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The mechanisms of photoreceptor cell death (360G-Wellcome-205041_Z_16_Z)

<p>Problems of protein homeostasis (proteostasis) that lead to protein misfolding, improper traffic and aggregation are associated with many forms of neurodegeneration. The neurodegeneration retinitis pigmentosa (RP) offers an excellent paradigm to study why proteostasis is critical for neuronal function and survival. Rhodopsin mutations cause dominant RP and disturb proteostasis, yet the underlying disease mechanisms and effective therapies remain elusive. I will exploit recent advances in gene editing technology and stem cell biology to produce new animal and patient derived models of the most common rhodopsin mutations in the UK. I will use these to address my key goals, i) to define any common disease mechanisms between the different classes of mutation, and ii) identify new therapeutic approaches for rhodopsin RP. I will use a combination of genetic and chemical manipulation of the major cell stress and degradation pathways, and identify the partner proteins of rhodopsin mutants using unbiased proteomic analyses. These complementary studies will be based on a series of interlinked hypotheses to determine how rhodopsin mutations disturb protein homeostasis and if this can be restored. The findings will have broader implications not only for other forms of retinal degeneration, but also neurodegenerative disease where proteostasis is disturbed.&nbsp;</p>

£1,529,466

30 Nov 2016

Grant details
Amount Awarded 1529466
Applicant Surname Cheetham
Approval Committee Science Interview Panel
Award Date 2016-11-30T00:00:00+00:00
Financial Year 2016/17
Grant Programme: Title Investigator Award in Science
Internal ID 205041/Z/16/Z
Lead Applicant Prof Michael Cheetham
Planned Dates: End Date 2022-07-01T00:00:00+00:00
Planned Dates: Start Date 2017-07-01T00:00:00+00:00
Recipient Org: Country United Kingdom
Region Greater London
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