Retinitis pigmentosa: cone photoreceptors starving to death

2009 ◽  
Vol 12 (1) ◽  
pp. 5-6 ◽  
Author(s):  
Paola Bovolenta ◽  
Elsa Cisneros
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors

scholarly journals Genetic Reactivation of Cone Photoreceptors Restores Visual Responses in Retinitis Pigmentosa

Science ◽  
2010 ◽  
Vol 329 (5990) ◽  
pp. 413-417 ◽  
Author(s):  
V. Busskamp ◽  
J. Duebel ◽  
D. Balya ◽  
M. Fradot ◽  
T. J. Viney ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors ◽  
Visual Responses

scholarly journals ℮-conome: an automated tissue counting platform of cone photoreceptors for rodent models of retinitis pigmentosa

2011 ◽  
Vol 11 (1) ◽  
Author(s):  
Emmanuelle Clérin ◽  
Nicolas Wicker ◽  
Saddek Mohand-Saïd ◽  
Olivier Poch ◽  
José-Alain Sahel ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Rodent Models ◽  
Cone Photoreceptors

scholarly journals AAV-Txnip prolongs cone survival and vision in mouse models of retinitis pigmentosa

2021 ◽  
Author(s):  
Yunlu Xue ◽  
Sean K. Wang ◽  
Parimal Rana ◽  
Emma R. West ◽  
Christin M. Hong ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Mouse Models ◽  
Retinal Disease ◽  
Disease Genes ◽  
Cone Photoreceptors ◽  
Adeno Associated Virus ◽  
Rescue Effect ◽  
Inherited Retinal Disease ◽  
High Acuity

AbstractRetinitis pigmentosa (RP) is an inherited retinal disease, affecting >20 million people worldwide. Loss of daylight vision typically occurs due to the dysfunction/loss of cone photoreceptors, the cell type that initiates our color and high acuity vision. Currently, there is no effective treatment for RP, other than gene therapy for a limited number of specific disease genes. To develop a gene-agnostic therapy, we screened ≈20 genes for their ability to prolong cone photoreceptor survival in vivo. Here, we report an adeno-associated virus (AAV) vector expressing Txnip, which prolongs the survival of cone photoreceptors and improves visual acuity in RP mouse models. A Txnip allele, C247S, which blocks the association of Txnip with thioredoxin, provides an even greater benefit. Additionally, the rescue effect of Txnip depends on lactate dehydrogenase b (Ldhb), and correlates with the presence of healthier mitochondria, suggesting that Txnip saves RP cones by enhancing their lactate catabolism.

Measuring dysfunction of cone photoreceptors in retinitis pigmentosa with phase-sensitive AO-OCT

2020 ◽  
Author(s):  
Ayoub Lassoued ◽  
Furu Zhang ◽  
Kazuhiro Kurokawa ◽  
Yan Liu ◽  
James A. Crowell ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors ◽  
Phase Sensitive

Functional rescue of cone photoreceptors in retinitis pigmentosa

2013 ◽  
Vol 251 (7) ◽  
pp. 1669-1677 ◽  
Author(s):  
José-Alain Sahel ◽  
Thierry Léveillard ◽  
Serge Picaud ◽  
Deniz Dalkara ◽  
Katia Marazova ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors

scholarly journals Metabolic rescue of cone photoreceptors in retinitis pigmentosa

2021 ◽  
Vol 0 (0) ◽  
pp. 0
Author(s):  
HenryJ Kaplan ◽  
Wei Wang ◽  
Niloofar Piri ◽  
DouglasC Dean
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors

scholarly journals AAV-Txnip prolongs cone survival and vision in mouse models of retinitis pigmentosa

eLife ◽  
2021 ◽  
Vol 10 ◽  
Author(s):  
Yunlu Xue ◽  
Sean K Wang ◽  
Parimal Rana ◽  
Emma R West ◽  
Christin M Hong ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Mouse Models ◽  
Retinal Disease ◽  
Disease Genes ◽  
Cone Photoreceptors ◽  
Adeno Associated Virus ◽  
Rescue Effect ◽  
Inherited Retinal Disease ◽  
High Acuity

Retinitis pigmentosa (RP) is an inherited retinal disease, affecting >20 million people worldwide. Loss of daylight vision typically occurs due to the dysfunction/loss of cone photoreceptors, the cell type that initiates our color and high acuity vision. Currently, there is no effective treatment for RP, other than gene therapy for a limited number of specific disease genes. To develop a disease gene-agnostic therapy, we screened 20 genes for their ability to prolong cone photoreceptor survival in vivo. Here, we report an adeno-associated virus (AAV) vector expressing Txnip, which prolongs the survival of cone photoreceptors and improves visual acuity in RP mouse models. A Txnip allele, C247S, which blocks the association of Txnip with thioredoxin, provides an even greater benefit. Additionally, the rescue effect of Txnip depends on lactate dehydrogenase b (Ldhb), and correlates with the presence of healthier mitochondria, suggesting that Txnip saves RP cones by enhancing their lactate catabolism.

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