The Anti-Aging Hormone Klotho Promotes Retinal Pigment Epithelium Cell Viability and Metabolism by Activating the AMPK/PGC-1α Pathway
Antioxidants (Basel). 2023 Feb 5;12(2):385. doi: 10.3390/antiox12020385. ABSTRACT Initially discovered by Makuto Kuro-o in 1997, Klotho is a putative aging-suppressor gene when overexpressed and accelerates aging when deleted in mice. Previously, we showed that α-Klotho regulates retinal pigment epithelium (RPE) functions and protects against oxidative stress. However, the mechanisms by which Klotho influences RPE and […]
TRAP1 Is Expressed in Human Retinal Pigment Epithelial Cells and Is Required to Maintain their Energetic Status
Antioxidants (Basel). 2023 Feb 4;12(2):381. doi: 10.3390/antiox12020381. ABSTRACT Age-related macular degeneration (AMD) is the leading cause of severe vision loss and blindness in elderly people worldwide. The damage to the retinal pigment epithelium (RPE) triggered by oxidative stress plays a central role in the onset and progression of AMD and results from the excessive accumulation […]
Enhanced mitochondrial biogenesis promotes neuroprotection in human pluripotent stem cell derived retinal ganglion cells
Commun Biol. 2023 Feb 24;6(1):218. doi: 10.1038/s42003-023-04576-w. ABSTRACT Mitochondrial dysfunctions are widely afflicted in central nervous system (CNS) disorders with minimal understanding on how to improve mitochondrial homeostasis to promote neuroprotection. Here we have used human stem cell differentiated retinal ganglion cells (hRGCs) of the CNS, which are highly sensitive towards mitochondrial dysfunctions due to […]
Airborne Exposure of the Cornea to PM10 Induces Oxidative Stress and Disrupts Nrf2 Mediated Anti-Oxidant Defenses
Int J Mol Sci. 2023 Feb 15;24(4):3911. doi: 10.3390/ijms24043911. ABSTRACT The purpose of this study is to test the effects of whole-body animal exposure to airborne particulate matter (PM) with an aerodynamic diameter of <10 μm (PM10) in the mouse cornea and in vitro. C57BL/6 mice were exposed to control or 500 µg/m3 PM10 for […]
Quantitative Proteomics of Human Retinal Pigment Epithelium Reveals Key Regulators for the Pathogenesis of Age-Related Macular Degeneration
Int J Mol Sci. 2023 Feb 7;24(4):3252. doi: 10.3390/ijms24043252. ABSTRACT Age-related macular degeneration (AMD) is the leading cause of blindness in elderly people, with limited treatment options available for most patients. AMD involves the death of retinal pigment epithelium (RPE) and photoreceptor cells, with mitochondria dysfunction being a critical early event. In the current study, […]
Harel Yoon syndrome: a novel mutation in ATAD3A gene and expansion of the clinical spectrum
Ophthalmic Genet. 2023 Mar 1:1-8. doi: 10.1080/13816810.2023.2183223. Online ahead of print. ABSTRACT BACKGROUND: Harel-Yoon syndrome (HAYOS) is a recently described neurodevelopmental disorder characterized by psychomotor delay, truncal hypotonia, appendicular spasticity, and peripheral neuropathy. It is caused by mutations in ATAD3A gene located on chromosome 1p.36.33 whose functions include mitochondrial DNA stabilization, the regulation of mitochondrial […]
Rescuing cellular function in Fuchs endothelial corneal dystrophy by healthy exogenous mitochondrial internalization
Sci Rep. 2023 Feb 28;13(1):3380. doi: 10.1038/s41598-023-30383-8. ABSTRACT Fuchs endothelial corneal dystrophy (FECD) is characterized by an accelerated loss of corneal endothelial cells. Since the function of these cells is to maintain the cornea in a state of deturgescence necessary for its transparency, the depletion of corneal endothelial cells ultimately causes corneal edema and irreversible […]
FDX1-dependent and independent mechanisms of elesclomol-mediated intracellular copper delivery
Proc Natl Acad Sci U S A. 2023 Mar 7;120(10):e2216722120. doi: 10.1073/pnas.2216722120. Epub 2023 Feb 27. ABSTRACT Recent studies have uncovered the therapeutic potential of elesclomol (ES), a copper-ionophore, for copper deficiency disorders. However, we currently do not understand the mechanism by which copper brought into cells as ES-Cu(II) is released and delivered to cuproenzymes […]
Short communication: unique metabolic signature of proliferative retinopathy in the tear fluid of diabetic patients with comorbidities – preliminary data for PPPM validation
EPMA J. 2023 Feb 22;14(1):1-9. doi: 10.1007/s13167-023-00318-4. Online ahead of print. ABSTRACT Type 2 diabetes (T2DM) defined as the adult-onset type that is primarily not insulin-dependent, comprises over 95% of all diabetes mellitus (DM) cases. According to global records, 537 million adults aged 20-79 years are affected by DM that means at least 1 out […]
Optimisation of AAV-NDI1 Significantly Enhances Its Therapeutic Value for Correcting Retinal Mitochondrial Dysfunction
Pharmaceutics. 2023 Jan 18;15(2):322. doi: 10.3390/pharmaceutics15020322. ABSTRACT AAV gene therapy for ocular disease has become a reality with the market authorisation of LuxturnaTM for RPE65-linked inherited retinal degenerations and many AAV gene therapies currently undergoing phase III clinical trials. Many ocular disorders have a mitochondrial involvement from primary mitochondrial disorders such as Leber hereditary optic […]