scholarly journals Necrotic cone photoreceptor cell death in retinitis pigmentosa

2015 ◽  
Vol 6 (12) ◽  
pp. e2038-e2038 ◽  
Author(s):  
Y Murakami ◽  
Y Ikeda ◽  
S Nakatake ◽  
J W Miller ◽  
D G Vavvas ◽  
...  
Keyword(s):  
Cell Death ◽  
Retinitis Pigmentosa ◽  
Photoreceptor Cell ◽  
Cone Photoreceptor

scholarly journals Knockout of ccr2 alleviates photoreceptor cell death in a model of retinitis pigmentosa

2012 ◽  
Vol 104 ◽  
pp. 39-47 ◽  
Author(s):  
Congrong Guo ◽  
Atsushi Otani ◽  
Akio Oishi ◽  
Hiroshi Kojima ◽  
Yukiko Makiyama ◽  
...  
Keyword(s):  
Cell Death ◽  
Retinitis Pigmentosa ◽  
Photoreceptor Cell

Apoptosis, retinitis pigmentosa, and degeneration

1994 ◽  
Vol 72 (11-12) ◽  
pp. 489-498 ◽  
Author(s):  
Paul Wong
Keyword(s):  
Cell Death ◽  
Retinitis Pigmentosa ◽  
Retinal Degeneration ◽  
Photoreceptor Cell ◽  
Retinal Cell ◽  
Current Evidence ◽  
Cell Degeneration ◽  
Genetic Lesion ◽  
Common Causes ◽  
Active Cell Death

The mechanism of photoreceptor cell death in different inherited retinal degenerations is not fully understood. Mutations in a number of different genes (such as rhodopsin, the beta subunit of cGMP phosphodiesterase, and peripherin) have been identified as the primary genetic lesion in different forms of human retinitis pigmentosa, one of the most common causes of inherited blindness. In all cases the manifestation of the disorder regardless of the specific primary genetic lesion is similar, resulting in photoreceptor cell degeneration and blindness. A recent hypothesis is that the active photoreceptor cell death, which is characteristic of these genetically distinct disorders, is mediated by a common induction of apoptosis. In the present review, the current evidence for active cell death during retinal cell death in several different rodent models of retinitis pigmentosa and retinal degeneration is examined.Key words: retinal degeneration, apoptosis, retinitis pigmentosa, clusterin, DNA fragmentation.

scholarly journals Metabolite therapy guided by liquid biopsy proteomics delays retinal neurodegeneration

2019 ◽  
Author(s):  
Katherine J. Wert ◽  
Gabriel Velez ◽  
Kanchustambham Vijayalakshmi ◽  
Vishnu Shankar ◽  
Jesse D. Sengillo ◽  
...  
Keyword(s):  
Cell Death ◽  
Retinitis Pigmentosa ◽  
Ketogenic Diet ◽  
Proteomic Analysis ◽  
Photoreceptor Cell ◽  
Neuroprotective Effect ◽  
Tca Cycle ◽  
Ionization Mass ◽  
Molecular Pathways ◽  
Aconitic Acid

AbstractNeurodegenerative diseases are debilitating, incurable disorders caused by progressive neuronal cell death. Retinitis pigmentosa (RP) is a blinding neurodegenerative disease that results in retinal photoreceptor cell death and progresses to the loss of the entire neural retinal network. We previously found that proteomic analysis of the adjacent vitreous serves as way to indirectly biopsy the neural retina and identify changes in the retinal proteome. We therefore analyzed protein expression in liquid vitreous biopsies from autosomal recessive retinitis pigmentosa (arRP) patients with PDE6A mutations and arRP mice with Pde6ɑ mutations. Proteomic analysis of retina and vitreous samples identified molecular pathways affected at the onset of photoreceptor cell death. Based on affected molecular pathways, arRP mice were treated with a ketogenic diet or metabolites involved in fatty-acid synthesis, oxidative phosphorylation, and the tricarboxylic acid (TCA) cycle. Dietary supplementation of a single metabolite, ɑ-ketoglutarate, increased docosahexaeonic acid (DHA) levels, provided neuroprotection, and enhanced visual function in arRP mice. A ketogenic diet delayed photoreceptor cell loss, while vitamin B supplementation had a limited effect. Finally, desorption electrospray ionization mass spectrometry imaging (DESI-MSI) revealed restoration of key metabolites that correlated with our proteomic findings: pyrimidine and purine metabolism (uridine, dihydrouridine, and thymidine), glutamine and glutamate (glutamine/glutamate conversion), and succinic and aconitic acid (TCA cycle). This study demonstrates that replenishing TCA cycle metabolites via oral supplementation prolongs vision and provides a neuroprotective effect on the photoreceptor cells and inner retinal network.One Sentence SummaryThe study shows protein and metabolite pathways affected during neurodegeneration and that replenishing metabolites provides a neuroprotective effect on the retina.

scholarly journals Absence of Sigma 1 Receptor Accelerates Photoreceptor Cell Death in a Murine Model of Retinitis Pigmentosa

2017 ◽  
Vol 58 (11) ◽  
pp. 4545 ◽  
Author(s):  
Jing Wang ◽  
Alan Saul ◽  
Xuezhi Cui ◽  
Penny Roon ◽  
Sylvia B. Smith
Keyword(s):  
Cell Death ◽  
Retinitis Pigmentosa ◽  
Murine Model ◽  
Photoreceptor Cell ◽  
Sigma 1 Receptor ◽  
Sigma 1

scholarly journals PKG activity causes photoreceptor cell death in two retinitis pigmentosa models

2009 ◽  
Vol 108 (3) ◽  
pp. 796-810 ◽  
Author(s):  
François Paquet-Durand ◽  
Stefanie M. Hauck ◽  
Theo van Veen ◽  
Marius Ueffing ◽  
Per Ekström
Keyword(s):  
Cell Death ◽  
Retinitis Pigmentosa ◽  
Photoreceptor Cell

scholarly journals NLRP3 inflammasome activation drives bystander cone photoreceptor cell death in a P23H rhodopsin model of retinal degeneration

2016 ◽  
Vol 25 (8) ◽  
pp. 1501-1516 ◽  
Author(s):  
Ishaq A. Viringipurampeer ◽  
Andrew L. Metcalfe ◽  
Abu E. Bashar ◽  
Olena Sivak ◽  
Anat Yanai ◽  
...  
Keyword(s):  
Cell Death ◽  
Retinal Degeneration ◽  
Nlrp3 Inflammasome ◽  
Photoreceptor Cell ◽  
Inflammasome Activation ◽  
Cone Photoreceptor ◽  
Nlrp3 Inflammasome Activation
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