Prof. Dr. Elena Rugarli

Research Area: Regulation of mitochondrial Function / mitochondrial Diseases / Neurodegeneration 


1. Research Background:

Mitochondria are essential organelles involved in ATP production, Fe-S cluster biogenesis, calcium buffering, and several metabolic biosynthetic pathways. Mitochondrial homeostasis depends on regulated biogenesis and efficient quality control mechanisms, and is key to cell survival. Dysfunctional mitochondria can trigger apoptotic cell death, and play an important role in the pathogenesis of several ageing-associated diseases, including hereditary spastic paraplegia. We are especially interested in post-transcriptional mechanisms of regulation of mitochondrial biogenesis, as well as quality control pathways that are crucial to maintain the organelle function.

2. Research questions addressed by the group:

  • What are the mechanisms that regulate mitochondrial function in long axons and which role do they have in human diseases?
  • Is mitochondrial metabolism controlled at post-transcriptional level and what is the physiological significance of such mechanisms?

3. Possible projects:

If you are interested in how mitochondrial function is regulated during disease and ageing, come and visit our lab. I will be happy to discuss possible projects with you.

4. Applied Methods and model organisms:

We use mammalian cells and mouse models to answer our scientific questions. Several methods are integrated including unbiased omics approaches, advanced microscopy, a variety of biochemical methods, and immunohistochemistry. 

5. Desirable skills and qualifications:

Solid background knowledge acquired during master studies (biology, biochemistry, biotechnology), motivation and enthusiasm. 

6. References:

  1. Murru S, Hess S, Barth E, Almajan ER, Schatton D, Hermans S, Brodesser S, Langer T, Kloppenburg P, and Rugarli EI. (2019) Astrocyte-specific deletion of the mitochondrial m-AAA protease reveals glial contribution to neurodegeneration. Glia 67:1526-1541. doi: 10.1002/glia.23626. 
  2. 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, and 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. 
  3. Wang S, Jacquemyn J, Murru S, Martinelli P, Barth E, Langer T, Niessen CM, and Rugarli EI. (2016) The mitochondrial m-AAA protease prevents demyelination and hair greying. PLoS Genet. 12: e1006463. doi:10.1371/journal.pgen.1006463. 
  4. Papadopoulos, C., Orso, G., Mancuso, G., Herholz, M., Gumeni, S., Tadepalle, N., Jüngst, C., Tzschichholz, A., Schauss, A., Höning, S., Trifunovic, A., Daga, A., and Rugarli, E.I.(2015). Spastin binds to lipid droplets and affects lipid metabolism. PLoS Genet.11, e1005149. 
  5. Gao, J., Schatton, D., Martinelli, P., Hansen, H., Pla-Martin, D., Barth, E., Becker, C., Altmueller, J., Frommolt, P., Sardiello, M., and Rugarli, E.I.(2014). CLUH regulates mitochondrial biogenesis by binding mRNAs of nuclear-encoded mitochondrial proteins. J. Cell Biol. 207, 213-223. 
  6. Kondadi A.K., Wang, S., Montagner, S., Kladt, N., Korwitz, A., Martinelli, P., Herholz, D., Baker, M.J., Schauss, A.C., Langer, T., and Rugarli, E.I.(2014). Loss of the m-AAA protease subunit AFG3L2 causes mitochondrial transport defects and tau hyperphosphorylation. EMBO J. 33, 1011-1026.