Accumulation of branched-chain amino acids deteriorates the neuroinflammatory response of Müller cells in diabetic retinopathy via leucine/Sestrin2-mediated sensing of mTOR signaling
Acta Diabetol. 2024 Aug 16. doi: 10.1007/s00592-024-02349-3. Online ahead of print. ABSTRACT AIMS: This study aimed to investigate branched-chain amino acid (BCAA) catabolism in diabetic retinopathy (DR). METHODS: Wild-type and db/db mice were fed BCAAs (5 or 10 mg/kg/day) for 12 weeks, and hyperglycemia-exposed Müller cells were treated with BCAAs (2 or 5 mmol/L) for […]
The genomic mosaic of mitochondrial dysfunction: Decoding nuclear and mitochondrial epigenetic contributions to maternally inherited diabetes and deafness pathogenesis
Heliyon. 2024 Jul 17;10(14):e34756. doi: 10.1016/j.heliyon.2024.e34756. eCollection 2024 Jul 30. ABSTRACT AIMS: Maternally inherited diabetes and deafness (MIDD) is a complex disorder characterized by multiorgan clinical manifestations, including diabetes, hearing loss, and ophthalmic complications. This pilot study aimed to elucidate the intricate interplay between nuclear and mitochondrial genetics, epigenetic modifications, and their potential implications in […]
Photobiomodulation efficacy in age-related macular degeneration: a systematic review and meta-analysis of randomized clinical trials
Int J Retina Vitreous. 2024 Aug 15;10(1):54. doi: 10.1186/s40942-024-00569-x. ABSTRACT BACKGROUND: Age-related macular degeneration (AMD) is a leading cause of vision loss. Photobiomodulation (PBM) offers a controversial approach for managing dry AMD, aiming to halt or reverse progression through mitochondrial activity modulation. However, the efficacy and clinical relevance of PBM as a potential approach for […]
Dysregulation of mitochondria, apoptosis and mitophagy in Leber’s hereditary optic neuropathy with MT-ND1 3635G>A mutation
Gene. 2024 Aug 13:148853. doi: 10.1016/j.gene.2024.148853. Online ahead of print. ABSTRACT Leber’s hereditary optic neuropathy (LHON) is a maternal inherited disorder, primarily due to mitochondrial DNA (mtDNA) mutations. This investigation aimed to assess the pathogenicity of m.3635G>A alteration known to confer susceptibility to LHON. The disruption of electrostatic interactions among S110 of the MT-ND1 and […]
Generation of an Armcx1 Conditional Knockout Mouse
Genesis. 2024 Aug;62(4):e23615. doi: 10.1002/dvg.23615. ABSTRACT Armadillo repeat-containing X-linked protein-1 (Armcx1) is a poorly characterized transmembrane protein that regulates mitochondrial transport in neurons. Its overexpression has been shown to induce neurite outgrowth in embryonic neurons and to promote retinal ganglion cell (RGC) survival and axonal regrowth in a mouse optic nerve crush model. In order […]
High-throughput ultrastructural analysis of macular telangiectasia type 2
Front Ophthalmol (Lausanne). 2024 Jul 30;4:1428777. doi: 10.3389/fopht.2024.1428777. eCollection 2024. ABSTRACT INTRODUCTION: Macular Telangiectasia type 2 (MacTel), is an uncommon form of late-onset, slowly-progressive macular degeneration. Associated with regional Müller glial cell loss in the retina and the amino acid serine synthesized by Müller cells, the disease is functionally confined to a central retinal region […]
Inherited mitochondrial dysfunction triggered by OPA1 mutation impacts the sensory innervation fibre identity, functionality and regenerative potential in the cornea
Sci Rep. 2024 Aug 13;14(1):18794. doi: 10.1038/s41598-024-68994-4. ABSTRACT Mitochondrial dysfunctions are detrimental to organ metabolism. The cornea, transparent outmost layer of the eye, is prone to environmental aggressions, such as UV light, and therefore dependent on adequate mitochondrial function. While several reports have linked corneal defects to mitochondrial dysfunction, the impact of OPA1 mutation, known […]
MSC-mediated mitochondrial transfer restores mitochondrial DNA and function in neural progenitor cells of Leber’s hereditary optic neuropathy
Sci China Life Sci. 2024 Aug 8. doi: 10.1007/s11427-024-2647-8. Online ahead of print. ABSTRACT Leber’s hereditary optic neuropathy (LHON) is a debilitating mitochondrial disease associated with mutations in mitochondrial DNA (mtDNA). Unfortunately, the available treatment options for LHON patients are limited due to challenges in mitochondrial replacement. In our study, we reprogramming LHON urine cells […]
Effects of Acute High-Altitude Exposure on Morphology and Function of Retinal Ganglion Cell in Mice
Invest Ophthalmol Vis Sci. 2024 Aug 1;65(10):19. doi: 10.1167/iovs.65.10.19. ABSTRACT PURPOSE: High altitude retinopathy (HAR) is a retinal functional disorder caused by inadequate adaptation after exposure to high altitude. However, the cellular and molecular mechanisms underlying retinal dysfunction remain elusive. Retinal ganglion cell (RGC) injury is the most important pathological basis for most retinal and […]
Melanin-Like Nanomedicine Functions as a Novel RPE Ferroptosis Inhibitor to Ameliorate Retinal Degeneration and Visual Impairment in Dry Age-Related Macular Degeneration
Adv Healthc Mater. 2024 Aug 11:e2401613. doi: 10.1002/adhm.202401613. Online ahead of print. ABSTRACT Ferrous ion accumulation and lethal oxidative stress mediate irreversible retinal pigment epithelial (RPE) cell ferroptosis and subsequent photoreceptor degeneration, a potential key pathogenic factor in the onset of dry age-related macular degeneration (dAMD), causing irreversible vision loss in the global elderly population. […]