Leber Hereditary Optic Neuropathy Case Report: Clinical Presentation and Treatment with Idebenone Reinforce the Evidence for m.3866T>C as a Causative Variant

Case Rep Ophthalmol. 2024 Jun 20;15(1):513-517. doi: 10.1159/000539445. eCollection 2024 Jan-Dec. ABSTRACT INTRODUCTION: Leber hereditary optic neuropathy (LHON) is a mitochondrial disorder that typically presents with painless, central visual loss, hyperaemia of the optic nerve head, and peripapillary telangiectasias. Most LHON cases are due to one of three variants, but several less common variants also […]

Single-cell transcriptomic analysis reveals the antiangiogenic role of Mgarp in diabetic retinopathy

BMJ Open Diabetes Res Care. 2024 Jul 16;12(4):e004189. doi: 10.1136/bmjdrc-2024-004189. ABSTRACT INTRODUCTION: Diabetic retinopathy (DR) is a common vascular complication of diabetes mellitus and a leading cause of vision loss worldwide. Endothelial cell (EC) heterogeneity has been observed in the pathogenesis of DR. Elucidating the underlying mechanisms governing EC heterogeneity may provide novel insights into […]

Organic photovoltaic biomaterial with fullerene derivatives for near-infrared light sensing in neural cells

Biointerphases. 2024 Jul 1;19(4):041001. doi: 10.1116/6.0003279. ABSTRACT Retinal degenerative diseases, which can lead to photoreceptor cell apoptosis, have now become the leading irreversible cause of blindness worldwide. In this study, we developed an organic photovoltaic biomaterial for artificial retinas, enabling neural cells to detect photoelectric stimulation. The biomaterial was prepared using a conjugated polymer donor, […]

Protocol for real-time measurement of mitochondrial respiration in the mouse ocular posterior pole using a Seahorse XFe24 analyzer

STAR Protoc. 2024 Jul 11;5(3):103150. doi: 10.1016/j.xpro.2024.103150. Online ahead of print. ABSTRACT During aging and in retinal degenerative diseases, vulnerable retinal pigment epithelial (RPE) cells are subject to mitochondrial dysfunction, creating a need for accessibility to tools which can facilitate assessment of the ocular posterior pole bioenergetics. Here, we present a protocol for quantifying mitochondrial […]

Daily Light Onset and Plasma Membrane Tethers Regulate Mitochondria Redistribution within the Retinal Pigment Epithelium

Cells. 2024 Jun 25;13(13):1100. doi: 10.3390/cells13131100. ABSTRACT The retinal pigment epithelium (RPE) is an essential component of the retina that plays multiple roles required to support visual function. These include light onset- and circadian rhythm-dependent tasks, such as daily phagocytosis of photoreceptor outer segments. Mitochondria provide energy to the highly specialized and energy-dependent RPE. In […]

Biomechanic, proteomic and miRNA transcriptional changes in the trabecular meshwork of primates injected with intravitreal triamcinolone

Vision Res. 2024 Jul 10;222:108456. doi: 10.1016/j.visres.2024.108456. Online ahead of print. ABSTRACT Although biomechanical changes of the trabecular meshwork (TM) are important to the pathogenesis of glucocorticoids-induced ocular hypertension (GC-OHT), there is a knowledge gap in the underlying molecular mechanisms of the development of it. In this study, we performed intravitreal triamcinolone injection (IVTA) in […]

Uncovering the role of ferroptosis in Bietti crystalline dystrophy and potential therapeutic strategies

Cell Commun Signal. 2024 Jul 11;22(1):359. doi: 10.1186/s12964-024-01710-x. ABSTRACT PURPOSE: Bietti crystalline dystrophy (BCD) is an inherited retinal degeneration disease caused by mutations in the CYP4V2 gene. Currently, there is no clinical therapy approach available for BCD patients. Previous research has suggested that polyunsaturated fatty acids (PUFAs) may play a significant role in the development […]

Leber’s hereditary optic neuropathy: Update on current diagnosis and treatment

Front Ophthalmol (Lausanne). 2023 Jan 11;2:1077395. doi: 10.3389/fopht.2022.1077395. eCollection 2022. ABSTRACT Leber’s hereditary optic neuropathy (LHON) is a fairly prevalent mitochondrial disorder (1:50,000) arising from the dysfunction of the mitochondrial respiratory chain, which eventually leads to apoptosis of retinal ganglion cells. The usual presentation is that of a young male with a sequential reduction in […]

Flavoprotein fluorescence elevation is a marker of mitochondrial oxidative stress in patients with retinal disease

Front Ophthalmol (Lausanne). 2023 Feb 16;3:1110501. doi: 10.3389/fopht.2023.1110501. eCollection 2023. ABSTRACT PURPOSE: Recent studies of glaucoma, age-related macular degeneration, and diabetic retinopathy have demonstrated that flavoprotein fluorescence (FPF) can be utilized non-invasively as an indicator of mitochondrial oxidative stress in the retina. However, a comprehensive assessment of the validity and reliability of FPF in differentiating […]