2023年1月26日 · In this issue of Blood, Xiao et al 1 demonstrate that the hemochromatosis protein HFE is required for the participation of hepatocellular transferrin receptor 1 (TFR1) in the co-regulation of iron metabolism and erythropoiesis.

Transferrin receptor (TfR) is a dimeric cell surface protein that binds both the serum iron transport protein transferrin (Fe-Tf) and HFE, the protein mutated in patients with the iron overload disorder hereditary hemochromatosis.

2023年1月26日 · Hepatocyte TfR1 has been proposed to influence hepcidin expression through its interaction with homeostatic iron regulator, HFE, the most commonly mutated gene in the iron overload disorder, hereditary hemochromatosis. 7 HFE functions as a positive regulator of hepcidin expression by enhancing the activation of the BMP-SMAD signaling cascade, 8 ...

Hemochromatosis is caused by mutations in HFE, a protein that competes with transferrin (TF) for binding to transferrin receptor 1 (TFR1). We developed mutant mouse strains to gain insight into the role of the Hfe/Tfr1 complex in regulating iron homeostasis.

循环中携带铁原子的转铁蛋白与靶细胞表面特异的转铁蛋白受体（tfr）结合，通过网格蛋白介导的内吞作用进入靶细胞。 质子流入所引起的内体酸化导致Tf-TFR1复合物发生构象转化而解离，同时释放出Fe 3+ ，后者在细胞内铁还原酶的作用下转化为Fe 2+ ，经DMT-1穿过 ...

2004年6月11日 · Transferrin receptor (TfR) is a dimeric cell surface protein that binds both the serum iron transport protein transferrin (Fe-Tf) and HFE, the protein mutated in patients with the iron overload disorder hereditary hemochromatosis. HFE and Fe-Tf can bind simultaneously to TfR to form a ternary comple …

2000年1月6日 · HFE binds to the transferrin receptor (TfR), a receptor by which cells acquire iron-loaded transferrin. The 2.8 Å crystal structure of a complex between the extracellular portions of HFE and...

1999年5月11日 · Recent demonstration of an association between transferrin receptor (TfR) and HFE, a major histocompatibility complex class I-like molecule that has been implicated to play a role in hereditary hemochromatosis, further strengthens the notion that HFE is …

2004年2月20日 · The HFE binding site on TfR is on the helical domain, and mutational analysis of TfR shows that it overlaps with the binding site for Tf (West et al., 2001). In this study, we have used cryo-electron microscopy (cryo-EM) and single particle averaging techniques to determine a density map for the human TfR-diferric Tf (dTf) complex at ...

2001年10月19日 · To develop a more detailed microscopic model of the HFE-TfR-Tf system, we have further investigated the TfR/HFE stoichiometry in solution using equilibrium gel-filtration and analytical ultracentrifugation, and verified the overlapping binding site hypothesis by demonstrating that TfR mutations that affect HFE binding also affect Tf binding.