Dysfunction in mitochondrial protein import and mitochondrial disease

Research Fellowships

$200,000 AUD

01/01/2019 →

Carolyn Sue


Dr Diana Stojanovski

Research Institution

The University of Melbourne

Project Name

Dysfunction in mitochondrial protein import and mitochondrial disease

Project Details

Future development of treatments will, it is hoped, stem from this important research. The project aims to improve our understanding of mitochondrial biology, mitochondrial function and mitochondrial physiology in disease. This knowledge will have far reaching implications for many areas of mitochondrial research.

Mitochondrial diseases are clinically and genetically varied, with over 250 disease genes identified so far. Although many aspects of human mitochondrial biology are well understood, others such as mitochondrial protein import remain less defined. Dr Diana Stojanovski will lead a team of
experts to investigate how mitochondrial protein import dysfunction leads to mitochondrial disease (mito).

Expanding the understanding of the biology of mito will facilitate the creation of new models to uncover pathomechanisms overseeing mitochondrial disorders linked with protein dysfunction.

The mitochondrion is the cell’s power plant, where sugars from the food we eat are converted into energy that fuels our body. Inside the power plant is a workforce of ‘proteins’ that perform many critical functions of mitochondria. Mitochondrial protein import is the process through which mitochondrial proteins are delivered to and assembled correctly within mitochondria. If this process fails, energy production is affected, which has drastic implication on mitochondrial health and function.

While investigating protein dysfunction, Dr Stojanovski hopes to identify new therapeutic targets for mito.

Through the training of students and personnel involved in the research, this project will expand research efforts in mitochondrial medicine.