Leber’s Hereditary Optic Neuropathy with Mitochondrial DNA Mutation G11778A: A Systematic Literature Review and Meta-Analysis

Biomed Res Int. 2023 Jan 24;2023:1107866. doi: 10.1155/2023/1107866. eCollection 2023. ABSTRACT BACKGROUND: LHON is a progressive disease with early disease onset and male predominance, usually causing devastating visual loss to patients. These systematic review and meta-analysis are aimed at summarizing epidemiology, disease onset and progression, visual recovery, risk factors, and treatment options of Leber’s hereditary […]

A bibliometric analysis of the application of stem cells in glaucoma research from 1999 to 2022

Front Cell Dev Biol. 2023 Jan 18;11:1081898. doi: 10.3389/fcell.2023.1081898. eCollection 2023. ABSTRACT Background: Glaucoma, a neurodegenerative disease of the retina, is the leading cause of irreversible blindness. Stem cells have therapeutic potential for glaucoma. However, few bibliometric studies have been published in this field. Concerning a visual map, this article aims to characterize the research […]

Glutathione system enhancement for cardiac protection: pharmacological options against oxidative stress and ferroptosis

Cell Death Dis. 2023 Feb 16;14(2):131. doi: 10.1038/s41419-023-05645-y. ABSTRACT The glutathione (GSH) system is considered to be one of the most powerful endogenous antioxidant systems in the cardiovascular system due to its key contribution to detoxifying xenobiotics and scavenging overreactive oxygen species (ROS). Numerous investigations have suggested that disruption of the GSH system is a […]

A lncRNA-encoded mitochondrial micropeptide exacerbates microglia-mediated neuroinflammation in retinal ischemia/reperfusion injury

Cell Death Dis. 2023 Feb 15;14(2):126. doi: 10.1038/s41419-023-05617-2. ABSTRACT As a common pathology of many ocular disorders such as diabetic retinopathy and glaucoma, retinal ischemia/reperfusion (IR) triggers inflammation and microglia activation that lead to irreversible retinal damage. The detailed molecular mechanism underlying retinal IR injury, however, remains poorly understood at present. Here we report the […]

Retinal pathological features and proteome signatures of Alzheimer’s disease

Acta Neuropathol. 2023 Feb 11. doi: 10.1007/s00401-023-02548-2. Online ahead of print. ABSTRACT Alzheimer’s disease (AD) pathologies were discovered in the accessible neurosensory retina. However, their exact nature and topographical distribution, particularly in the early stages of functional impairment, and how they relate to disease progression in the brain remain largely unknown. To better understand the […]

Knockout of AMD-associated gene POLDIP2 reduces mitochondrial superoxide in human retinal pigment epithelial cells

Aging (Albany NY). 2023 Feb 16;15. doi: 10.18632/aging.204522. Online ahead of print. ABSTRACT Genetic and epidemiologic studies have significantly advanced our understanding of the genetic factors contributing to age-related macular degeneration (AMD). In particular, recent expression quantitative trait loci (eQTL) studies have highlighted POLDIP2 as a significant gene that confers risk of developing AMD. However, […]

Cone photoreceptors transfer damaged mitochondria to Müller glia

Cell Rep. 2023 Feb 15;42(2):112115. doi: 10.1016/j.celrep.2023.112115. Online ahead of print. ABSTRACT Mitochondria are vital organelles that require sophisticated homeostatic mechanisms for maintenance. Intercellular transfer of damaged mitochondria is a recently identified strategy broadly used to improve cellular health and viability. Here, we investigate mitochondrial homeostasis in the vertebrate cone photoreceptor, the specialized neuron that […]

N-Acetylcysteine and cysteamine bitartrate prevent azide-induced neuromuscular decompensation by restoring glutathione balance in two novel surf1-/- zebrafish deletion models of Leigh syndrome

Hum Mol Genet. 2023 Feb 16:ddad031. doi: 10.1093/hmg/ddad031. Online ahead of print. ABSTRACT SURF1 deficiency (OMIM # 220110) causes Leigh syndrome (LS, OMIM # 256000), a mitochondrial disorder typified by stress-induced metabolic strokes, neurodevelopmental regression, and progressive multisystem dysfunction. Here, we describe two novel surf1-/- zebrafish knockout models generated by CRISPR/Cas9 technology. While gross larval […]

Mitochondrial Genome Alterations, Cytochrome C Oxidase Activity, and Oxidative Stress: Implications in Primary Open-angle Glaucoma

J Curr Glaucoma Pract. 2022 Sep-Dec;16(3):158-165. doi: 10.5005/jp-journals-10078-1376. ABSTRACT AIM: To evaluate mitochondrial genome alterations, cytochrome c oxidase (COX) activity, and oxidative stress in primary open-angle glaucoma (POAG). METHODOLOGY: Whole mitochondrial genome was screened in 75 POAG cases and 105 controls by polymerase chain reaction (PCR) sequencing. COX activity was measured from peripheral blood mononuclear […]