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Title: Developing bone marrow derived cell therapies for cerebellar disorders
Author: Dey, Rimi
ISNI:       0000 0004 5917 3336
Awarding Body: University of Bristol
Current Institution: University of Bristol
Date of Award: 2015
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Friedreich's ataxia (FRDA) is a neurodegenerative disorder, which is progressive and currently incurable. The genetic defect causes a reduced expression of the mitochondrial protein, frataxin. Oxidative damage and mitochondrial dysfunction are key factors of this disorder. A potential strategy of treating FRDA is by the use of cell replacement therapies. Bone marrow derived stem cell therapies are being investigated as a means to prevent and halt underlying diseases. Human bone marrow derived mesenchymal stem cells (MSCs) possess promising anti-inflammatory properties. Demonstrated within, levels of superoxide dismutase enzymes are decreased in fibroblasts derived from patients with FRDA, leading to in creased sensitivity to oxidative damage. Furthermore, I have demonstrated that exposure to factors produced by MSCs increase resistance to oxidative stress, by restoring the expression of the superoxide dismutase enzymes and activation of intracellular cell signalling pat hways. In order to determine the processes occurring in FRDA cells with a neuronal phenotype, a cellular model using SH-SYSY cells was established. SH-SYSY cells are a cell line originating from a bone marrow biopsy of a human neuroblastoma patient. The findings from this study further imply that MSCs have the potential to be used as therapeutic agents for FRDA.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available