University Research Groups
Below is an outline of the research undertaken by the research groups within the MRC Prion Unit. Click on a link below for more details about a specific topic.
- Cellular mechanisms of neurodegeneration - Prof. Sarah Tabrizi
- Molecular Cell biology - Prof. Parmjit Jat
- Mammalian Neurogenetics - Prof. Elizabeth Fisher
- Prion immunology and Immunotherapy - Dr. Azadeh Khalili-Shirazi
- Experimental neuropathology - Prof. Sebastian Brandner
- Small molecule therapeutics - Dr. Mark Farrow
- Molecular basis of frontotemporal dementia and related disorders - Dr Adrian Isaacs
Cellular mechanisms of neurodegeneration - Prof. Sarah Tabrizi
Dr Sarah Tabrizi is Professor of Clinical Neurology based in the Dept of Neurodegenerative Disease at the Institute of Neurology, University College London and also an Honorary Consultant Neurologist/Neurogeneticist at the National Hospital for Neurology and Neurosurgery at Queen Square in London. Her group focuses on studying cellular mechanisms of neurodegeneration in a variety of different systems from in vitro modelling to direct study of the human disease. Her main areas of research are in protein misfolding diseases, with focus on Huntington’s disease and prion biology. More detail.
Molecular Cell biology - Prof. Parmjit Jat
Cell lines have proven to be invaluable for in vitro studies of many complex processes and diseases including studying aspects of prion biology. The use of in vitro cell culture models to study prion propagation has been limited to a few cell-lines that are susceptible either to mouse-adapted or sheep scrapie prion strains, with none yet described able to stably propagate human prions. Long term studies at the MRC Prion Unit derived the cell line, PK1, a subline of neuroblastoma N2a cells, which efficiently propagate RML prions and which has allowed cell culture based bioassay for this strain of mouse prions. However, this cell line is not permissive to many other prion strains. Read more.
Mammalian Neurogenetics - Prof. Elizabeth Fisher
The Mammalian Neurogenetics Group, led by Professor Elizabeth Fisher, is studying the genetic causes of neuronal dysfunction and degeneration, in two main areas, motor neuron degeneration and Down syndrome. Read more.
Prion immunology and Immunotherapy - Dr. Azadeh Khalili-Shirazi
Prion diseases like variant Creutzfeldt-Jakob disease (vCJD) in humans, BSE in cattle and scrapie in sheep are fatal brain diseases caused by an infectious agent, known as a prion. Prions are thought to be composed of abnormal forms (generally referred to as PrPSc) of a protein which is a normal constituent of brain cells, known as the cellular prion protein (PrPC). These disease-associated forms of prion protein form clumps or plaques in the brain and this process causes damage to, and then loss of, brain cells. Read more.
Experimental neuropathology - Prof. Sebastian Brandner
Our research focuses on mechanisms of brain development and tumour formation. One of the essential questions in understanding the biology of these tumours is to identify from which cell they arise from and what are the cellular and molecular events that occur early in this transformation. To study the mechanisms of malignant transformation of neural cells, we are using in vitro and in vivo model systems. Read more.
Small molecule therapeutics - Dr. Mark Farrow
This is a major collaborative project with GlaxoSmithKline (GSK) directly funded by the Department of Health with the aim of developing an effective small molecule therapeutic for the treatment of prion disease in humans. The unique academic-industrial collaboration assembled for this project, bringing together one of the world’s leading academic centres of expertise in prion biology and neurodegeneration with one of the world’s leading pharmaceutical companies not only provides an unparalleled combination of expertise to tackle prion disease therapeutics, but also allows this to be performed at a fraction of normal commercial costs. Read more.
Molecular basis of frontotemporal dementia and motor neuron disease
My group studies the molecular basis of two related neurodegenerative diseases: frontotemporal dementia (FTD) and motor neuron disease (MND). FTD is a common form of young-onset dementia and is characterised by changes in personality and language. MND is characterised by progressive weakness and paralysis.. Read more.