Divergent Phenotypes of Vision and Accessory Visual Function in Mice with Visual Cycle Dysfunction (Rpe65rd12) or Retinal Degeneration (rd/rd)

2008 ◽  
Vol 49 (6) ◽  
pp. 2737 ◽  
Author(s):  
Stewart Thompson ◽  
Robert F. Mullins ◽  
Alisdair R. Philp ◽  
Edwin M. Stone ◽  
N. Mrosovsky
Keyword(s):  
Retinal Degeneration ◽  
Visual Function ◽  
Visual Cycle

scholarly journals Antisense oligonucleotides targeting mutant Ataxin-7 restore visual function in a mouse model of spinocerebellar ataxia type 7

2018 ◽  
Vol 10 (465) ◽  
pp. eaap8677 ◽  
Author(s):  
Chenchen Niu ◽  
Thazah P. Prakash ◽  
Aneeza Kim ◽  
John L. Quach ◽  
Laryssa A. Huryn ◽  
...  
Keyword(s):  
Retinal Degeneration ◽  
Spinocerebellar Ataxia ◽  
Visual Function ◽  
Antisense Oligonucleotides ◽  
Neurodegenerative Disorder ◽  
Retinal Disease ◽  
Fundus Photography ◽  
Spinocerebellar Ataxia Type ◽  
Autofluorescence Imaging ◽  
Spinocerebellar Ataxia Type 7

Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurodegenerative disorder characterized by cerebellar and retinal degeneration, and is caused by a CAG-polyglutamine repeat expansion in the ATAXIN-7 gene. Patients with SCA7 develop progressive cone-rod dystrophy, typically resulting in blindness. Antisense oligonucleotides (ASOs) are single-stranded chemically modified nucleic acids designed to mediate the destruction, prevent the translation, or modify the processing of targeted RNAs. Here, we evaluated ASOs as treatments for SCA7 retinal degeneration in representative mouse models of the disease after injection into the vitreous humor of the eye. Using Ataxin-7 aggregation, visual function, retinal histopathology, gene expression, and epigenetic dysregulation as outcome measures, we found that ASO-mediated Ataxin-7 knockdown yielded improvements in treated SCA7 mice. In SCA7 mice with retinal disease, intravitreal injection of Ataxin-7 ASOs also improved visual function despite initiating treatment after symptom onset. Using color fundus photography and autofluorescence imaging, we also determined the nature of retinal degeneration in human SCA7 patients. We observed variable disease severity and cataloged rapidly progressive retinal degeneration. Given the accessibility of neural retina, availability of objective, quantitative readouts for monitoring therapeutic response, and the rapid disease progression in SCA7, ASOs targeting ATAXIN-7 might represent a viable treatment for SCA7 retinal degeneration.

scholarly journals Retinal degeneration associated with RDH12 mutations results from decreased 11-cis retinal synthesis due to disruption of the visual cycle

2006 ◽  
Vol 15 (9) ◽  
pp. 1559-1559
Author(s):  
Debra A. Thompson ◽  
Andreas R. Janecke ◽  
Jessica Lange ◽  
Kecia L. Feathers ◽  
Christian A. Hübner ◽  
...  
Keyword(s):  
Retinal Degeneration ◽  
Visual Cycle

scholarly journals Retinal degeneration in animal models with a defective visual cycle

2013 ◽  
Vol 10 (4) ◽  
pp. e163-e172 ◽  
Author(s):  
Akiko Maeda ◽  
Krzysztof Palczewski
Keyword(s):  
Animal Models ◽  
Retinal Degeneration ◽  
Visual Cycle

scholarly journals Curcumin Delays Retinal Degeneration by Regulating Microglia Activation in the Retina of rd1 Mice

2017 ◽  
Vol 44 (2) ◽  
pp. 479-493 ◽  
Author(s):  
Yanhe Wang ◽  
Zhiyuan Yin ◽  
Lixiong Gao ◽  
Dayu Sun ◽  
Xisu Hu ◽  
...  
Keyword(s):  
Retinal Degeneration ◽  
Neurodegenerative Disorders ◽  
Visual Function ◽  
Underlying Mechanism ◽  
Microglia Activation ◽  
Swine Model ◽  
Behavioral Tests ◽  
Curcumin Treatment ◽  
Neuroprotective Effects ◽  
Bv2 Microglial Cells

Background/Aims: Retinitis pigmentosa (RP) is characterized by degeneration of photoreceptors, and there are currently no effective treatments for this disease. However, curcumin has shown neuroprotectant efficacy in a RP rat and swine model, and thus, may have neuroprotective effects in this disease. Methods: Immunofluorescence staining, electroretinogram recordings, and behavioral tests were used to analyze the effects of curcumin and the underlying mechanism in retinal degeneration 1 (rd1) mice. Results: The number of apoptotic cells in the retina of rd1 mice at postnatal day 14 significantly decreased with curcumin treatment and visual function was improved. The activation of microglia and secretion of chemokines and matrix metalloproteinases in the retina were inhibited by curcumin. These effects were also observed in a co-culture of BV2 microglial cells and retina-derived 661W cells. Conclusions: Curcumin delayed retinal degeneration by suppressing microglia activation in the retina of rd1 mice. Thus, it may be an effective treatment for neurodegenerative disorders such as RP.

scholarly journals Restoration of visual function in retinal degeneration mice by ectopic expression of melanopsin

2008 ◽  
Vol 105 (41) ◽  
pp. 16009-16014 ◽  
Author(s):  
B. Lin ◽  
A. Koizumi ◽  
N. Tanaka ◽  
S. Panda ◽  
R. H. Masland
Keyword(s):  
Retinal Degeneration ◽  
Visual Function ◽  
Ectopic Expression

scholarly journals Reversal of end-stage retinal degeneration and restoration of visual function by photoreceptor transplantation

2013 ◽  
Vol 110 (3) ◽  
pp. 1101-1106 ◽  
Author(s):  
M. S. Singh ◽  
P. Charbel Issa ◽  
R. Butler ◽  
C. Martin ◽  
D. M. Lipinski ◽  
...  
Keyword(s):  
Retinal Degeneration ◽  
Visual Function ◽  
End Stage

scholarly journals Retinal degeneration associated with RDH12 mutations results from decreased 11- cis retinal synthesis due to disruption of the visual cycle

2005 ◽  
Vol 14 (24) ◽  
pp. 3865-3875 ◽  
Author(s):  
Debra A. Thompson ◽  
Andreas R. Janecke ◽  
Jessica Lange ◽  
Kecia L. Feathers ◽  
Christian A. Hübner ◽  
...  
Keyword(s):  
Retinal Degeneration ◽  
Visual Cycle
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