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An electrophysiological assessment of the use of stem cells in neurological disease modelling and therapy

Steele, Oliver G. 2019. An electrophysiological assessment of the use of stem cells in neurological disease modelling and therapy. MPhil Thesis, Cardiff University.
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Electrophysiology is the best discipline to functionally characterise neuronal behaviour, as such, electrophysiology plays a key role in neurological disease modelling and therapy. Totipotent stem cells have the limitless possibility to differentiate into any type of cell within the human body and therefore used frequently in disease modelling and therapy. This thesis aims to electrophysiologically assess the use of stem cells in disease modelling and therapy across two diseases; the infantile epilepsy disorder - cyclin dependant kinase like 5 (CDKL5) deficiency, and the fatal neurodegenerative condition - Huntington’s Disease, respectively. This thesis shows that a patient induced pluripotent stem cell derived cortical neuronal model of CDKL5-deficiency recreates the epilepsy seen in patients, providing a valid disease model. Furthermore, these neurons can be cultured on a high-throughput multi-electrode array for the rapid testing of novel therapeutic compounds. In the disease therapy side of the project, stem cell grafts fail to functionally integrate synaptically into a rat model of Huntington’s Disease (HD), however restore synaptic function in the lesioned striatum. These data suggest that the stem cell graft may provide therapeutic benefits, just not electrophysiologically. Taken together, these observations suggest that stem cells can reproduce the defining clincal charactersistics, epilepsy, lacking from other model systems in an infantile epilepsy model and induce acute therapeutic benefits in a rodent model of HD.

Item Type: Thesis (MPhil)
Date Type: Completion
Status: Unpublished
Schools: Biosciences
Uncontrolled Keywords: Electrophysiology; Huntington's Disease; CDKL5 Deficiency; Stem Cells
Date of First Compliant Deposit: 19 May 2019
Last Modified: 19 May 2020 02:05

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