Live Metabolic Profile Analysis of Zebrafish Embryos Using a Seahorse XF 24 Extracellular Flux Analyzer

Bond, Simon T.; McEwen, Kevin; Yoganantharajah, Prusothman; Gibert, Yann

doi:10.1007/978-1-4939-7883-0_21

Cited by 16 publications

(8 citation statements)

References 10 publications

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“…In addition to its role in ER stress, WFS1 is involved in the regulation of the mitochondrial activity in human fibroblasts ( 9 ). Therefore, we took advantage of the capacity of the seahorse XFe24 to measure the OCR in the living zebrafish ( 45 ) and to decipher the impact of the loss of function of wfs1a and wfs1b genes on the different mitochondrial parameters. At 2 dpf, wfs1b W493X mutant larvae showed a decrease in basal, ATP-linked and maximal respiration.…”

Section: Discussionmentioning

confidence: 99%

Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines

Crouzier

Richard

Diez

et al. 2022

Human Molecular Genetics

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Wolfram syndrome is a rare genetic disease characterized by diabetes, optic atrophy and deafness. Patients die at 35 years old, mainly from respiratory failure or dysphagia. Unfortunately, there is no treatment to block the progression of symptoms and an urgent need for adequate research models. Here, we report on the phenotypical characterization of two loss-of-function zebrafish mutant lines: wfs1aC825X and wfs1bW493X. We observed that wfs1a deficiency altered the size of the ear and the retina of the fish. We also documented a decrease in the expression level of unfolded protein response (UPR) genes in basal condition and in stress condition, i.e. after Tunicamycin treatment. Interestingly, both mutants lead to a decrease of their visual function measured behaviorally. These deficits were associated with a decrease in the expression level of UPR genes in basal and stress conditions. Interestingly, basal, ATP-linked and maximal mitochondrial respirations were transiently decreased in the wfs1b mutant. Taken together, these zebrafish lines highlight the critical role of wfs1a and wfs1b in UPR, mitochondrial function and visual physiology. These models will be useful tools to better understand the cellular function of Wfs1 and to develop novel therapeutic approaches for Wolfram syndrome.

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Section: Discussionmentioning

confidence: 99%

Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines

Crouzier

Richard

Diez

et al. 2022

Human Molecular Genetics

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“…However, the basal OCR in idebenone treatment groups tended to be higher than that in MO- tcap zebrafish ( Figures 6A, B ). Oligomycin OCR is used to determine ATP-linked respiration ( Bond et al, 2018 ). A significant increase in the ATP-linked respiration was observed in idebenone treatment zebrafish groups ( Figures 6A, C ).…”

Section: Resultsmentioning

confidence: 99%

Tcap Deficiency in Zebrafish Leads to ROS Production and Mitophagy, and Idebenone Improves its Phenotypes

Zhang

et al. 2022

Front. Cell Dev. Biol.

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Limb-girdle muscular dystrophy 2G (LGMD2G) is a subtype of limb-girdle muscular dystrophy. However, the disease’s mechanisms are still not fully understood, and no established therapeutic targets have been found. Using a morpholino-based knockdown approach, we established an LGMD2G zebrafish model. In this study, we found that the ROS level increased in LGMD2G zebrafish. The expression of the mitophagy-related protein BNIP3L, LC3A-II/LC3A-I, and LAMP1 were increased in LGMD2G zebrafish. The oxygen consumption rate and citrate synthase expression was significantly decreased. Thus, mitophagy was presumed to be involved in the LGMD2G to reduce ROS levels. Then, we administered vitamin C, coenzyme Q10, idebenone, metformin, or dexamethasone to rescue LGMD2G in zebrafish. Idebenone reduced the curly tail phenotype and ROS level. Also, it reduced BNIP3L expression in LGMD2G zebrafish models and improved their motor function. In conclusion, mitophagy might be involved in the LGMD2G, and idebenone ameliorated LGMD2G by downregulating ROS level.

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“…The islet plate acquisition screens were placed on the measurement area to hold the larvae in place. Four wells were left empty as a control, following the already published protocols on the use of the Seahorse analyser on zebrafish larvae [ 60 , 61 , 62 ]. Each well was filled with 500 µL of egg water (pH 7.4).…”

Section: Methodsmentioning

confidence: 99%

β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy

et al. 2021

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Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular childhood disorder that causes progressive muscle weakness and degeneration. A lack of dystrophin in DMD leads to inflammatory response, autophagic dysregulation, and oxidative stress in skeletal muscle fibers that play a key role in the progression of the pathology. β-glucans can modulate immune function by modifying the phagocytic activity of immunocompetent cells, notably macrophages. Mitochondrial function is also involved in an important mechanism of the innate and adaptive immune responses, owing to high need for energy of immune cells. In the present study, the effects of 1,3-1,6 β-glucans on five-day-old non-dystrophic and dystrophic (sapje) zebrafish larvae were investigated. The effects of the sonication of β-glucans and the dechorionation of embryos were also evaluated. The results showed that the incidence of dystrophic phenotypes was reduced when dystrophic embryos were exposed to 2 and 4 mg L−1 of 1,3-1,6 β-glucans. Moreover, when the dystrophic larvae underwent 8 mg L−1 treatment, an improvement of the locomotor performances and mitochondrial respiration were observed. In conclusion, the observed results demonstrated that 1,3-1,6 β-glucans improve locomotor performances and mitochondrial function in dystrophic zebrafish. Therefore, for ameliorating their life quality, 1,3-1,6 β-glucans look like a promising diet supplement for DMD patients, even though further investigations are required.

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Live Metabolic Profile Analysis of Zebrafish Embryos Using a Seahorse XF 24 Extracellular Flux Analyzer

Cited by 16 publications

References 10 publications

Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines

Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines

Tcap Deficiency in Zebrafish Leads to ROS Production and Mitophagy, and Idebenone Improves its Phenotypes

β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy

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