Recombinant Rabbit mAb

转铁蛋白受体2重组兔单抗

HFE3; TFRC2; Trfr2; TFR2_HUMAN; TFR2; TFR2_MOUSE; TFR2_RAT; transferrin receptor 2

Rabbit

A synthesized peptide derived from human Transferrin receptor 2: 150-300/801

IgG

Liquid

affinity purified by Protein A

Recombinant

5B10

89 kDa

100 kDa

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

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

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.

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

TFR2

Transferrin receptor protein 2

Homodimer.

Cell membrane and Cytoplasm. Lacks the transmembrane domain. Probably intracellular.

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.

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.

Belongs to the peptidase M28 family. M28B subfamily.

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