Decrease in DHA and other fatty acids correlates with photoreceptor degeneration in retinitis pigmentosa

Ruiz-Pastor, María José; Kutsyr, Oksana; Lax, Pedro; Cuenca, Nicolás

doi:10.1016/j.exer.2021.108667

Cited by 13 publications

(7 citation statements)

References 91 publications

(128 reference statements)

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“…In particular, in vitro studies suggested their role in increased cellular levels of GSH as well as enzymatic antioxidants such as SOD and GPXs [ 82 , 83 ]. Among them, DHA is a long chain ω-3 PUFA particularly enriched in some fish species (i.e., salmon, mackerel, and anchovies), which is essential for retinal photoreceptor integrity and for the photo-transduction cascade [ 84 ]. It also counteracts lipid peroxidation that may occur at the cellular membranes [ 85 ].…”

Section: Antioxidants In Pharmaceuticalsmentioning

confidence: 99%

Oxidative Stress and Antioxidant-Based Interventional Medicine in Ophthalmology

Honisch

Rodella

Gatto³

et al. 2023

Pharmaceuticals

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The different anatomical compartments of the eye are highly subjected to reactive oxygen species (ROS) generation due to internal factors, such as metabolic high oxygen consumption, as well as environmental factors, including UV light. An antioxidant defense system is endowed in the eye tissues to regulate ROS quantity and activity. When this homeostatic system is overwhelmed, oxidative stress occurs, causing cellular damage, chronic inflammation, and tissue degeneration. It also plays a significant role in the development and progression of various ocular diseases. Understanding the mechanisms underlying oxidative stress in ocular conditions is thus crucial for the development of effective prevention and treatment strategies. To track marketed products based on antioxidant substances as active ingredients, the databases of the European Medicines Agency and the U.S. Food and Drug Administration were consulted. Only a limited number of items were identified, which were either used as therapeutic treatment or during ocular surgery, including antioxidants, synthetical derivatives, or pro-drugs designed to enhance tissue permeation and activity. This review aims to provide an overview of the primary ocular pathologies associated with oxidative stress and of the available pharmacological interventions centered around antioxidant molecules. Such insights are essential for advancing the development of effective prevention and novel treatment approaches.

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Section: Antioxidants In Pharmaceuticalsmentioning

confidence: 99%

Oxidative Stress and Antioxidant-Based Interventional Medicine in Ophthalmology

Honisch

Rodella

Gatto³

et al. 2023

Pharmaceuticals

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“…Recent work from our laboratory highlighted the relevant changes in fatty acids, especially docosahexaenoic acid (DHA), in rd10 mice during the degenerative process ( Ruiz-Pastor et al, 2021 ). Fatty acids are an essential part of photoreceptor membranes, and of their cell integrity and are involved in the phototransduction cascade and change to their levels during retinal degeneration.…”

Section: Cellular Responses In the Inherited Retinal Dystrophies In R...mentioning

confidence: 99%

Cellular and molecular alterations in neurons and glial cells in inherited retinal degeneration

et al. 2022

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Multiple gene mutations have been associated with inherited retinal dystrophies (IRDs). Despite the spectrum of phenotypes caused by the distinct mutations, IRDs display common physiopathology features. Cell death is accompanied by inflammation and oxidative stress. The vertebrate retina has several attributes that make this tissue vulnerable to oxidative and nitrosative imbalance. The high energy demands and active metabolism in retinal cells, as well as their continuous exposure to high oxygen levels and light-induced stress, reveal the importance of tightly regulated homeostatic processes to maintain retinal function, which are compromised in pathological conditions. In addition, the subsequent microglial activation and gliosis, which triggers the secretion of pro-inflammatory cytokines, chemokines, trophic factors, and other molecules, further worsen the degenerative process. As the disease evolves, retinal cells change their morphology and function. In disease stages where photoreceptors are lost, the remaining neurons of the retina to preserve their function seek out for new synaptic partners, which leads to a cascade of morphological alterations in retinal cells that results in a complete remodeling of the tissue. In this review, we describe important molecular and morphological changes in retinal cells that occur in response to oxidative stress and the inflammatory processes underlying IRDs.

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“…20 Other observations in animal models include decreased fatty acids in the retina which are correlated with photoreceptor degeneration. 21 Also, the metabolism of amino acids, carbohydrates, nucleotides, and lipids has been proposed to play an important role in retinitis pigmentosa, the most common form of retinal degeneration. [22][23][24][25][26] Oral supplementation to correct these metabolic changes proved to have a neuroprotective effect on the photoreceptor cells in mouse models of retinal degeneration.…”

Section: Introductionmentioning

confidence: 99%

The Gut Metabolism is altered in patients withCRB1-associated inherited retinal degeneration

Moekotte,

de Boer,

Hiddingh

et al. 2024

Preprint

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PurposeTo compare the plasma metabolic profile of patients with aCRB1-associated inherited retinal degeneration (CRB1-IRD) with healthy controls (HCs).DesignA case-control study.MethodsPlasma concentration of 619 metabolites was measured with the MxP®Quant 500 Kit in 30 patients with aCRB1-IRD and 29 HCs. We fitted a linear regression model with adjustments for age and sex based on the concentration of metabolites in µM (µmol/L), or on the sums and ratios of metabolites, to determine differences between patients and controls.ResultsOver-representation of pathways among metabolites associated strongest toCRB1-IRDs (P< 0.05, n = 62) identified amino acid pathways (such as beta-alanine, histidine, and glycine/serine) and bile acid biosynthesis, driven by a decrease in deoxycholic acid derivatives produced by gut microbiota. Enrichment analysis of metabolic classes across the plasma metabolic profile further identified significant positive enrichment for lipid metabolites glycerophospholipids, cholesterol esters, and ceramides, and significant depletion for bile acid metabolites. Further investigation of the sums and ratios (i.e., metabolism indicators) ascertained a significant decrease in intestinal microbial-dependent secondary bile acid classes.ConclusionsLipid metabolic alterations and decreased microbiota-related secondary bile acid concentrations indicate significant alterations in gut metabolism in patients with aCRB1-IRD.

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Decrease in DHA and other fatty acids correlates with photoreceptor degeneration in retinitis pigmentosa

Cited by 13 publications

References 91 publications

Oxidative Stress and Antioxidant-Based Interventional Medicine in Ophthalmology

Oxidative Stress and Antioxidant-Based Interventional Medicine in Ophthalmology

Cellular and molecular alterations in neurons and glial cells in inherited retinal degeneration

The Gut Metabolism is altered in patients withCRB1-associated inherited retinal degeneration

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