Researchers from the IRB Barcelona, the IBUB, VIMM, and the University of Padua reveal the vital function of Mitofusin 2 cellular structure in connecting organelles within cells. Among these organelles, the endoplasmic reticulum, which is in charge of synthesising proteins and lipids, and mitochondria, known as the “cell powerhouses,” interact in a variety of crucial ways.
ERMIT2 and ERMIN2 are two distinct “variants” of the Mitofusin 2 protein that have now been identified by the research team. These variations are produced by the process of alternative splicing, which reorganises gene segments called “exons” to produce various proteins from the same DNA sequence. Surprisingly, ERMIN2 and ERMIT2, which are derived from the mitochondrial protein Mitofusin 2, are found on the endoplasmic reticulum rather than on the mitochondria.
The team discovered in 2008 that the mitochondrial protein Mitofusin 2, which is reduced in metabolic diseases like diabetes and fatty liver and is mutated in the peripheral neuropathy Charcot-Marie-Tooth IIA, is crucial in promoting these interactions. However, the partner protein on the endoplasmic reticulum remained unknown.
María Isabel Hernández Alvarez from the IBUB is a co-author of the sudy led by Antonio Zorzano from the IRB Barcelona and the UB, Luca Scorrano from the VIMM and the University of Padua and Deborah Naón, member of both teams.
The work was conducted within the framework of projects funded by the ‘la Caixa’ Foundation, the Spanish Ministry of Science and Innovation, the Carlos III Health Institute, the European Research Council, the Muscular Dystrophy Association (MDA), the Italian Ministry of University and Research, and the Italian National Recovery and Resilience Plan (NRRP), Mission 4, Component 2, Investment 1.2, supported by the European Union – NextGenerationEU.
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Reference Article:
Naón, D., Hernández-Alvarez, M. I., Shinjo, S., Wieczór, M., Ivanova, S., De Brito, O. M., Quintana, A., Hidalgo, J., Palacín, M., Aparicio, P., Castellanos, J., Lores, L., Sebastián, D., Fernández-Veledo, S., Vendrell, J., Joven, J., Orozco, M., Zorzano, A., & Scorrano, L. (2023). “Splice variants of mitofusin 2 shape the endoplasmic reticulum and tether it to mitochondria“. Science, 380(6651). https://doi.org/10.1126/science.adh9351
