Consensus Recommendations for the Clinical Management of Wolfram syndrome Using a Delphi Method
medRxiv [Preprint]. 2026 Jul 2:2026.07.02.26357130. doi: 10.64898/2026.07.02.26357130. ABSTRACT BACKGROUND: Wolfram syndrome is a rare neurodegenerative disorder, most commonly caused by pathogenic variants in WFS1, while cases due to CISD2 are exceedingly rare. The estimated prevalence is 1 in 160,000 to 770,000 individuals worldwide. In these clinical guidelines, disorders caused by WFS1 are referred to as […]
Quercetin is associated with photoreceptor protection in retinal degeneration
Sci Rep. 2026 Jul 10. doi: 10.1038/s41598-026-61773-3. Online ahead of print. ABSTRACT Retinal degeneration (RD) is a group of retinopathies characterized by progressive photoreceptor death and chronic neuroinflammation. Quercetin (QUE) is a natural flavonol with potent anti-inflammatory and free-radical scavenging properties. However, its protective effects against RD remain poorly characterized. This study aims to investigate […]
Late-Onset Leber Hereditary Optic Neuropathy: A Report of a Case and Review of the Literature
Cureus. 2026 Jun 9;18(6):e110551. doi: 10.7759/cureus.110551. eCollection 2026 Jun. ABSTRACT Leber hereditary optic neuropathy (LHON) constitutes a mitochondrial disorder characterized by subacute, bilateral central vision impairment, secondary to mitochondrial DNA (mtDNA) mutations. These mutations compromise Complex I, subsequently precipitating the degeneration of retinal ganglion cells (RGCs). While traditionally manifesting in young males, contemporary literature has […]
Characterization of Rcbtb1 Knockout Mice and Evaluation of AAV2-RCBTB1 Gene Replacement Therapy
Transl Vis Sci Technol. 2026 Jul 1;15(7):12. doi: 10.1167/tvst.15.7.12. ABSTRACT PURPOSE: Biallelic pathogenic variants in the RCC1 and BTB domain-containing protein 1 (RCBTB1) gene cause an adult-onset retinal dystrophy. Here, we generated a knockout mouse model of RCBTB1 deficiency for the evaluation of RCBTB1 gene therapy. METHODS: Rcbtb1-knockout (KO) mice were generated with a homozygous […]
MAM-Localized MANF Counteracts Microinflammatory Stress to Attenuate Mitochondrial Dysfunction and Cataractogenesis in High Myopia
Adv Sci (Weinh). 2026 Jul 8:e76342. doi: 10.1002/advs.76342. Online ahead of print. ABSTRACT Chronic microinflammation drives tissue degeneration, particularly in age-related and metabolic diseases, yet how it disrupts inter-organelle communication that leads to cellular failure remains largely unexplored. Utilizing highly myopic cataract (HMC) as a paradigm, we uncover a non-canonical defense mechanism centered on mitochondria-associated […]
Exogenous mitochondrial transplantation attenuates oxidative stress-driven retinal degeneration in a sodium iodate – induced mouse model
Neurobiol Aging. 2026 Jul 6;168:1-13. doi: 10.1016/j.neurobiolaging.2026.07.002. Online ahead of print. ABSTRACT Age-related macular degeneration (AMD) is a degenerative retinal disease initiated by dysfunction of the retinal pigment epithelium (RPE), in which age-related mitochondrial impairment, oxidative stress, chronic inflammation, and complement activation collectively drive outer retinal dysfunction and RPE atrophy, ultimately leading to progressive central […]
Ocular IL-1alpha/IFN-gamma Delay the Corneal Wound Healing in Rheumatoid Arthritis Through Mitochondrial Dysfunction and NLRP3 Inflammasome Activation
Invest Ophthalmol Vis Sci. 2026 Jul 1;67(8):27. doi: 10.1167/iovs.67.8.27. ABSTRACT PURPOSE: Rheumatoid arthritis (RA) is an autoimmune disease often associated with corneal ulceration and impaired corneal epithelial wound healing (CEWH), although the underlying cause remains unclear. This study aims to elucidate the pathogenic mechanism of RA-associated delayed CEWH. METHODS: We integrated analyses of human clinical […]
The first complete mitochondrial genome sequences of Neopsylla specialis dechingensis and Neopsylla stevensi sichuanyunnana, with an assessment of their phylogenetic placement
Front Vet Sci. 2026 Jun 23;13:1865746. doi: 10.3389/fvets.2026.1865746. eCollection 2026. ABSTRACT Fleas are common blood-sucking external parasites found on mammals; they serve as important vectors for various pathogenic microorganisms and pose a serious threat to human and animal health. Mitochondrial genomes have been widely used in studies such as DNA barcoding for species identification and […]
Attenuating Ischemia and Reperfusion Injury Using NAD+-Loaded Nanoparticles in Mouse Kidneys
Transplant Direct. 2025 Dec 12;12(1):e1890. doi: 10.1097/TXD.0000000000001890. eCollection 2026 Jan. ABSTRACT BACKGROUND: Ischemia and reperfusion (IR) injury is a major complication in solid organ transplantation, necessitating new therapeutic strategies to suppress IR injury and thereby reduce early allograft dysfunction and delayed graft function. In kidney transplantation, IR injury is associated with disruption of mitochondrial homeostasis, […]
TRPM7-mediated calcium signaling contributes to Hyperglycemia-induced mitochondrial dysfunction and apoptosis in retinal Muller cells
Cell Signal. 2026 Jul 7:112728. doi: 10.1016/j.cellsig.2026.112728. Online ahead of print. ABSTRACT Calcium signaling dysregulation is a critical trigger of mitochondrial dysfunction in metabolic disorders, yet the upstream mechanisms linking hyperglycemic stress to organellar Ca2+ overload remain poorly defined. The transient receptor potential melastatin 7 (TRPM7) channel functions as a Ca2+-permeable signaling node with unique […]