Stojanovski laboratory: Mitochondrial biology and disease

• Professor Diana Stojanovski

  Head of Laboratory

  d.stojanovski@unimelb.edu.au
  +61 3 9035 3197

Research Overview

The Stojanovski Lab focuses on understanding the fundamental processes governing mitochondrial function and their implications for human health and disease. Our research program encompasses the following themes.

1. Mitochondrial Protein Biogenesis: Investigating the mechanisms and machinery involved in the synthesis, import, and assembly of proteins within mitochondria.
2. Mitochondrial Dysfunction and Disease: Exploring how defects in fundamental mitochondrial protein pathways, like protein biogenesis cause mitochondrial disease.
3. Host-Pathogen Interactions: Examining how effector proteins from intracellular bacterial pathogens target mitochondria to manipulate organelle function.

Figure 1: Mitochondria in a HeLa cell (shown in yellow) radiate out from the nucleus (shown in blue) and form a reticular network.

Staff

• 
  Professor Diana Stojanovski

  Head of Laboratory

• 
  Dr Catherine Palmer

  Research Fellow

• 
  Dr Yilin Kang

  Research Fellow

• 
  Phoebe Leeming

  Graduate Researcher

• 
  Julia Mercuri-Svik

  Honours Student

• 
  Evie Hodgson

  Research Assistant

Stojanovski Laboratory Group

Collaborators

Professor David Thorburn, Murdoch Children’s Research Institute
Dr Ann Frazier, Murdoch Children’s Research Institute Associate
Professor David Stroud, The University of Melbourne
Professor Hayley Newton, Department of Microbiology, Monash University
Professor Mike Ryan, Monash University

Funding

Research Publications

For complete list of publications visit Diana Stojanovski.

Recent senior author research publications:

Crameri, J.J., Palmer, C.S., Stait., T., Jackson, T.D., Lynch, M, Sinclair, A., Frajman, L.E., Compton, A.G., Coman, D., Thorburn, D.R., Frazier, A.E Stojanovski D. 2024. Reduced Protein Import via TIM23 SORT Drives Disease Pathology in TIMM50-Associated Mitochondrial Disease. Molecular and Cellular Biology. 44(6): 226-244.

Baker M., Blau K., Anderson A., Palmer CS., Fielden LF., Crameri JJ., Milenkovic D., Thorburn DR., Frazier AE., Langer T., Stojanovski D. 2024. CLPB disaggregase dysfunction impacts the functional integrity of the proteolytic SPY complex. Journal of Cell Biology. (2024) 223 (3):e202305087.

Anderson AJ., Crameri JJ., Ang CS., Malcolm TR., Kang Y., Baker MJ., Palmer CS., Sharpe AJ., Formosa LE., Ganio K., Baker MJ., McDevitt CA., Ryan MT., Maher MJ., Stojanovski D. 2023. Human Tim8a, Tim8b and Tim13 are auxiliary assembly factors of mature Complex IV. EMBO Reports.24(8):e56430.

Loterio, RK., Thomas DR., Andrade WL., Yi W., Santos LL., Mascarenhas DPA., Steiner, TM., Chiaratto J., Fielden LF., Lopes L, Bird LE., Goldman GH., Stojanovski D, Scott NE., Zamboni DS. Newton HJ. 2023. Coxiella co-opts the Glutathione Peroxidase 4 to protect the host cell from oxidative stress–induced cell death. Proceedings of the National Academy of Sciences. 120, 36, e2308752120.

Jackson, T.D., Crameri, J.J., Muellner-Wong, L., Frazier, A.E., Palmer, C.S., Formosa, L.E., Hock, D.H., Fujihara, K.M., Stait, T., Sharpe, A.J., Thorburn, D.R., Ryan, M.T., Stroud, D.A., Stojanovski, D. 2022. Sideroflexin 4 is a complex I assembly factor that interacts with the MCIA complex and is required for the assembly of the ND2 module. Proceedings of the National Academy of Sciences. 119, e2115566119. 10.1073/pnas.2115566119.

Jackson, T. D., Hock, D. H., Fujihara, K. M., Palmer, C. S., Frazier, A. E., Low, Y. C., Kang, Y., Ang, C. S., Clemons, N. J., Thorburn, D. R., Stroud, D. A. & Stojanovski, D. 2021. The TIM22 complex mediates the import of Sideroflexins and is required for efficient mitochondrial one-carbon metabolism. Molecular Biology of the Cell. mbcE20060390.

Palmer, C.S., Lou, J., Kouskousis, B., Pandzic, E., Anderson, A.J., Kang, Y., Hinde E., & Stojanovski, D. (2021). Super-resolution microscopy reveals the arrangement of inner membrane protein complexes in mammalian mitochondria. Journal of Cell Science. 134, no. 13.

Fielden, L. F., Scott, N. E., Palmer, C. S., Khoo, C. A., Newton, H. J. & Stojanovski, D. (2020). Proteomic identification of Coxiella burnetii effector proteins targeted to the host cell mitochondria during infection. Mol Cell Proteomics. doi:10.1074/mcp.RA120.002370.

Kang, Y., Anderson, A. J., Jackson, T. D., Palmer, C. S., De Souza, D. P., Fujihara, K. M., Stait, T., Frazier, A. E., Clemons, N. J., Tull, D., Thorburn, D. R., McConville, M. J., Ryan, M. T., Stroud, D. A. & Stojanovski, D. (2019). Function of hTim8a in complex IV assembly in neuronal cells provides insight into pathomechanism underlying Mohr-Tranebjaerg syndrome. Elife, vol. 8, doi:10.7554/eLife.48828.

Kang, Y., Stroud, D. A., Baker, M. J., De Souza, D. P., Frazier, A. E., Liem, M., Tull, D., Mathivanan, S., McConville, M. J., Thorburn, D. R., Ryan, M. T. & Stojanovski, D. (2017) Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex. Molecular Cell. vol. 67, no. 3, pp. 457-470 e5.

Kang, Y., Baker, M. J., Liem, M., Louber, J., McKenzie, M., Atukorala, I., Ang, C. S., Keerthikumar, S., Mathivanan, S. & Stojanovski, D. (2016). Tim29 is a novel subunit of the human TIM22 translocase and is involved in complex assembly and stability. Elife, vol. 5, doi:10.7554/eLife.17463.

Fielden, L. F., Moffatt, J. H., Kang, Y., Baker, M. J., Khoo, C. A., Roy, C. R., Stojanovski, D. & Newton, H. J. (2017) A Farnesylated Coxiella burnetii Effector Forms a Multimeric Complex at the Mitochondrial Outer Membrane during Infection. Infect Immun, vol. 85, no. 5, doi:10.1128/IAI.01046-16

Book Chapters

• Stojanovski D, Bohnert M, Pfanner N, van der Laan M.  Mechanisms of Protein Sorting in Mitochondria. Cold Spring Harbor Perspectives in Biology 2012; 4 (10).
• Stojanovski D, Pfanner N, Wiedemann N.  Protein import into mitochondria. Meth Cell Biol 2007; 80: 783-806.