Category: Ophthalmology AND mitochondrial

NAD(+) Supplementation Improves Mitochondrial Functions and Normalizes Glaucomatous Trabecular Meshwork Features

Exp Cell Res. 2024 Jun 17:114137. doi: 10.1016/j.yexcr.2024.114137. Online ahead of print. ABSTRACT Glaucoma is characterized by pathological elevation of intraocular pressure (IOP) due to dysfunctional trabecular meshwork (TM), which is the primary cause of irreversible vision loss. There are currently no effective treatment strategies for glaucoma. Mitochondrial function plays a crucial role in regulating […]

Spinocerebellar ataxias: from pathogenesis to recent therapeutic advances

Front Neurosci. 2024 Jun 4;18:1422442. doi: 10.3389/fnins.2024.1422442. eCollection 2024. ABSTRACT Spinocerebellar ataxia is a phenotypically and genetically heterogeneous group of autosomal dominant-inherited degenerative disorders. The gene mutation spectrum includes dynamic expansions, point mutations, duplications, insertions, and deletions of varying lengths. Dynamic expansion is the most common form of mutation. Mutations often result in indistinguishable clinical […]

Manipulating mitochondrial pyruvate carrier function causes metabolic remodeling in corneal myofibroblasts that ameliorates fibrosis

Redox Biol. 2024 Jun 8;75:103235. doi: 10.1016/j.redox.2024.103235. Online ahead of print. ABSTRACT Myofibroblasts are key cellular effectors of corneal wound healing from trauma, surgery, or infection. However, their persistent deposition of disorganized extracellular matrix can also cause corneal fibrosis and visual impairment. Recent work showed that the PPARγ agonist Troglitazone can mitigate established corneal fibrosis, […]

Optic Nerve T2 Signal Intensity and Caliber Reflect Clinical Severity in Genetic Optic Atrophy

J Neuroophthalmol. 2024 Jun 17. doi: 10.1097/WNO.0000000000002191. Online ahead of print. ABSTRACT BACKGROUND: Genetic optic atrophies comprise phenotypically heterogenous disorders of mitochondrial function. We aimed to correlate quantitative neuroimaging findings of the optic nerves in these disorders with clinical measures. METHODS: From a retrospective database of 111 patients with bilateral optic atrophy referred for genetic […]

Mir-204-5p alleviates mitochondrial dysfunction by targeting IGFBP5 in diabetic cataract

Mol Biol Rep. 2024 Jun 14;51(1):755. doi: 10.1007/s11033-024-09701-4. ABSTRACT BACKGROUND: Cataract contributes to visual impairment worldwide, and diabetes mellitus accelerates the formation and progression of cataract. Here we found that the expression level of miR-204-5p was diminished in the lens epithelium with anterior lens capsule of cataract patients compared to normal donors, and decreased more […]

Alternative oxidase blunts pseudohypoxia and photoreceptor degeneration due to RPE mitochondrial dysfunction

Proc Natl Acad Sci U S A. 2024 Jun 18;121(25):e2402384121. doi: 10.1073/pnas.2402384121. Epub 2024 Jun 12. ABSTRACT Loss of mitochondrial electron transport complex (ETC) function in the retinal pigment epithelium (RPE) in vivo results in RPE dedifferentiation and progressive photoreceptor degeneration, and has been implicated in the pathogenesis of age-related macular degeneration. Xenogenic expression of […]

Electrodiagnostic tests of the visual pathway and applications in neuro-ophthalmology

Eye (Lond). 2024 Jun 11. doi: 10.1038/s41433-024-03154-6. Online ahead of print. ABSTRACT This article describes the main visual electrodiagnostic tests relevant to neuro-ophthalmology practice, including the visual evoked potential (VEP), and the full-field, pattern and multifocal electroretinograms (ffERG; PERG; mfERG). The principles of electrophysiological interpretation are illustrated with reference to acquired and inherited optic neuropathies, […]

Reassessing Retinal Pigment Epithelial Ketogenesis: Enzymatic Assays for Ketone Body Levels Provide Inaccurate Results

Exp Eye Res. 2024 Jun 8:109966. doi: 10.1016/j.exer.2024.109966. Online ahead of print. ABSTRACT The retinal pigment epithelium (RPE) is omnivorous and can utilize a wide range of substrates for oxidative phosphorylation. Certain tissues with high mitochondrial metabolic load are capable of ketogenesis, a biochemical pathway that consolidates acetyl-CoA into ketone bodies. Earlier work demonstrated that […]

IP3R2 regulates apoptosis by Ca2+ transfer through mitochondria-ER contacts in hypoxic photoreceptor injury

Exp Eye Res. 2024 Jun 6:109965. doi: 10.1016/j.exer.2024.109965. Online ahead of print. ABSTRACT Mitochondria-associated ER membranes (MAMs) are contact sites that enable bidirectional communication between the ER (endoplasmic reticulum) and mitochondria, including the transfer of Ca2+ signals. MAMs are essential for mitochondrial function and cellular energy metabolism. However, unrestrained Ca2+ transfer to the mitochondria can […]