bims-mideyd Biomed News
on Mitochondrial dysfunction in eye diseases
Issue of 2025–12–07
six papers selected by
Rajalekshmy “Raji” Shyam, University of Iowa



  1. Front Endocrinol (Lausanne). 2025 ;16 1690553
       Objective: Diabetic retinopathy (DR) is a leading cause of vision loss in diabetes, yet its underlying molecular drivers remain poorly defined. This study aimed to identify diabetic stress-responsive targets and explore the therapeutic potential of the reactive sulfur donor PSCP by integrating transcriptomic and functional analyses in diabetic mouse and cell models.
    Methods: Retinal transcriptomic datasets from type 1 and type 2 diabetic mice (GSE111465 and GSE55389) were analyzed for mitochondrial and antioxidant gene expression. In vitro, ARPE-19 retinal pigment epithelial cells were exposed to hydrogen peroxide (H2O2) or methylglyoxal (MGO) to induce oxidative and carbonyl stress. Mitochondrial function, gene expression, and antioxidant pathway activation were assessed in the presence or absence of PSCP or/and Nrf2 inhibitor ML385.
    Results: Transcriptomic analysis revealed consistent dysregulation of mitochondrial antioxidant enzymes IDH2 and MGST1 in diabetic retinas. Oxidative and carbonyl stress in ARPE-19 cells led to reactive oxygen species accumulation, loss of mitochondrial membrane potential, and reduced cell viability, accompanied by suppression of IDH2 and MGST1. PSCP treatment induced Keap1 modification and promoted Nrf2 nuclear translocation, restoring the expression of IDH2, MGST1, and mitochondrial dynamics regulators MFN2 and FIS1. PSCP also preserved mitochondrial membrane potential and improved cell survival. This protective effect was abrogated by ML385.
    Conclusions: Our findings identify IDH2 and MGST1 as stress-responsive mitochondrial targets in DR and demonstrate that PSCP activates the Keap1-Nrf2 pathway to preserve mitochondrial integrity under diabetic stress.
    Keywords:  IDH2; Keap1-Nrf2 pathway; MGST1; diabetic retinopathy; mitochondrial dysfunction; reactive sulfur species
    DOI:  https://doi.org/10.3389/fendo.2025.1690553
  2. Cell Death Dis. 2025 Dec 01. 16(1): 876
      Retinal degeneration comprises a diverse group of progressive disorders leading to visual impairment and ultimately blindness. These include inherited retinal dystrophies (IRDs), diabetic retinopathy (DR), age-related macular degeneration (AMD), and glaucoma, affecting millions worldwide. The underlying pathology involves dysfunction and death of photoreceptor cells and the retinal pigment epithelium (RPE), driven by various stress-induced cell death mechanisms. Although multiple pathways have been reported, the relative contribution of each remains incompletely understood, highlighting the need for further investigation. Therefore, we studied how different stress types that induce retinal degeneration alter the global gene expression profile in vivo (C57BL/6 mice), aiming to identify predominant cell death mechanisms as well as key genes and networks. Retinal toxicity was induced using established models of oxidative stress, hypoxia, endoplasmic reticulum (ER) stress, and chronic inflammation. Transcriptomic profiling revealed both unique and convergent gene expression changes associated with each stressor. In total, 170, 328, 146, and 151 genes were significantly altered under oxidative stress, inflammation, ER stress, and hypoxia, respectively (Log2 fold change >2 or <-2; p < 0.05). Genes such as Arhgap26, Ccdc9, Ube2e2, and Fndc3b were commonly dysregulated across ER stress, inflammation, and oxidative stress, whereas Nfix, Elp6, Naca, and Plcd3 were selectively altered in oxidative stress, inflammation, ER stress, and hypoxia, respectively. Analysis of cell death-related gene subsets revealed that pyroptosis was commonly activated across different stress types. Additionally, autophagy-mediated cell death, ferroptosis, and extrinsic apoptosis were preferentially associated with oxidative stress, chronic inflammation, and hypoxia, respectively. Both ER and oxidative stress models showed strong activation of autophagy-associated cell death. Together, these findings delineate distinct molecular signatures and predominant cell death mechanisms triggered by specific stressors, providing important insights that could aid in developing targeted therapies to prevent or slow retinal degeneration.
    DOI:  https://doi.org/10.1038/s41419-025-08257-w
  3. Invest Ophthalmol Vis Sci. 2025 Dec 01. 66(15): 1
       Purpose: Retinitis pigmentosa (RP) is a hereditary retinal disease. MERTK-associated RP is characterized by earlier onset and rapid progression, featuring retinal pigment epithelium (RPE) inflammation and microglial activation, yet the underlying mechanisms remain incompletely understood. The study aimed to elucidate the intrinsic interactions between RPE inflammation and microglial activation mediated by RPE-derived extracellular vesicles (EVs).
    Methods: Induced pluripotent stem cell (iPSC)-derived RPE models from MERTK mutant patients and healthy controls were established and characterized by transcriptomic analysis. RPE-derived EVs were isolated, and their RNA and protein cargo were systematically profiled using transcriptomic and proteomic data, revealing a potential role in retinal inflammation regulation. The effects of RPE-EVs on microglial activation were confirmed through in vitro co-culture and in vivo animal experiments.
    Results: Transcriptomic analysis revealed that differentially expressed genes between MERTK mut-RPE (M-RPE) and control RPE (C-RPE) were enriched in inflammatory signaling pathways and EV-related terms. Multi-omics data further indicated that the altered RNA and protein cargo of M-RPE-derived EVs were closely associated with inflammation and immune regulation. In vitro co-culture confirmed that M-EVs could rapidly activate microglia and upregulate pro-inflammatory factors. In vivo experiments indicated that microglia phagocytosed M-EVs exhibited more pronounced M1 polarization and migratory changes.
    Conclusions: RPE-derived EVs act as critical drivers of microglial M1 polarization in MERTK-associated RP. Our study revealed their pivotal role in the progression of early-onset severe RP, providing theoretical support for the potential of targeting EVs to modulate the retinal immune microenvironment and intervene in the progression of retinal degeneration.
    DOI:  https://doi.org/10.1167/iovs.66.15.1
  4. Int J Biochem Cell Biol. 2025 Nov 27. pii: S1357-2725(25)00149-9. [Epub ahead of print]190 106881
      Light-induced retinal damage is a significant contributor to age-related macular degeneration (AMD). Qihuang granule (QHG), a traditional Chinese herbal formulation, has been clinically employed in the treatment of retinal diseases, including AMD; however, the precise protective mechanisms remain unclear. This study investigated the protective effects and underlying mechanisms of QHG using a rat model of blue light-induced retinal injury and a human retinal pigment epithelial (ARPE-19) cell model. The results demonstrated that QHG significantly alleviated retinal morphological abnormalities, ultrastructural damage, and apoptosis induced by light exposure. Single-cell RNA sequencing further revealed that specific cell clusters were notably enriched in the PI3K-AKT-mTOR and autophagy-related signaling pathways after QHG treatment, characterized by increased MAP1LC3B (LC3B) expression and decreased SQSTM1 (P62) expression. Validation at the protein and gene levels in vivo confirmed that QHG activated the autophagy pathway by downregulating PI3K, AKT, mTOR, and P62 expression while upregulating LC3B expression. Collectively, this study demonstrates that QHG protects against retinal photodamage by modulating autophagy via the PI3K/AKT/mTOR signaling pathway, providing theoretical support for its clinical application in the treatment of AMD.
    Keywords:  Age-related macular degeneration; Apoptosis; Autophagy; Qihuang granule; Retinal photodamage
    DOI:  https://doi.org/10.1016/j.biocel.2025.106881
  5. Mol Med Rep. 2026 Feb;pii: 59. [Epub ahead of print]33(2):
      Proliferative vitreoretinopathy (PVR), a leading complication of retinal detachment with high recurrence rates and no effective pharmacological treatments, is driven by retinal pigment epithelium (RPE) cells through epithelial‑mesenchymal transition (EMT), a process promoted by methyl‑CpG binding protein 2 (MeCP2). There is bidirectional crosstalk between ferroptosis, an iron‑dependent cell death pathway characterized by lipid peroxidation and EMT, suggesting their interaction may influence PVR pathogenesis. However, the mechanistic involvement of ferroptosis in PVR and its interaction with the MeCP2/EMT axis remain poorly understood. In the present study, a scratch assay demonstrated that MeCP2 enhanced ARPE‑19 cell migration, which was markedly suppressed by erastin. Cell Counting Kit‑8 assays and western blot analysis confirmed that Erastin inhibited cell proliferation without triggering apoptosis. Western blotting and corresponding assay kits both revealed that MeCP2 upregulated glutathione peroxidase 4 (GPX4), glutamate‑cysteine ligase modifier subunit and solute carrier family 7 member 11, increased glutathione levels and decreased malondialdehyde and Fe2+ concentrations, indicating ferroptosis suppression. Erastin reversed EMT by reducing fibronectin (FN) and α‑smooth muscle actin (α‑SMA) expression and restoring E‑cadherin, as shown by western blotting. Further investigation revealed that GPX4 activation exacerbated EMT marker expression (FN, α‑SMA and N‑cadherin), while GPX4 inhibition mitigated these effects, confirming that MeCP2 regulates EMT through GPX4‑dependent ferroptosis. Erastin inhibited MeCP2‑driven ARPE‑19 proliferation, migration and EMT via ferroptosis induction, independent of apoptosis. MeCP2 suppressed ferroptosis through GPX4 upregulation, using this pathway to orchestrate EMT, thus revealing a critical GPX4‑dependent mechanism that links ferroptosis to RPE plasticity in PVR. These findings highlighted ferroptosis modulation as a promising therapeutic strategy for PVR.
    Keywords:  epithelial‑mesenchymal transition; ferroptosis; glutathione peroxidase; methyl‑CpG binding protein 2; proliferative vitreoretinopathy
    DOI:  https://doi.org/10.3892/mmr.2025.13769
  6. Biomed Pharmacother. 2025 Nov 29. pii: S0753-3322(25)01029-7. [Epub ahead of print]193 118835
      Retinal ganglion cells (RGCs) serve as the terminal output neurons in the retina and are responsible for transmitting visual information from photoreceptors to higher-level centers in the brain. Because of their highly polarized structure, substantial energy demands, and complex protein synthesis activities, the function of RGCs is critically dependent on the homeostasis of intracellular organelles, particularly the endoplasmic reticulum (ER) and mitochondria. Recent studies have shown that these two organelles engage in close physical and functional crosstalk through specific microdomains known as "mitochondria-associated ER membranes" (MAMs), which are crucial for the survival and function of RGCs. This review delves into the critical roles of the ER and mitochondria in the mechanisms of RGC degeneration. Furthermore, the mechanisms by which mitochondrial-ER contact site (MERC)-mediated interorganelle communication exacerbates RGC degeneration by disrupting Ca2 + homeostasis and inducing ER stress and oxidative stress are elucidated. Drugs targeting mitochondria, ER, and MERCs to prevent and treat RGC degeneration are summarized to provide new perspectives and references for studying the pathological mechanisms of RGC degeneration and developing targeted therapeutic strategies.
    Keywords:  Endoplasmic reticulum; Mitochondria; Mitochondria-associated membrane; Retinal ganglion cell; Retinal neuron degeneration
    DOI:  https://doi.org/10.1016/j.biopha.2025.118835