Figure 5: Pearson’s patient shows mitochondria in peripheral blood erythrocytes. | Nature Communications

Figure 5: Pearson’s patient shows mitochondria in peripheral blood erythrocytes.

From: MtDNA mutagenesis impairs elimination of mitochondria during erythroid maturation leading to enhanced erythrocyte destruction

Figure 5

(a) FACS analysis of peripheral blood. TO: ribosomes; MitoTracker Red: mitochondria. Reticulocytes (dashed box); abnormal cell population with mitochondria (red box). (b) Ultrastructure of peripheral blood erythrocytes of a healthy individual (left), the Pearson’s patient in acute anaemic phase (middle) with morphologically abnormal erythrocytes (asterisk) carrying mitochondria (arrowheads), and Pearson patient after anaemia recovery with only mature erythrocytes similar to control (arrows). Scale bars, 2 μm; inset scale bar, 200 nm. (c) Pathomechanistic model for mitochondrial anaemia. (1) Primary mtDNA mutagenesis induces slow maturation of erythroblasts and delayed mitochondrial clearance from reticulocytes (2) with delayed TfR exclusion, extended iron loading, increase of total iron, increase of free iron, extensive oxidative stress with membrane phosphatidylserine (PS) exposure in the peripheral erythrocytes, which (3) activates splenic macrophages and leads to premature destruction of the erythrocytes.

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