Erythropoietic regulators of iron metabolism

Erythropoiesis and iron metabolism in dominant erythropoietic protoporphyria.

Erythropoietic protoporphyria (EPP) results from deficiency of ferrochelatase (FECH). Accumulation of protoporphyrin IX causes life-long acute photosensitivity. Microcytic anemia occurs in 20% to 60% of patients. We investigated 178 patients with dominant EPP confirmed by molecular analysis. Erythropoiesis was impaired in all patients; all had a downward shift in hemoglobin (Hb), and the mean d...

Mitofusins: Mighty Regulators of Metabolism

Mitochondria are central regulators of cellular metabolism but how their function in a subset of cells affects whole-body energy balance is less understood. Two studies in this issue of Cell identify how diet-dependent modulation of mitochondrial fusion in specific neuronal circuits impact the metabolic status of an animal.

Regulators of iron balance in humans.

Macrophages: central regulators of iron balance.

Macrophages are important to immune function and also actively participate in iron homeostasis. The involvement of splenic and liver macrophages in the processing of effete erythrocytes and the subsequent return of iron to the circulation is well established, and the molecular details of iron recycling have been characterized recently. Another important aspect regarding iron handling by macroph...

Iron in erythropoietic protoporphyrias: Dr. Jekyll or Mr. Hyde?

Erythropoietic protoporphyria consists of two different genetic diseases, erythropoietic protoporphyria (EPP) and X-linked erythropoietic protoporphyria (XLEPP). Both of them are often accompanied by iron deficiency. Iron supplementation appears to be beneficial in XLEPP, although the clinical experience until to date is limited. In EPP, iron supplementation is discussed ambiguously and may cau...