Department of Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, University of California, Los Angeles, California, USA.
Department of Medicine, David Geffen School of Medicine at UCLA, University of California, Los Angeles, California, USA.
J Cell Physiol. 2021 Jul;236(7):4888-4901. doi: 10.1002/jcp.30247. Epub 2020 Dec 28.
Erythroferrone (ERFE) is the main erythroid regulator of hepcidin, the homeostatic hormone controlling plasma iron levels and total body iron. When the release of erythropoietin from the kidney stimulates the production of new red blood cells, it also increases the synthesis of ERFE in bone marrow erythroblasts. Increased ERFE then suppresses hepcidin synthesis, thereby mobilizing cellular iron stores for use in heme and hemoglobin synthesis. Recent mechanistic studies have shown that ERFE suppresses hepcidin transcription by inhibiting bone morphogenetic protein signaling in hepatocytes. In ineffective erythropoiesis, pathological overproduction of ERFE by an expanded population of erythroblasts suppresses hepcidin and causes iron overload, even in non-transfused patients. ERFE may be a useful biomarker of ineffective erythropoiesis and an attractive target for treating its systemic effects.
红细胞生成素 (Erythroferrone, ERFE) 是调节铁调素 (hepcidin) 的主要红细胞因子，铁调素是一种维持体内铁平衡的激素，可调节血浆铁水平和全身铁含量。当肾脏分泌的促红细胞生成素刺激新的红细胞生成时，它也会增加骨髓红细胞中 ERFE 的合成。增加的 ERFE 随后抑制铁调素的合成，从而动员细胞内铁储存以用于血红素和血红蛋白的合成。最近的机制研究表明，ERFE 通过抑制肝细胞核因子中的骨形态发生蛋白信号来抑制铁调素的转录。在无效性红细胞生成中，大量扩增的红细胞产生病理性 ERFE 会抑制铁调素并导致铁过载，即使是非输血患者也是如此。ERFE 可能是无效性红细胞生成的有用生物标志物，也是治疗其全身作用的有吸引力的靶点。
