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Prof Helene Plun-Favreau
Professor of Cellular Neurodegeneration
Neurodegenerative Diseases
UCL Queen Square Institute of Neurology
- Joined UCL
- 5th Mar 2007
Research summary
In recent years, it has become clear that even in clinically distinct neurodegenerative conditions, there are common underlying themes in how the neurons become sick and die. One such theme is a breakdown in the maintenance of mitochondria, which plays a central role in Parkinson’s disease and in other neurodegenerative conditions.
Mitochondria are the ‘energy powerhouses’ of cells. Their function is vital in long-lived neurons, where mitochondria must be maintained for an entire lifetime, and where a great deal of energy is required for them to function and survive.
The selective autophagy of damaged mitochondria (mitophagy) is critical for cell survival as it maintains optimal cellular energy production whilst avoiding the toxic accumulation of damaged mitochondria. Important information about the control of mitophagy has come from the study of the genes associated with autosomal recessive Parkinson’s disease. Of particular interest, PINK1 (mitochondrial kinase) and Parkin (E3-ubiquitin ligase) have been found to play crucial roles in mitophagy.
Against this background, our lab focuses on the following themes:
- Unravelling the upstream pathways that regulate the mitophagy process.
- Assessing mitophagy and other mitochondrial dysfunctions in iPSC-derived neurons from patients with Parkinson’s disease, and with other neurodegenerative diseases (E.g Alzheimer’s disease, Frontotemporal Dementia, Amyotrophic Lateral Sclerosis, mitochondrial DNA disease etc).
Our ultimate goal is to identify compounds that are able to modulate mitophagy and rescue mitochondrial pathophysiology and neuronal death.
Teaching summary
Prof Helene Plun-Favreau is teaching "mitochondrial dysfunction associated with neurodegeneration" in the following MScs:
- UCL Cinical Neuroscience MSs
- UCL Neuroscience MSc
- UCL Neurogenetics MSc
-UCL CELL0015, 'Mitochondrial Biology and metabolism in health and disease'
-UCL MRes Brain Sciences
-UCL NEUR0010-0011-0012 Neurobiology of Brain Injury and Disease
Education
- Universite d'Angers
- Doctorate, Doctor of Philosophy | 2002
- Universite de Poitiers
- Other higher degree, Master of Science | 1999
- Universite d'Angers
- First Degree, Bachelor of Science | 1998
Biography
Prof Helene Plun-Favreau is a cell biologist by training.
After a PhD in France (Angers University) in signal transduction, Helene did her postdoctoral work with Professor Julian Downward at CRUK. The discoveries and work she did in this area led her towards neurodegeneration and she was successful in being appointed to an MRC Career Development Fellowship to work in the Department of Molecular Neuroscience at UCL Institute of Neurology. Since her arrival in 2007 Helene has carried out some significant work on the molecular pathways associated with mitophagy and other mitochondrial dysfunctions in neurodegenerative disorders. The approaches they have undertaken require live cell microscopy and complex molecular and cellular biology, and provide a more complete picture of the pathways that play a role in the pathogenesis of neurodegeneration.