Jonathan Cooper

Jonathan Cooper


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Personal profile

Research interests

Research has focused upon the neurobiology of Batten Disease or neuronal ceroid lipofuscinosis (NCLs). Batten disease is the collective name for a group of neurodegenerative childhood disorders that invariably prove fatal, with no effective treatment available. Together with colleagues in the US, Europe and New Zealand, we are working to discover precisely how Batten disease affects the brain. The main thrust of this work is to study patterns of neurodegeneration in mouse and large animal models of NCL, comparing our findings with the brains of affected individuals. In this manner we can identify and follow the earliest effects of disease and discover how and why these changes subsequently develop. These studies not only provide us with important landmarks of disease progression, but also the ability to judge the efficacy of candidate therapeutic strategies. Particular interests include: the comparative study of NCL pathogenesis; the role of neuroimmune and autoimmune responses in the NCLs and other storage disorders; the developmental neurobiology of the NCLs; assessment of therapeutic efficacy. Other collaborative studies with colleagues in the Institute's Neurodegeneration IRG continue to explore the role of novel proteins in neurodegenerative mechanisms.

Research interests (short)

Neurobiology of paediatric lysosomal storage disorders.

Expertise related to UN Sustainable Development Goals

In 2015, UN member states agreed to 17 global Sustainable Development Goals (SDGs) to end poverty, protect the planet and ensure prosperity for all. This person’s work contributes towards the following SDG(s):

  • SDG 3 - Good Health and Well-being

Education/Academic qualification

Doctor of Philosophy, Anatomical investigations of visual and visuomotor-related nuclei within the meso-diencephalon in the rat, University of Bristol

Award Date: 1 Jan 1990

Bachelor of Science, University of Sheffield

Award Date: 1 Jan 1986


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Collaborations and top research areas from the last five years

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