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Atm deficient zebrafish model reveals conservation of the tumour suppressor function and a role in fertility
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作者 Jeroen Vierstraete Charlotte Fieuws +4 位作者 David Creytens Jo Van Dorpe Andy Willaert Anne Vral Kathleen BM Claes 《Genes & Diseases》 SCIE CSCD 2023年第2期381-384,共4页
Biallelic loss-of-function variants in ATM (Ataxia Telangiectasia Mutated) cause Ataxia Telangiectasia (AT), a rare disorder associated with cerebellar degeneration and ataxia, cancer predisposition, infertility, grow... Biallelic loss-of-function variants in ATM (Ataxia Telangiectasia Mutated) cause Ataxia Telangiectasia (AT), a rare disorder associated with cerebellar degeneration and ataxia, cancer predisposition, infertility, growth retardation, etc. ATM is a phosphoinositide 3-kinase-related kinase (PIKK) with a role in DNA repair and maintenance of genome stability. Studying a multisystem genetic disease like AT requires animal models to ascertain its pathogenesis at the level of tissues, organs and the organism. Due to its small size, cheap maintenance, large progeny, rapid development and initial transparency, zebrafish (Danio rerio) is an increasingly popular vertebrate model organism, suitable for genetic modifications and large-scale in vivo therapeutic screens as embryos are chemically permeable to small compounds. Currently, no zebrafish model for AT exists.1 We generated atm knock-outs through CRIPSR-Cas9 mutagenesis. We show that atm conserved its function as a tumour suppressor gene and is involved in gametogenesis and fertility. Therefore, this mutant is of great value for further studies investigating the role of atm in reproduction and tumorigenesis. 展开更多
关键词 ATM FUNCTION TRANSPARENCY
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Short-term nitisinone discontinuation of hereditary tyrosinemia type 1 mice causes metabolic alterations in glutathione metabolism/biosynthesis and multiple amino acid degradation pathways
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作者 Colemonts-Vroninks Haaike Norman P.Brendan +11 位作者 Van Laere Sven Davison S.Andrew Marcélis Lionel Casimir Georges Goyens Philippe Claes Paul De Bundel Dimitri Martens Geert Ranganath Lakshminarayan Rao Vanhaecke Tamara Gallagher James A De Kock Joery 《Genes & Diseases》 SCIE CSCD 2023年第5期1759-1762,共4页
Hereditary tyrosinemia type 1(HT1)is a life-threatening disease caused by the patient's inability to break down tyrosine due to loss-of-function mutations in the fumarylacetoacetate hydrolase(FAH)enzyme(Fig.S1).Cu... Hereditary tyrosinemia type 1(HT1)is a life-threatening disease caused by the patient's inability to break down tyrosine due to loss-of-function mutations in the fumarylacetoacetate hydrolase(FAH)enzyme(Fig.S1).Currently,the only available life-saving treatment is nitisinone(NTBC).However,nitisinone therapy comes with debilitating side effects and requires a strict drug regime combined with a tyrosine-and phenylalanine-restricted diet.Consequently,therapy adherence is often experienced as an additional burden.In this study,transcriptional profiling was conducted parallel to high-resolution metabolomics on,respectively,liver tissue and serum samples of Fah-deficient mice.The experimental workflow of our study is shown in Figure 1A.Canonical pathway analyses showed manifest activation of the NRF2-stress response,associated with hepatocellular damage and mainly driven by a markedly increased oxidative burden.Other altered canonical pathways were primarily a downstream consequence of the latter.Furthermore,we observed an overall aminoacidemia and changes related to aminoacyl-tRNA biosynthesis,which postulates that short-term nitisinone discontinuation can already put the patient at risk for hepatocellular carcinoma development.Finally,we did not only identify changes in numerous pathways,directly and indirectly,related to the metabolism of tyrosine and glutathione but also the presence of“side-chain”metabolites,such as y-glutamyl-and N-acetyl-coupled amino acids,indicating that non-directly related pathways like phase II biotransformation reactions are also affected. 展开更多
关键词 CONTINUATION Figure HEREDITARY
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Binding of Orysata lectin induces an immune response in insect cells
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作者 Pengyu Chen Kristof De Schutter +3 位作者 Jarne Pauwels Kris Gevaert Els J.M.Van Damme Guy Smagghe 《Insect Science》 SCIE CAS CSCD 2022年第3期717-729,共13页
In mammals,plant lectinshave been shown to possess immunomodulatory properties,acting in both the innate and adaptive immune system to modulate the production of mediators of the immune response,ultimately improving h... In mammals,plant lectinshave been shown to possess immunomodulatory properties,acting in both the innate and adaptive immune system to modulate the production of mediators of the immune response,ultimately improving host defences.At present,knowledge of immunomodulatory effects of plant lectins in insects is scarce.Treatment of insect cells with the Orysa sativa lectin,Orysata,was previously reported to induce cell aggregation,mimicking the immune process of encapsulation.In this project we investigated the potential immunomodulatory effects of this mannose-binding lectin using Drosophila melanogaster S2 cells.Identification of the Orysata binding partners on the surface of S2 cells through a pull-down assay andproteomic analysis revealed 221 putative interactors,several of which were immunity-related proteins.Subsequent qPCR analysis revealed the upregulation of Toll-and immune deficiency(IMD)-regulated antimicrobial peptides(Drs,Mtk,AttA,and Dpt)and signal transducers(Rel and Hid)belonging to the IMD pathway.In addition,the iron-binding protein Transferrin 3 was identified as a putative interactor for Orysata,and treatment of S2 cells with Orysata was shown to reduce the intracellular iron concentration.All together,we believe these results offer a new perspective on the effects by which plant lectins influence insect cells and contribute to the study of their immunomodulatory properties. 展开更多
关键词 cell culture immune response iron uptake Orysata plant lectin pull-down
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Long-term live-cell microscopy with labeled nanobodies delivered by laser-induced photoporation 被引量:1
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作者 Jing Liu Tim Hebbrecht +10 位作者 Toon Brans Eef Parthoens Saskia Lippens Chengnan Li Herlinde De Keersmaecker Winnok H.De Vos Stefaan C.De Smedt Rabah Boukherroub Jan Gettemans Ranhua Xiong Kevin Braeckmans 《Nano Research》 SCIE EI CAS CSCD 2020年第2期485-495,共11页
Fluorescence microscopy is the method of choice for studying intracellular dynamics.However,its success depends on the.availability of specific and stable markers.A prominent example of markers that are rapidly gainin... Fluorescence microscopy is the method of choice for studying intracellular dynamics.However,its success depends on the.availability of specific and stable markers.A prominent example of markers that are rapidly gaining interest are nanobodies(Nbs.-15 kDa),which can be functionalized with bright and photostable organic fluorophores.Due to their relatively small size and high specificity,Nbs offer great potential for high-quality long-term subcellular imaging,but suffer from the fact that they cannot spontaneously cross the plasma membrane of live cells.We have recently discovered that laser-induced photoporation is well suited to deliver extrinsic labels to living cells without compromising their viability.Being a laser-based technology,it is readily compatible with light microscopy and the typical cell recipients used for that.Spurred by these promising initial results,we demonstrate here for the first time successful long-term imaging of specific subcellular structures with labeled nanobodies in living cells.We illustrate this using Nbs that target GFP/YFP-protein constructs accessible in the cytoplasm,actin-bundling protein Fascin,and the histone H2A/H2B heterodimers.With an efficiency of more than 80%labeled cells and minimal toxicity(-2%),photoporation proved to be an excellent intracellular delivery method for Nbs.Time-lapse microscopy revealed that cell division rate and migration remained unaffected,confirming excellent cell viability and functionality.We conclude that laser-induced photoporation labeled Nbs can be easily delivered into living cells,laying the foundation for further development of a broad range of Nbs with intracellular targets as a toolbox for long-term live-cell microscopy. 展开更多
关键词 laser-induced photoporation vapor nanobubble long-term microscopy imaging NANOBODY intracellular delivery living cell labeling
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A Phelipanche ramosa KAI2 protein perceives strigolactones and isothiocyanates enzymatically
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作者 Alexandre de Saint Germain Anse Jacobs +13 位作者 Guillaume Brun Jean-Bernard Pouvreau Lukas Braem David Cornu Guillaume Clavé Emmanuelle Baudu Vincent Steinmetz Vincent Servajean Susann Wicke Kris Gevaert Philippe Simier Sofie Goormachtig Philippe Delavault François-Didier Boyer 《Plant Communications》 2021年第5期132-147,共16页
Phelipanche ramosa is an obligate root-parasitic weed that threatens major crops in central Europe.In order to germinate,it must perceive various structurally divergent host-exuded signals,including isothiocyanates(IT... Phelipanche ramosa is an obligate root-parasitic weed that threatens major crops in central Europe.In order to germinate,it must perceive various structurally divergent host-exuded signals,including isothiocyanates(ITCs)and strigolactones(SLs).However,the receptors involved are still uncharacterized.Here,we identify five putative SL receptors in P.ramosa and show that PrKAI2d3 is involved in the stimulation of seed germination.We demonstrate the high plasticity of PrKAI2d3,which allows it to interact with different chemicals,including ITCs.The SL perception mechanism of PrKAI2d3 is similar to that of endogenous SLs in non-parasitic plants.We provide evidence that PrKAI2d3 enzymatic activity confers hypersensitivity to SLs.Additionally,we demonstrate that methylbutenolide-OH binds PrKAI2d3 and stimulates P.ramosa germination with bioactivity comparable to that of ITCs.This study demonstrates that P.ramosa has extended its signal perception system during evolution,a fact that should be considered for the development of specific and efficient biocontrol methods. 展开更多
关键词 a/b-hydrolase receptor seed germination stimulant STRIGOLACTONES ISOTHIOCYANATES Phelipanche ramosa
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