ArticlePloS one2014
Early retinal function deficit without prominent morphological changes in the R6/2 mouse model of Huntington's disease.
Article in PloS one, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 1 of them a synthesis that pooled it.
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Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Optical coherence tomography measurements in Huntington's disease: a systematic review and meta-analysis.Journal of neurology · 2024Pooled it
- Nutrient supplementation mitigates retinal dysfunction in Acox1 knockout mice with impaired peroxisomal fatty acid oxidation.Journal of advanced research · 2025Article
- Retinal Neurovascular Coupling: From Mechanisms to a Diagnostic Window into Brain Disorders.Cells · 2025Review
- Optical coherence tomography - A possible biomarker in early huntington's disease.Neurological research and practice · 2025Article
- The Retinal Dopaminergic Circuit as a Biomarker for Huntington's and Alzheimer's Diseases.International journal of molecular sciences · 2025Article
- Retinal dysfunction in Huntington's disease mouse models concurs with local gliosis and microglia activation.Scientific reports · 2024Article
- Abnormal outer and inner retina in a mouse model of Huntington's disease with age.Frontiers in aging neuroscience · 2024Article
- Intrinsic signal optoretinography of dark adaptation abnormality due to rod photoreceptor degeneration.Experimental biology and medicine (Maywood, N.J.) · 2024Article
- Increase in brain glycogen levels ameliorates Huntington's disease phenotype and rescues neurodegeneration in Drosophila.Disease models & mechanisms · 2023Article
- Retinal thickness as a biomarker of cognitive impairment in manifest Huntington's disease.Journal of neurology · 2023Article
- Transcriptional and Histone Acetylation Changes Associated with CRE Elements Expose Key Factors Governing the Regulatory Circuit in the Early Stage of Huntington's Disease Models.International journal of molecular sciences · 2023Article
- Myelinosome organelles in pathological retinas: ubiquitous presence and dual role in ocular proteostasis maintenance.Neural regeneration research · 2023Review
- Sleep Disorders and Circadian Disruption in Huntington's Disease.Journal of Huntington's disease · 2023Review
- A New Perspective on Huntington's Disease: How a Neurological Disorder Influences the Peripheral Tissues.International journal of molecular sciences · 2022Review
- Myelinosome Organelles in the Retina of R6/1 Huntington Disease (HD) Mice: Ubiquitous Distribution and Possible Role in Disease Spreading.International journal of molecular sciences · 2021Article
- Neuroprotection of retinal ganglion cells by the sigma-1 receptor agonist pridopidine in models of experimental glaucoma.Scientific reports · 2021Article
- Past, present and future role of retinal imaging in neurodegenerative disease.Progress in retinal and eye research · 2021Review
- Visual Behavior Impairments as an Aberrant Sensory Processing in the Mouse Model of Fragile X Syndrome.Frontiers in behavioral neuroscience · 2019Article
- Retinal dysfunction in a presymptomatic patient with Huntington's disease.Documenta ophthalmologica. Advances in ophthalmology · 2018Article
- Retinal Degeneration In A Mouse Model Of CLN5 Disease Is Associated With Compromised Autophagy.Scientific reports · 2017Article
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Authors and funding
11 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Huntington's disease (HD) is an inherited neurodegenerative disorder that primarily affects the medium-size GABAergic neurons of striatum. The R6/2 mouse line is one of the most widely used animal models of HD. Previously the hallmarks of HD-related pathology have been detected in photoreceptors and interneurons of R6/2 mouse retina. Here we aimed to explore the survival of retinal ganglion cells (RGCs) and functional integrity of distinct retinal cell populations in R6/2 mice. The pattern electroretinography (PERG) signal was lost at the age of 8 weeks in R6/2 mice in contrast to the situation in wild-type (WT) littermates. This defect may be attributable to a major reduction in photopic ERG responses in R6/2 mice which was more evident in b- than a-wave amplitudes. At the age of 4 weeks R6/2 mice had predominantly the soluble form of mutant huntingtin protein (mHtt) in the RGC layer cells, whereas the aggregated form of mHtt was found in the majority of those cells from the 12-week-old R6/2 mice and onwards. Retinal astrocytes did not contain mHtt deposits. The total numbers of RGC layer cells, retinal astrocytes as well as optic nerve axons did not differ between 18-week-old R6/2 mice and their WT controls. Our data indicate that mHtt deposition does not cause RGC degeneration or retinal astrocyte loss in R6/2 mice even at a late stage of HD-related pathology. However, due to functional deficits in the rod- and cone-pathways, the R6/2 mice suffer progressive deficits in visual capabilities starting as early as 4 weeks; at 8 weeks there is severe impairment. This should be taken into account in any behavioral testing conducted in R6/2 mice.
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