Peroxisomal disorders and the PEX1-G844D mouse model for mild Zellweger Spectrum Disorder (ZSD), leads to retinal degeneration and vision loss. Catherine Argyriou will describe how her team used AAV8-mediated gene delivery to robustly and durably improve functional vision, retinal response, retinal structure, and biochemical metabolites in ZSD-mice.
This presentation will outline the pathobiology and visual phenotype of immune mediated optic neuropathies that include Multiple Sclerosis (MS), Neuromylitis optica spectrum disorders (NMOSD), and Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
In retinal vein occlusion (RVO), non-apoptotic expression of endothelial caspase-9 (EC Casp9) induces pathology including retinal edema, capillary ischemia, and neurodegeneration. Crystal Colón Ortiz presents mechanistic insights into EC Casp9 action, and the behavioral consequences such as contrast sensitivity decline.
Find us at booth #2523 in Denver! After more than 3 years, we cannot wait to connect with you in-person again.
Jana Remlinger et al. showed preserved visual function and ameliorated course of disease after anti-FcRn treatment in an experimental model using a monoclonal MOG-IgG to mimic MOGAD.
In this “OptoDrum Applications” video, Dr. Maximiliano Presa from The Jackson Laboratory presents results of his research in a model of Multiple Sulfatase Deficiency (MSD).
Demyelinating autoimmune diseases usually impact the optic nerve and thus visual abilities. Optomotor reflex measurements can therefore be used to non-invasively monitor disease progression and treatment efficacy. Remlinger et al studied MOGAD, a rare autoimmune demyelinating CNS disorder, in a murine model of experimental autoimmune encephalomyelitis (EAE). They assessed the effects of treating those mice with monoclonal antibodies against the neonatal Fc receptor and found that this treatment reduced the severity of the disease. Measurements of visual acuity with Striatech’s OptoDrum correlated well with other disease markers.
Scientists from Harvard Medical School were able to reverse the consequences of aging in the visual system, successfully treating optic nerve injury and glaucoma. Here is an overview of their 2020 study and it´s key findings.
Experimental autoimmune encephalomyelitis (EAE) is the most widely used animal model for multiple sclerosis (MS), an autoimmune disease where the immune system attacks myelin, which coats neurons in the CNS. Histamine has been thought to play a role in MS, effecting disease progression either positively or negatively, depending on the location of histamine action. In this study, Morin et al created a conditional mouse KO model of Hdc, the enzyme that synthesizes histamine, and induced EAE in these mice. Surprisingly, while some phenotypes in these mice were consistent with the lack of histamine, it had no impact on the development and severity of EAE. One behavioral readout of the disease progression in EAE is the decline of visual acuity, which can be measured with our OptoDrum. Consistent with the other observation, visual acuity declined in EAE Hdc-KO animals in the same way as in EAE Hdc-WT animals.
CLN disease, a subtype of Batten disease, is characterized by progressive neurodegeneration with onset in early childhood. This also affects vision. Some immunomodulatory drugs are clinically established to significantly attenuate the condition. Groh et al show in a mouse model of CLN1 that such treatment can help both when given before symptom onset, and to a lesser degree also during disease progression, by interfering with neuroinflammatory activity of immune cells.
