Geographic characterization of RPE structure and lipid changes in the PEX1-p.Gly844Asp mouse model for Zellweger spectrum disorder DOI Creative Commons
Samy Omri, Catherine Argyriou, Rachel S. Pryce

и другие.

bioRxiv (Cold Spring Harbor Laboratory), Год журнала: 2024, Номер unknown

Опубликована: Сен. 10, 2024

Abstract Peroxisome Biogenesis Disorders-Zellweger Spectrum (PBD-ZSD) are a heterogenous group of autosomal recessive disorders caused by defects in PEX genes whose proteins required for peroxisome assembly and function. Peroxisomes ubiquitous organelles that play critical role complex lipid metabolism. Dysfunctional peroxisomes ZSD cause multisystem effects, with progressive retinal degeneration (RD) leading to childhood blindness being one the most frequent clinical findings. Despite progress understanding normal cellular functions, much remains unknown about how their deficiency causes RD, there is no treatment. To study RD pathophysiology this disease, we used knock-in PEX1-p.GlyG844Asp (G844D) mouse model milder ZSD, which represents common human PEX1-p.Gly843Asp allele. We previously reported diminished function, functional vision, neural retina structural model. Beyond retina, pigment epithelium (RPE) have been patients murine models single enzyme deficiency, suggesting RPE may contribute overall progression disease. Here, investigate phenotype our PEX1-G844D model, observing morphological, inflammatory, changes at 1, 3, 6 months age. report cell appears 3 age worsens time, starts dorsal pole, accompanied subretinal inflammatory infiltration. match these events remodelling using imaging mass spectrometry allowed regional analysis specific layer. identified 47 alterations precede changes, 10 localized pole. 32 persist months, signature 14 new occur concurrent histological changes. Changes peroxisome-dependent lipids detected liquid chromatography tandem (reduced docosahexanoic acid increased very long chain lysophosphatidylcholines) exacerbated over time. This first characterization any animal situ peroxisome-deficient tissue. Our findings reveal candidate drivers could be targeted alleviate as well biomarkers evaluate retinopathy response therapy.

Язык: Английский

Facile preparation of Fe3O4/CNTs nanocomposites by catalytic chemical vapor deposition for nonenzymatic hydrogen peroxide sensor DOI
Tian Zhang, Sasa Gu, Wenjie Zhang

и другие.

Journal of Alloys and Compounds, Год журнала: 2025, Номер unknown, С. 179075 - 179075

Опубликована: Фев. 1, 2025

Язык: Английский

Процитировано

0
Molecular identification of a thioredoxin peroxidase in Babesia gibsoni with potential against oxidative stress DOI Creative Commons
Jiaying Guo, Fangjie Li,

Lingna Wang

и другие.

Parasitology Research, Год журнала: 2025, Номер 124(3)

Опубликована: Март 1, 2025

Язык: Английский

Процитировано

0
Modelling Peroxisomal Disorders in Zebrafish DOI Creative Commons
C. Jiang, Michael Schrader

Cells, Год журнала: 2025, Номер 14(2), С. 147 - 147

Опубликована: Янв. 20, 2025

Peroxisomes are ubiquitous, dynamic, oxidative organelles with key functions in cellular lipid metabolism and redox homeostasis. They have been linked to healthy ageing, neurodegeneration, cancer, the combat of pathogens viruses, infection immune responses. Their biogenesis relies on several peroxins (encoded by PEX genes), which mediate matrix protein import, membrane assembly, peroxisome multiplication. Defects or peroxisomal enzymes can result severe disorders, including developmental neurological abnormalities. The drive understand role peroxisomes human health disease, as well their tissues organs during development, has led establishment vertebrate models. zebrafish (Danio rerio) become an attractive model organism investigate functions. Here, we provide overview visualisation zebrafish, metabolic inventory comparison peroxisomes. We then present models established disorders. These include for disorders/Zellweger Spectrum single enzyme deficiencies, particularly adrenoleukodystrophy fatty acid beta-oxidation Finally, highlight deficiencies dually targeted peroxisomal/mitochondrial proteins. Advantages investigation development approaches application drug screening discussed.

Язык: Английский

Процитировано

0
Spatial characterization of RPE structure and lipids in the PEX1-p.Gly844Asp mouse model for Zellweger spectrum disorder DOI Creative Commons
Samy Omri, Catherine Argyriou, Rachel S. Pryce

и другие.

Journal of Lipid Research, Год журнала: 2025, Номер unknown, С. 100771 - 100771

Опубликована: Март 1, 2025

Zellweger Spectrum Disorder (ZSD) is caused by defects in PEX genes, whose proteins are required for peroxisome assembly and function. Peroxisome dysfunction ZSD causes multisystem effects, with progressive retinal degeneration (RD) among the most frequent clinical findings. However, much remains unknown about how deficiency RD. To study RD pathophysiology ZSD, we used PEX1-p.Gly844Asp (G844D) mouse model, which represents common human PEX1-p.Gly843Asp allele. We previously reported diminished function, functional vision, neural retina structural this model. Here, investigate pigment epithelium (RPE) phenotype, examining morphological, inflammatory, lipid changes at 1, 3, 6 months of age. report that RPE cells exhibit evident 3 worsens time, starts dorsal pole, accompanied subretinal inflammatory cell infiltration. match these events imaging mass spectrometry regional analysis lipids RPE. identified 47 alterations preceding changes, 9 localize to pole. 29 persist months, remodeling pole signature. 13 new occur concurrent histological changes. Abnormalities peroxisome-dependent detected LC/MS/MS exacerbated over time. This first characterization a situ peroxisome-deficient tissue. Our findings uncover potential drivers progression identify candidate biomarkers retinopathy response therapy.

Язык: Английский

Процитировано

0
A Simple Methoxyquinoline‐Based Ratiometric Fluorescent Probe for Specific Detection of Hydrogen Peroxide in Living Cells and Zebrafish DOI Open Access

Xinyu Cai,

Xinke Li,

Wenzhai Li

и другие.

Luminescence, Год журнала: 2025, Номер 40(3)

Опубликована: Март 1, 2025

A simple methoxyquinoline-based ratiometric fluorescent probe was synthesized for detecting hydrogen peroxide (H2O2). After the reacted with H2O2, 6-methoxyquinoline fluorescence dye released, and intensity increased at 366 nm decreased 450 nm. And ratio of is linearly related to concentration H2O2 in range 0-100 μM; it shows a good signal. In addition, has characteristics such as rapid response speed high sensitivity selectivity. This low biotoxicity excellent cell membrane permeability further used image HeLa cells zebrafish.

Язык: Английский

Процитировано

0
Analytical subcellular fractionation of microglial BV-2 cells with peroxisomal beta-oxidation defect DOI
Mounia Tahri-Joutey,

Isabelle Hamer,

Virginie Tevel

и другие.

Histochemistry and Cell Biology, Год журнала: 2025, Номер 163(1)

Опубликована: Апрель 14, 2025

Язык: Английский

Процитировано

0
ROS-mediated regulation of β2AR function: Does oxidation play a meaningful role towards β2-agonist tachyphylaxis in airway obstructive diseases? DOI

Razan L. Teyani,

F. Aarabi Moghaddam,

Nader H. Moniri

и другие.

Biochemical Pharmacology, Год журнала: 2024, Номер 226, С. 116403 - 116403

Опубликована: Июнь 28, 2024

Язык: Английский

Процитировано

2
SFRRI Inaugural Alberto Boveris Award Lecture Dynamics of Intracellular and Intercellular Redox Communication DOI Creative Commons
Helmut Sies

Free Radical Biology and Medicine, Год журнала: 2024, Номер 225, С. 933 - 939

Опубликована: Ноя. 1, 2024

Язык: Английский

Процитировано

2
Dual Gold Nanostructures-Based Stretchable Electrochemiluminescence Sensors for Hydrogen Peroxide Monitoring in Endothelial Mechanotransduction DOI
Hao Liu, Qian Wang, Shibo Cheng

и другие.

ACS Sensors, Год журнала: 2024, Номер 9(11), С. 6276 - 6283

Опубликована: Ноя. 7, 2024

Hydrogen peroxide (H

Язык: Английский

Процитировано

2
Research progress in mechanism of anticancer action of shikonin targeting reactive oxygen species DOI Creative Commons

Qi Ke,

Jiayi Li, Yang Hu

и другие.

Frontiers in Pharmacology, Год журнала: 2024, Номер 15

Опубликована: Июль 11, 2024

Excessive buildup of highly reactive molecules can occur due to the generation and dysregulation oxygen species (ROS) their associated signaling pathways. ROS have a dual function in cancer development, either leading DNA mutations that promote growth dissemination cells, or triggering death cells. Cancer cells strategically balance fate by modulating levels, activating pro-cancer pathways, suppressing antioxidant defenses. Consequently, targeting has emerged as promising strategy therapy. Shikonin its derivatives, along with related drug carriers, impact several pathways components involved oxidative stress induce processes such apoptosis, necroptosis, cell cycle arrest, autophagy, well modulation ferroptosis. Moreover, they increase responsiveness drug-resistant chemotherapy drugs, based on specific characteristics ROS, kind stage cancer. This research explores anti-cancer impacts summarize mechanisms achievements shikonin-targeted effects provide suggestions for designing further anti-tumor experiments undertaking experimental practical research.

Язык: Английский

Процитировано

1