ArticleScientific reports2015
Electrophysiological and Histological Characterization of Rod-Cone Retinal Degeneration and Microglia Activation in a Mouse Model of Mucopolysaccharidosis Type IIIB.
Article in Scientific reports, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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18 citing papers in PubMed, 27 citations in OpenAlex.
- Ankyrin-B and ankyrin-G are required for synaptic integrity in the mouse outer retina.PNAS nexus · 2026Article
- Cerebral Hypoperfusion Causes Behavioral Changes and Impairs the Rod Photoreceptor Pathway in the Retina of Aged Mice.Cellular and molecular neurobiology · 2026Article
- Retinal microstructual and microvascular changes in mucopolysaccharidoses.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2026Article
- Retinal Function Deficits in American Staffordshire Terriers with a Late-Onset Neurodegenerative Disease Associated with anVeterinary sciences · 2025Article
- Evaluation of neuroretina following i.v. or intra-CSF AAV9 gene replacement in mice with MPS IIIA, a childhood dementia.CNS neuroscience & therapeutics · 2024Article
- TRPV4 affects visual signals in photoreceptors and rod bipolar cells.Frontiers in cellular neuroscience · 2024Article
- Coenzyme Q10 eyedrops conjugated with vitamin E TPGS alleviate neurodegeneration and mitochondrial dysfunction in the diabetic mouse retina.Frontiers in cellular neuroscience · 2024Article
- Transferrin Receptor-Targeted Iduronate-2-sulfatase Penetrates the Blood-Retinal Barrier and Improves Retinopathy in Mucopolysaccharidosis II Mice.Molecular pharmaceutics · 2023Article
- Functional and structural changes in the neuroretina are accompanied by mitochondrial dysfunction in a type 2 diabetic mouse model.Eye and vision (London, England) · 2023Article
- βII-Spectrin Is Required for Synaptic Positioning during Retinal Development.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2023Article
- Histological characterization of retinal degeneration in mucopolysaccharidosis type IIIC.Experimental eye research · 2023Article
- Autophagy in the retinal pigment epithelium: a new vision and future challenges.The FEBS journal · 2022Review
- Increased Reliability of Visually-Evoked Activity in Area V1 of the MECP2-Duplication Mouse Model of Autism.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2022Article
- Neurofascin Is a Novel Component of Rod Photoreceptor Synapses in the Outer Retina.Frontiers in neural circuits · 2021Article
- Is the eye a window to the brain in Sanfilippo syndrome?Acta neuropathologica communications · 2020Article
- Retinal Degeneration in MPS-IIIA Mouse Model.Frontiers in cell and developmental biology · 2020Article
- Lysosome biogenesis in health and disease.Journal of neurochemistry · 2019Review
- Trehalose reduces retinal degeneration, neuroinflammation and storage burden caused by a lysosomal hydrolase deficiency.Autophagy · 2018Article
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Authors and funding
5 authors at 2 institutions in 2 countries.
Funding
Abstract
Sanfilippo syndrome Type B or Mucopolysaccharidosis IIIB (MPS IIIB) is a neurodegenerative autosomal recessive lysosomal storage disorder in which patients suffer severe vision loss from associated retinopathy. Here we sought to study the underlying retinal functional and morphological changes associated with MPS IIIB disease progression using the established model of MPS IIIB, the B6.129S6-Naglu(tm1Efn)/J mouse line. Electroretinogram (ERG) was recorded from MPS IIIB and wild-type (WT) mice at the age of 28 and 46 weeks, and retinal tissues were subsequently collected for immunohistochemistry analysis. At the 28th week, rod a- and b-wave amplitudes were significantly diminished in MPS IIIB compared to WT mice. The cone a- and b-waves of MPS IIIB mice were not significantly different from those of the control at the 28th week but were significantly diminished at the 46 th week, when MPS IIIB mice showed a major loss of rods and rod bipolar cells in both central and peripheral regions and a minor loss of cones in the periphery. Activation of microglia and neovascularization were also detected in the MPS IIIB retina. The new findings that cones and rod bipolar cells also undergo degeneration, and that retinal microglia are activated, will inform future development of therapeutic strategies.
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