ZFIN Image: Rondelli et al., 2021, Figure 5

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Figure 5

CLPX regulates mitochondrial iron utilization in erythroid cells.A, iron supplementation restored hemoglobinization in Clpx^−/− cells. B, Fe-hinokitiol significantly reduced the numbers of anemic fish in clpxb incrosses. As a control, we treated frs incrosses with Fe-hinokitiol. Fe-hinokitiol significantly decreased the number of anemic fish and severity of anemia. C, representative images of benzidine stained clpxb zebrafish treated with Fe-hinokitiol. Erythroid hemoglobinization was significantly increased. D, revised model of how CLPX regulates erythroid heme synthesis. CLPX regulates ALAS2 turnover. In its absence, ALAS2 protein levels and activity are increased, increasing ALA levels. Concomitantly, PPOX and FECH activities, and iron incorporation into heme, are decreased, resulting in anemia and porphyria during CLPX deficiency.

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Acknowledgments

This image is the copyrighted work of the attributed author or publisher, and ZFIN has permission only to display this image to its users. Additional permissions should be obtained from the applicable author or publisher of the image. Full text @ J. Biol. Chem.