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Strategies to Slow the Onset of Blindness in Retinitis Pigmentosa

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Air date: Wednesday, September 29, 2010, 3:00:00 PM
Time displayed is Eastern Time, Washington DC Local
Views: Total views: 160 * This only includes stats from October 2011 and forward.
Category: WALS - Wednesday Afternoon Lectures
Runtime: 01:01:49
Description: The disease, retinitis pigmentosa (RP), is a blinding illness with genetic etiology. Many different RP genes are expressed in rod photoreceptors exclusively. These are the retinal photoreceptors used for dim light perception, or night vision. Individuals born with these mutations have poor, or no, night vision, but typically have normal daylight and color vision. The rods often deteriorate and die, leading to a complete loss of night vision. Subsequently, the cones, which mediate daylight and color vision, also die, over a period of years, leading to a complete loss of all vision.

We are interested in learning how to stop the progression of RP. To this end, we have investigated the causes of cone death using microarrays and 4 mouse models of RP, looking for changes that are in common across all 4 models at the onset of cone death. Genes that are involved in metabolism were those with the most frequently observed changes. These observations were followed up through several independent assays. The results suggest that cones are starving, potentially leading to their malfunction and death. In addition, we examined the activity of histone deacetylase 4 (HDAC4) in promoting rod survival. When overexpressed in rods, HDAC4 significantly prolonged rod survival, and indirectly, cone survival. This activity required the activity of hypoxia inducible factor 1alpha.

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NLM Title: Strategies to slow the onset of blindness in retinitis pigmentosa / Connie Cepko.
Author: Cepko, Constance.
National Institutes of Health (U.S.)
Publisher:
Abstract: (CIT): The disease, retinitis pigmentosa (RP), is a blinding illness with genetic etiology. Many different RP genes are expressed in rod photoreceptors exclusively. These are the retinal photoreceptors used for dim light perception, or night vision. Individuals born with these mutations have poor, or no, night vision, but typically have normal daylight and color vision. The rods often deteriorate and die, leading to a complete loss of night vision. Subsequently, the cones, which mediate daylight and color vision, also die, over a period of years, leading to a complete loss of all vision. We are interested in learning how to stop the progression of RP. To this end, we have investigated the causes of cone death using microarrays and 4 mouse models of RP, looking for changes that are in common across all 4 models at the onset of cone death. Genes that are involved in metabolism were those with the most frequently observed changes. These observations were followed up through several independent assays. The results suggest that cones are starving, potentially leading to their malfunction and death. In addition, we examined the activity of histone deacetylase 4 (HDAC4) in promoting rod survival. When overexpressed in rods, HDAC4 significantly prolonged rod survival, and indirectly, cone survival. This activity required the activity of hypoxia inducible factor 1alpha.
Subjects: Blindness--etiology
Blindness--therapy
Retinitis Pigmentosa--complications
Publication Types: Lectures
Webcasts
Download: To download this event, select one of the available bitrates:
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NLM Classification: WW 276
NLM ID: 101549164
CIT Live ID: 9525
Permanent link: https://videocast.nih.gov/launch.asp?16149

 

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