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Researchers identify a novel mechanism of fetal anemia linked to mitochondrial protein synthesis deficiency
- July 02, 2025
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A team of researchers has uncovered a previously unrecognized role of mitochondrial protein synthesis in the maintenance of intracellular iron distribution. Disruption of this process was found to cause lethal anemia in the fetal stage. This novel molecular mechanism will contribute to the understanding of the pathophysiology of iron-related diseases and the development of innovative therapeutic strategies.
A researcher team from International Research Center for Medical Sciences (IRCMS) at Kumamoto University, led by Dr. Tatsuya Morishima (Lecturer, Wakakusu researcher at IRCMS), and Prof. Hitoshi Takizawa, has identified a novel mechanism linking fetal anemia to disrupted intracellular iron distribution due to impaired mitochondrial protein synthesis. In this study, a mouse model with a knockout of the mitochondrial tRNA modification enzyme (Mto1) exhibited defective mitochondrial protein synthesis, revealing a previously unknown molecular mechanism. These findings could enhance our understanding of iron-related diseases and open the door to new therapeutic approaches. This research was published online on 21st of February, 2025 in Science Advances.
Study Overview
Most of the proteins are synthesized in the cytosol. However, a very small proportion is synthesized within the mitochondria, which are essential for energy production. Traditionally, the protein synthesis in mitochondria has been considered primarily responsible for ATP production. Mitochondrial tRNAs undergo diverse chemical modifications that are post-transcriptionally introduced by tRNA modification enzymes, and these chemical modifications play a crucial role for efficient protein synthesis. One key enzyme in this process is MTO1, which facilitates mitochondrial protein synthesis by modifying mitochondrial tRNAs. It is known as an important enzyme indispensable for survival, and mutations in MTO1 gene have been associated with severe anemia in a patient. However, it remains unknown whether impaired mitochondrial protein synthesis causes hematological disorders.
List of Referenes
- Tatsuya Morishima, Md. Fakruddin, Yohei Kanamori, Takeshi Masuda, Akiko Ogawa, Yuxin Wang, Vivien A. C. Schoonenberg, Falk Butter, Yuichiro Arima, Takaaki Akaike, Toshiro Moroishi, Kazuhito Tomizawa, Toshio Suda, Fan-Yan Wei, Hitoshi Takizawa. Mitochondrial translation regulates terminal erythroid differentiation by maintaining iron homeostasis. Science Advances, 2025; 11 (8) DOI: 10.1126/sciadv.adu3011
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