CECAD Microsite

The role of mitochondrial dysfunction in neurodegeneration


Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of control mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Moreover well-known transcriptional cascades control mitochondrial biogenesis under different metabolic conditions. In contrast, little is known about post-transcriptional mechanisms of regulation of mitochondrial function. Recently, we have identified a novel RNA-binding protein, CLUH, which specifically bind mRNAs encoding mitochondrial proteins. We aim to understand the molecular mechanisms underlying CLUH function and the physiological and pathological conditions in which CLUH is implicated.

Selected publications:

Pla‐Martín, D., Schatton, D., Wiederstein, J. L., Marx, M. C., Khiati, S., Krüger, M., & Rugarli, E. I. (2020). CLUH granules coordinate translation of mitochondrial proteins with mTORC1 signaling and mitophagy. The EMBO Journal, e102731. https://doi.org/10.15252/embj.2019102731

Murru, S., Hess, S., Barth, E., Almajan, E.R., Schatton, D., Hermans, S., Brodesser, S., Langer, T., Kloppenburg, P., Rugarli, E.I. (2019). Astrocyte-specific deletion of the mitochondrial m-AAA protease reveals glial contribution to neurodegeneration. GLIA 67, 1526-1541.

Schatton D., Pla-Martin D., Marx MC., Hansen H., Mourier A., Nemazanyy I., Pessia A., Zentis P., Corona T., Kondylis V., Barth E., Schauss AC., Velagapudi V., Rugarli EI. (2017) CLUH regulates mitochondrial metabolism by controlling translation and decay of target mRNAs. J Cell Biol. 216: 675-693. doi: 10.1083/jcb.201607019.

Gao J., Schatton D., Martinelli P., Hansen H., Pla-Martin D., Barth E., Becker C., Altmueller J., Frommolt P., Sardiello M., Rugarli, E.I. (2014) CLUH regulates mitochondrial biogenesis by binding mRNAs of nuclear-encoded mitochondrial proteins. J Cell Biol, 207(2):213-23. doi: 10.1083/jcb.201403129.

List of all publications