mRNA-binding protein tristetraprolin is essential for cardiac response to iron deficiency by regulating mitochondrial function

T Sato, HC Chang, M Bayeva… - Proceedings of the …, 2018 - National Acad Sciences
T Sato, HC Chang, M Bayeva, JS Shapiro, L Ramos-Alonso, H Kouzu, X Jiang, T Liu, S Yar
Proceedings of the National Academy of Sciences, 2018National Acad Sciences
Cells respond to iron deficiency by activating iron-regulatory proteins to increase cellular
iron uptake and availability. However, it is not clear how cells adapt to conditions when
cellular iron uptake does not fully match iron demand. Here, we show that the mRNA-
binding protein tristetraprolin (TTP) is induced by iron deficiency and degrades mRNAs of
mitochondrial Fe/S-cluster-containing proteins, specifically Ndufs1 in complex I and Uqcrfs1
in complex III, to match the decrease in Fe/S-cluster availability. In the absence of TTP …
Cells respond to iron deficiency by activating iron-regulatory proteins to increase cellular iron uptake and availability. However, it is not clear how cells adapt to conditions when cellular iron uptake does not fully match iron demand. Here, we show that the mRNA-binding protein tristetraprolin (TTP) is induced by iron deficiency and degrades mRNAs of mitochondrial Fe/S-cluster-containing proteins, specifically Ndufs1 in complex I and Uqcrfs1 in complex III, to match the decrease in Fe/S-cluster availability. In the absence of TTP, Uqcrfs1 levels are not decreased in iron deficiency, resulting in nonfunctional complex III, electron leakage, and oxidative damage. Mice with deletion of Ttp display cardiac dysfunction with iron deficiency, demonstrating that TTP is necessary for maintaining cardiac function in the setting of low cellular iron. Altogether, our results describe a pathway that is activated in iron deficiency to regulate mitochondrial function to match the availability of Fe/S clusters.
National Acad Sciences