Wolfberry water extract attenuates blue light-emitting diode damage to ARPE-19 cells and mouse retina by activating the NRF2 signaling pathway
J Food Sci. 2023 Apr 6. doi: 10.1111/1750-3841.16559. Online ahead of print. ABSTRACT The wolfberry is believed to improve eyesight in traditional Chinese medicine. Soaking wolfberry in thermos cups has become a common health-preserving practice. The object of this paper was to research the protective effects of wolfberry water extract (WWE) on oxidative injury induced […]
Nonsyndromic Retinitis Pigmentosa Overview
2000 Aug 4 [updated 2023 Mar 30]. In: Adam MP, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2023. ABSTRACT The goals of this overview on nonsyndromic retinitis pigmentosa are the following. GOAL 1: Describe the clinical characteristics of nonsyndromic retinitis pigmentosa. […]
Ocular Stress Enhances Contralateral Transfer of Lenadogene Nolparvovec Gene Therapy through Astrocyte Networks
Mol Ther. 2023 Apr 3:S1525-0016(23)00195-8. doi: 10.1016/j.ymthe.2023.03.035. Online ahead of print. ABSTRACT Lenadogene nolparvovec (GS010) was developed to treat a point mutation in mitochondrial ND4 that causes Leber hereditary optic neuropathy. Lenadogene nolparvovec delivers human cDNA encoding wild-type ND4 packaged into an rAAV2/2 vector that transduces retinal ganglion cells, to induce allotopic expression of hybrid […]
SRT1720 attenuates UVA-induced corneal endothelial damage via inhibition of oxidative stress and cellular apoptosis
Exp Eye Res. 2023 Apr 2:109464. doi: 10.1016/j.exer.2023.109464. Online ahead of print. ABSTRACT Corneal endothelium is mostly sensitive to oxidative pressure and mitochondrial dysfunction. However, the oxidative-antioxidant mechanism of corneal endothelial cells (CECs) remains partially defined. Silent information regulator 1 (SIRT1) is a well-studied therapeutic target of oxidative damage. This study aimed to determine the […]
Mutations in VWA8 cause autosomal-dominant retinitis pigmentosa via aberrant mitophagy activation
J Med Genet. 2023 Apr 3:jmg-2022-108888. doi: 10.1136/jmg-2022-108888. Online ahead of print. ABSTRACT BACKGROUND: Although retinitis pigmentosa (RP) is the most common type of hereditary retinal dystrophy, approximately 25%-45% of cases remain without a molecular diagnosis. von Willebrand factor A domain containing 8 (VWA8) encodes a mitochondrial matrix-targeted protein; its molecular function and pathogenic mechanism […]
Pathologically high intraocular pressure induces mitochondrial dysfunction through Drp1 and leads to retinal ganglion cell PANoptosis in glaucoma
Redox Biol. 2023 Mar 21;62:102687. doi: 10.1016/j.redox.2023.102687. Online ahead of print. ABSTRACT Glaucoma is a common neurodegenerative disease characterized by progressive retinal ganglion cell (RGC) loss and visual field defects. Pathologically high intraocular pressure (ph-IOP) is an important risk factor for glaucoma, and it triggers molecularly distinct cascades that control RGC death and axonal degeneration. […]
Live Birth of a Healthy Child in a Couple with Identical mtDNA Carrying a Pathogenic c.471_477delTTTAAAAinsG Variant in the MOCS2 Gene
Genes (Basel). 2023 Mar 15;14(3):720. doi: 10.3390/genes14030720. ABSTRACT Molybdenum cofactor deficiency type B (MOCODB; #252160) is an autosomal recessive metabolic disorder that has only been described in 37 affected patients. In this report, we describe the presence of an in-frame homozygous variant (c.471_477delTTTAAAAinsG) in the MOCS2 gene in an affected child, diagnosed with Ohtahara syndrome […]
Reduced OPA1, Mitochondrial Fragmentation and Increased Susceptibility to Apoptosis in Granular Corneal Dystrophy Type 2 Corneal Fibroblasts
Genes (Basel). 2023 Feb 24;14(3):566. doi: 10.3390/genes14030566. ABSTRACT The progressive degeneration of granular corneal dystrophy type 2 (GCD2) corneal fibroblasts is associated with altered mitochondrial function, but the underlying mechanisms are incompletely understood. We investigated whether an imbalance of mitochondrial dynamics contributes to mitochondrial dysfunction of GCD2 corneal fibroblasts. Transmission electron microscopy revealed several small, […]
Stability Determination of Intact Humanin-G with Characterizations of Oxidation and Dimerization Patterns
Biomolecules. 2023 Mar 11;13(3):515. doi: 10.3390/biom13030515. ABSTRACT Humanin is the first identified mitochondrial-derived peptide. Humanin-G (HNG) is a variant of Humanin that has significantly higher cytoprotective properties. Here, we describe the stability features of HNG in different conditions and characterize HNG degradation, oxidation, and dimerization patterns over short-term and long-term periods. HNG solutions were prepared […]
Blockade of ß-Adrenergic Receptors by Nebivolol Enables Tumor Control Potential for Uveal Melanoma in 3D Tumor Spheroids and 2D Cultures
Int J Mol Sci. 2023 Mar 20;24(6):5894. doi: 10.3390/ijms24065894. ABSTRACT Uveal melanoma (UM) is the most common primary cancer of the eye in adults. A new systemic therapy is needed to reduce the high metastasis and mortality rate. As β-blockers are known to have anti-tumor effects on various cancer entities, this study focuses on investigating […]