共 30 条
First phenotypic description of transferrin receptor 2 knockout mouse, and the role of hepcidin
被引:119
作者:

Wallace, DF
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机构:
Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia

Summerville, L
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Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia

Lusby, PE
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Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia

Subramaniam, VN
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Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia
机构:
[1] Queensland Inst Med Res, Canc & Cell Biol Div, Membrane Transport Lab, Brisbane, Qld 4006, Australia
来源:
关键词:
D O I:
10.1136/gut.2004.062018
中图分类号:
R57 [消化系及腹部疾病];
学科分类号:
摘要:
Background: Transferrin receptor 2 (TfR2) is a key molecule involved in the regulation of iron homeostasis. Mutations in humans cause type 3 haemochromatosis and a targeted mutation in mice leads to iron overload with a similar phenotype. We have previously described the generation of a complete TfR2-knockout (KO) mouse. Aims: The aims of this study were to determine the phenotype and analyse expression of iron related molecules in the liver, duodenum, and spleen of homozygous TfR2-KO, heterozygous, and wild-type mice. Methods: Serum and tissue iron levels were determined in 10 week old male mice. Expression of iron related mRNA transcripts were analysed in the liver, duodenum, and spleen using real time polymerase chain reaction. Expression of iron related proteins in the liver were analysed by immunoblotting and immunohistochemistry. Results: Homozygous TfR2-KO mice had no TfR2 protein expression and developed significant iron overload typical of TfR2 associated haemochromatosis. In the liver of TfR2-KO mice there was no upregulation of hepcidin mRNA or prohepcidin protein in response to iron loading. Conclusions: Our results suggest that TfR2 is required for iron regulated expression of hepcidin and is involved in a pathway related to Hfe and hemojuvelin.
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页码:980 / 986
页数:7
相关论文
共 30 条
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机构: Royal Brisbane Hosp, Queensland Inst Med Res, Lab Iron Metab, Iron Metab Grp, Herston, Qld 4029, Australia

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机构: Royal Brisbane Hosp, Queensland Inst Med Res, Lab Iron Metab, Iron Metab Grp, Herston, Qld 4029, Australia

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Royal Brisbane Hosp, Queensland Inst Med Res, Lab Iron Metab, Iron Metab Grp, Herston, Qld 4029, Australia Royal Brisbane Hosp, Queensland Inst Med Res, Lab Iron Metab, Iron Metab Grp, Herston, Qld 4029, Australia

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机构: St Louis Univ, Sch Med, Edward A Doisy Dept Biochem & Mol Biol, St Louis, MO 63104 USA

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机构: St Louis Univ, Sch Med, Edward A Doisy Dept Biochem & Mol Biol, St Louis, MO 63104 USA

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机构: St Louis Univ, Sch Med, Edward A Doisy Dept Biochem & Mol Biol, St Louis, MO 63104 USA

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Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA

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Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA

Gombart, AF
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h-index: 0
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Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA

Koeffler, HP
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h-index: 0
机构:
Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA Univ Calif Los Angeles, Cedars Sinai Med Ctr, Sch Med, Dept Med,Div Hematol Oncol,Burns & Allen Res Inst, Los Angeles, CA 90048 USA