Institute of Comparative Molecular Endocrinology, Ulm University, 89081 Ulm, Germany.
Institute of Analytical and Bioanalytical Chemistry, Ulm University, 89081 Ulm, Germany.
Int J Mol Sci. 2023 May 18;24(10):8948. doi: 10.3390/ijms24108948.
Mutations in the / gene cause Hereditary Hemochromatosis (HH), a highly prevalent genetic disorder characterized by elevated iron deposition in multiple tissues. HFE acts in hepatocytes to control hepcidin expression, whereas HFE actions in myeloid cells are required for cell-autonomous and systemic iron regulation in aged mice. To address the role of HFE specifically in liver-resident macrophages, we generated mice with a selective deficiency in Kupffer cells (). The analysis of the major iron parameters in this novel mouse model led us to the conclusion that HFE actions in Kupffer cells are largely dispensable for cellular, hepatic and systemic iron homeostasis.
基因突变导致遗传性血色素沉着症（HH），这是一种高度普遍的遗传疾病，其特征是多种组织中铁的沉积增加。HFE 在肝细胞中起作用，以控制铁调素的表达，而 HFE 在髓系细胞中的作用对于老年小鼠的细胞自主和系统铁调节是必需的。为了专门研究 HFE 在肝驻留巨噬细胞中的作用，我们生成了库普弗细胞（）选择性缺乏的小鼠（）。对这种新型 小鼠模型中的主要铁参数的分析使我们得出结论，即 HFE 在库普弗细胞中的作用对于细胞、肝脏和系统铁稳态在很大程度上是可有可无的。
