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Protecting Against Rod Degeneration In A Model Of Retinitis Pigmentosa

Title: Protecting Against Rod Degeneration In A Model Of Retinitis Pigmentosa.
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Name(s): Lebowitz, Joseph, author
Department of Biological Science
Type of Resource: text
Genre: Text
Issuance: serial
Date Issued: 2013
Physical Form: computer
online resource
Extent: 1 online resource
Language(s): English
Abstract/Description: Retinitis Pigmentosa (RP) is a heritable disease that affects the retina, the photosensitive neural tissue lining the back of the eye. RP results in the gradual degeneration of rod photoreceptor cells, but secondarily cone cells degenerate and all visual input is lost. Mutations in nearly 100 genes have been associated with this disease and related disorders, however little is known about the series of cellular events that precede photoreceptor death. Taking advantage of a transgenic zebrafish model of rod degeneration, a small molecule screen and morpholino injections were used to identify modifiers of the cell death typically observed in RP. Rod photoreceptors in the developing zebrafish larvae express a fluorescent reporter gene. This leads to rod degeneration similar to that seen in RP, but cell death can easily be observed under a microscope. Live larvae were observed under the fluorescence microscope to determine if a compound has slowed or altered the degeneration of the rod cells at 4 days post fertilization. A gene-dosage dependent rate of degeneration was uncovered when comparing larvae that were hetero- and homozygous for the transgene. Modulation of the retinoic acid pathway modestly altered cell death. Knocking down Nr2e3 and p53 expression also showed protective effects that were stronger than those following chemical treatment. p53 codes for a key regulator of apoptosis, and Nr2e3 codes for a transcription factor of the rhodopsin gene. These results suggest further study of genetic and chemical manipulation of photoreceptor degeneration, and genotype-phenotype correlations in such diseases.
Identifier: FSU_migr_uhm-0269 (IID)
Keywords: Retinitis Pigmentosa, Photoreceptor, Degeneration, Zebrafish
Submitted Note: A Thesis submitted to the Department of Biological Sciences in partial fulfillment of the requirements for graduation with Honors in the Major.
Degree Awarded: Fall Semester, 2013.
Date of Defense: October 25, 2013.
Subject(s): Biology
Persistent Link to This Record: http://purl.flvc.org/fsu/fd/FSU_migr_uhm-0269
Owner Institution: FSU
Is Part of Series: Honors Theses.

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Lebowitz, J. (2013). Protecting Against Rod Degeneration In A Model Of Retinitis Pigmentosa. Retrieved from http://purl.flvc.org/fsu/fd/FSU_migr_uhm-0269