What we can learn from immersing mice, fruit flies, and zebrafish in VR. Am J Hum Genet. XEV, MBHB and VN performed mice studies. Intracellular Insulin and Impaired Autophagy in a Zebrafish model and a Cell Model of Type 2 diabetes . PubMed Google Scholar. First, genetic evidence indicates that defects in MLC1 or CLCN2 lead to different diseases [34]. Brain. Neuron. Megalencephalic leukoencephalopathy with subcortical cysts. However, the use of an inappropriate animal model that is not similar to human pathology or disease progression can slow the development of new therapeutics. Studying early innate immune responses could lead to improved understanding of immune mechanisms against viral pathogens,as well as lead to the development of improved therapeutics. Region of interest (ROI) selected for T2 relaxation time measurements are shown in left images. Scale bars: 50 μm (c, d); 60 μm (e, f). Defects in Mlc1 protein expression and localization were rescued by expression of human GlialCAM using adenoviruses that expressed the protein (Fig. Thus, drug effect studies in zebrafish can achieve high-throughput at steady-state, avoiding pharmacodynamic variation due to complex drug pharmacokinetics following intraperitoneal or intravenous drug injections in mice. 6d). 2014;35:1175–8. Thus, we proposed that defects in ClC-2 might contribute partially to the MLC phenotype, but it is not the only reason to explain the phenotype of MLC patients. J Neurosci. California Privacy Statement, c, d mlc1 expression (arrowheads) observed in the optic tract of both wild type (c) and glialcama−/− (d) brains. No major differences were found in the vacuolization phenotype between both mice models [ 15 ]. However, since exposures to radio frequency radiations (RFR) >2.4 GHz are still uncommon, concerns about their potential health impacts are ongoing. The timing of the adoption of zebrafish as an emerging model organism could not be better, as mouse studies often fail to translate to humans. In order to compare the size of the telencephalon relative to the whole brain of the different groups one-way analysis of variance (ANOVA) was performed and indicated that the size was larger in all mutants as compared to wild type (*p < 0.05; **p < 0.005) (n = 3). Ridder MC, Boor I, Lodder JC, Postma NL, Capdevila-Nortes X, Duarri A, et al. Surprisingly, this localization defect could only be observed in primary cultured astrocytes from mouse after incubation with a depolarizing solution [29, 30]. Previous [13] and recent [11] reports indicate that the phenotype of patients with mutations in MLC1 is the same to those with recessive mutations in GLIALCAM. Megalencephalic leucoencephalopathy with cysts: defect in chloride currents and cell volume regulation. Kinetic profile of mucosal pro-inflammatory cytokine responses after zebrafish gill, mouse, and human nasal stimulation with R848. Neurobiol Dis. 2016;25:1543–58. Sugio S, Tohyama K, Oku S, Fujiyoshi K, Yoshimura T, Hikishima K, et al. In mouse primary astrocytes, lack of GlialCAM (Fig. This effort showed that their genome is remarkably similar to the human one, with at least 70% of human genes having a zebrafish equivalent – a figure that is even higher when only disease-causing genes are considered. This work has provided new insights into the molecular interplay that exists between GlialCAM and MLC1, confirming that both proteins form a functional unit that is physiologically relevant. J Neurosci. 2012;73:951–61. In contrast, the levels of mlc1 messenger RNA in the brain were increased in glialcama−/− zebrafish. Due to their small size and the relatively simple nature of … Neuroscientists interested in vertebrate development and diseases have recently turned to zebrafish … Lancet Neurol. Zebrafish genome contains numerous duplicate genes (Milan DJ et al, 2003) and this feature often hinders the generation of knockout strains, as approaches that disrupt one gene copy likely will not disrupt the second copy. 2019;20:1034. No statistical differences were observed in the percent area of telencephalon versus whole brain size between single knockout zebrafish for one gene with the single knockout/heterozygous or the double knockout (p > 0.05) (see Additional file 3: Table S1). Lopez-Hernandez T, Sirisi S, Capdevila-Nortes X, Montolio M, Fernandez-Duenas V, Scheper GC, et al. B Cell Activation and Plasma Cell Differentiation, 4. Unitat de Fisiologia, Departament de Ciències Fisiològiques, Genes Disease and Therapy Program IDIBELL-Institute of Neurosciences, Universitat de Barcelona, L’Hospitalet de Llobregat, Barcelona, Spain, Carla Pérez-Rius, Xabier Elorza-Vidal, Alejandro Barrallo-Gimeno & Raúl Estévez, Department of Biology, Faculty of Sciences, University of A Coruña, 15008-A, Coruña, Spain, Centro de Investigaciones Cientificas Avanzadas (CICA), University of A Coruña, 15008-A, Coruña, Spain, Leiden Institute of Chemistry, Leiden University, Leiden, The Netherlands, Institute of Medical Physics and Biophysics, University of Leipzig, Leipzig, Germany, Leibniz-Forschungsinstitut für Molekulare Pharmakologie (FMP), Department Physiology and Pathology of Ion Transport, D-13125, Berlin, Germany, Max-Delbruck-Centrum für Molekulare Medizin (MDC), D-13125, Berlin, Germany, Centro de Investigación en red de enfermedades raras (CIBERER), ISCIII, Madrid, Spain, Virginia Nunes, Alejandro Barrallo-Gimeno & Raúl Estévez, Unitat de Genètica, Departament de Ciències Fisiològiques, Genes Disease and Therapy Program IDIBELL, Universitat de Barcelona, L’Hospitalet de Llobregat, Barcelona, Spain, Facultat de Medicina, Departament de Ciències Fisiològiques, Universitat de Barcelona-IDIBELL, C/Feixa Llarga s/n 08907 L’Hospitalet de Llobregat, Barcelona, Spain, You can also search for this author in Antibody-mediated autoimmune diseases, Cancer Stem Cells and Tumour-initiating Cells, Discovery of the Thymus as a central immunological organ, 8. These are non-human animal species which can be used to understand specific biological processes and to obtain informations which can provide an insight into working of other organisms. We observed similar lesions in glialcama−/− zebrafish to what has been previously observed for the mlc1−/− zebrafish [29]. Functional analyses of mutations in HEPACAM causing megalencephalic leukoencephalopathy. However, challenge in mice significantly induced expression of TNF-α, IL-6 and IFN-γ. 2013;12:659–68. Mutant GlialCAM causes megalencephalic leukoencephalopathy with subcortical cysts, benign familial macrocephaly, and macrocephaly with retardation and autism. The authors declare that they have no competing interests. 4a). We then studied mlc1 localization in the brain and retina of wild type and glialcama−/− fish by immunofluorescence. 4. They are also very cheap; however, they do require more space than other model organisms such as flies. The fact that the MLC1/GlialCAM complex is a functional unit is evident in the zebrafish knockout for glialcama, in which mlc1 protein is neither reduced nor mislocalized but yet it displays an MLC-like phenotype. **P < 0.01 (vs. wild-type, two-way ANOVA) b Comparison of mlc1 protein levels in brain of wild-type (WT), mlc1−/−, glialcama−/− and mlc1glialcama−/− zebrafish by Western blots of extracts from 5-months-old zebrafish. 2018;61:50–60. Brain white matter oedema due to ClC-2 chloride channel deficiency: an observational analytical study. However, as other in vivo models, zebrafish present certain limitations in regard to modeling NDDs. 4e-f). Menichella DM, Majdan M, Awatramani R, Goodenough DA, Sirkowski E, Scherer SS, et al. Additionally, similar expression kinetics of IL-1β and IL-6 responses were observed in all 3 models. Declining CD4 count, despite viral suppression? MLC1 trafficking and membrane expression in astrocytes: role of caveolin-1 and phosphorylation. The supernatant (SN) of both purifications is included. To study if this was also the case in mice, we analysed the extent of myelin vacuolization in fibre tracts of the cerebellum in single or double knockout mice for Mlc1 and Glialcam (Fig. In this work, we have obtained and characterized a glialcama knockout in zebrafish. Int J Mol Sci. A-C (Source: Progatzky et al., 2019 JACI). Elorza-Vidal X, Sirisi S, Gaitán-Peñas H, Pérez-Rius C, Alonso-Gardón M, Armand-Ugón M, et al. © 2021 BioMed Central Ltd unless otherwise stated. Animals as model organisms, the silent sentinels, stand watch over the environmental health of the world. Regarding the zebrafish models, in mlc1−/− animals, MRI showed that the telencephalon is larger in comparison to the wild-type, and there are several lesions due to increased fluid in the telencephalon and mesencephalon [ 29 ]. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. ABG is a Serra Hunter fellow. Depolarization causes the formation of a ternary complex between GlialCAM, MLC1 and ClC-2 in astrocytes: implications in megalencephalic leukoencephalopathy. GlialCAM, a protein defective in a Leukodystrophy, serves as a ClC-2 cl - channel auxiliary subunit. Breaking Tolerance: Autoimmunity & Dysregulation, 6. Actin served as a loading control. a The sagittal slices were obtained with an in-plane resolution of 47 μm. While mouse “knockouts” are generated by isolating embryoni… 2011;134:3342–54. Histopathology of brain sections from Mlc1−/− and Glialcam−/− mice revealed the presence of vacuolization mainly in fibre tracts of the cerebellum [15, 16, 21, 33]. ns: not significative. Teijido O, Martinez A, Pusch M, Zorzano A, Soriano E, Del Rio JA, et al. Sirisi S, Elorza-Vidal X, Arnedo T, Armand-Ugón M, Callejo G, Capdevila-Nortes X, et al. Capdevila-Nortes X, López-Hernández T, Apaja PM, López de Heredia M, Sirisi S, Callejo G, et al. Google Scholar. Raúl Estévez. Vaccines for Neurodegenerative Diseases, 1. J Neurosci. The nasal mucosal site is the main entry point for respiratory viral pathogens that are responsible for a, b Primary astrocytes isolated from Glialcam−/− mice were incubated for 18 h at 28 °C. 2018;98:1493–590. (c) GlialCAM and Mlc1 protein levels primary astrocytes from wild-type (WT, left), Glialcam−/− (middle) and Glialcam−/− complemented with an adenoviruss expressing human GlialCAM. We do not know which could be the role of glialcamb in zebrafish. A-C (Source: Progatzky et al., 2019 JACI). 1c and [29]) and in the retina, where they are highly expressed at Müller glia end-feet at the inner limiting membrane (Fig. 2001;68:831–8. Journal Article: Progatzky et al., 2019. 2b) MR images of wild-type and various zebrafish mutants were obtained and analysed. In addition, measurements of brain volume revealed that the whole brain is bigger in MLC knockout models than in its wild-type littermates [14, 16, 29]. It is interesting to point out that no changes in Mlc1 messenger RNA levels were observed in Glialcam−/− mice [15]. Therefore, we analysed the brain phenotype of glialcama−/− zebrafish by MRI. Zebrafish take up less space on the shelves than mice. To test that this was not the case for their human orthologous, we repeated the same experiment now with an adenovirus expressing human MLC1. Introduction to Immunization Strategies. Jeworutzki E, López-Hernández T, Capdevila-Nortes X, Sirisi S, Bengtsson L, Montolio M, et al. Lanciotti A, Brignone MS, Visentin S, De Nuccio C, Catacuzzeno L, Mallozzi C, et al. These results indicate that the lack of glialcama causes two typical MLC features: megalencephaly and increased fluid accumulation. RE directed the project and wrote the manuscript, but all the authors contributed in writing and reviewing the manuscript. Scale bar, 400 μm. The nasal mucosal site is the main entry point for respiratory viral pathogens that are responsible for a high burden of global morbidity. 2001;21:5429–38. 4c-d) or in the retina (Fig. Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License. Insights into MLC pathogenesis: GlialCAM is an MLC1 chaperone required for proper activation of volume-regulated anion currents. 2019;20:282–97. Another experiment gave similar results. Jentsch TJ, Pusch M. CLC chloride channels and transporters: structure, function, physiology, and disease. Part of Article  We then reasoned that the zebrafish mlc1 protein might not need glialcama for its stabilization at the plasma membrane, unlike their orthologs in mice and human. Proc Natl Acad Sci. The situation is completely different for the ClC-2 protein. Megalencephalic leukoencephalopathy with subcortical cysts protein 1 regulates glial surface localization of GLIALCAM from fish to humans. Having demonstrated cross-species similarities and limited efficacy of tested AEDs, we propose the use of AG in zebrafish as a convenient and high-throughput model of treatment-resistant seizures. In WT and complemented astrocytes, GlialCAM and Mlc1 are located at cell-cell junctions (arrowheads). 2018;119:88–99. Eur J Med Genet. Tubulin served as a loading control. 2016;17:293–307. a Quantitative real-time PCR to determine levels of glialcama, glialcamb and mlc1 messenger RNA in the brain of glialcama−/− zebrafish. Lopez-Hernandez T, Ridder MC, Montolio M, Capdevila-Nortes X, Polder E, Sirisi S, et al. By Alessandra Potenza @ale_potenza Aug 23, … Megalencephalic leukoencephalopathy with subcortical cysts protein-1 regulates epidermal growth factor receptor signaling in astrocytes. Western blot is representative of three independent experiments. Induction of innate cytokine responses by respiratory mucosal challenge with R848 in zebrafish, mice, and humans. By using this website, you agree to our Lack of signal using GlialCAM antibodies confirmed the lack of protein expression. Cultured astrocytes were identified by their positive GFAP (Glial Fibrillary acid protein) staining (Dako), being > 95% of cells GFAP positive. All experimental procedures were performed in accordance with the European Community Guidelines on Animal Care and Experimentation and were approved by the institutional animal care and use committees. Model organisms are drawn from all three domains of life, as well as viruses.The most widely studied prokaryotic model organism is Escherichia coli (E. coli), which has been intensively investigated for over 60 years.It is a common, gram-negative gut bacterium which can be grown and cultured easily and inexpensively in a laboratory setting. After SDS PAGE, membranes were incubated with primary antibodies: anti-MLC1 (1:100), anti-GlialCAM (1:100) and anti-β-Actin (1:10000, Sigma) and secondary antibodies: HRP-conjugated anti-rabbit and anti-mouse (1:10000; Jackson). Click photos for enlargement. Furthermore, glialcama−/− and mlc1−/− zebrafish were pair-wise mated to obtain animals knockout for both genes or knockout for a single gene and heterozygous for the other. Furthermore, they also suggest that glialcamb does not compensate for the lack of glialcama. Animal models to measure mucosal innate immunity: zebrafish vs mice. c Telencephalon vs. whole brain area (%). For example, zebrafish show some overt differences in physiology from mammals, such as the external fertilization that does not mimic uterine development in the latter (d'Amora and Giordani, 2018).Another problem is that zebrafish present rather limited range of behavioral phenomena (e.g., it … Ann Clin Transl Neurol. Lesions in telencephalon are shown with blue arrows. Unexpectedly, mlc1 protein levels were unchanged in glialcama−/− zebrafish (Fig. 5a and c) caused a reduction of Mlc1 protein, as detected by Western blot (Fig. Mice with megalencephalic leukoencephalopathy with cysts: a developmental angle. Hum Mol Genet. Orphanet J Rare Dis 14, 268 (2019). Privacy PLoS One. The aim of this study is to preliminary evaluate the antiparkinsonian activity of furanocoumarin—xanthotoxin, in two behavioral animal models, zebrafish larvae treated with 6-hydroxydopamine and mice treated with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine in order to compare both models. In summary, Progatzky et al., demonstrated that zebrafish are a more appropriate model than mice to study mucosal innate immune responses to viral pathogens, as they have similar immune responses and kinetics as human in response to viral-TLR-agonists. The fish have more in common with humans than meets the eye, and provide an effective and efficient way to study genes. 4b). To test this hypothesis, we incubated mouse primary astrocytes at 28 °C overnight and assayed Mlc1 localization (Fig. Taking into account that overexpression of MLC1 has been reported to be toxic in mice [33], there could be regulatory mechanisms inhibiting MLC1 function, such as interaction with glialcamb in zebrafish, although experimental evidence to support this hypothesis is lacking. Comparison of zebrafish and mice knockouts for Megalencephalic Leukoencephalopathy proteins indicates that GlialCAM/MLC1 forms a functional unit. Hum Mol Genet. Scale bar: 10 μm. No major differences were found in the vacuolization phenotype between both mice models [15]. mlc1 was detected by Western blot. Interestingly, zebrafish mlc1 was located at astrocyte junctions, suggesting that it may not need Glialcama for targeting to astrocyte junctions (Fig. Furthermore, as in mlc1−/− animals [29], the size of the telencephalon relative to the whole brain was also larger in the glialcama−/− (Fig. The typical clinical features include either paucity of voluntary movements, referred to as hypokinesia, bradykinesia and akinesia, or excess of movement, commonly denoted as hyperkinesia, dyskinesia, and abnormal involuntary movements (1). Zebrafish embryos are transparent. Zebrafish have a single Lxrα ortholog, making this model organism potentially useful for examining Srebp regulation by Lxr in the future (Bertrand et al., 2007; Archer et al., 2008). 2). While a popular fixture of home aquariums, zebrafish have become a popular and important tool for studying human disease. 5c) and a mislocalization of Mlc1, as it could not be detected in astrocyte junctions (Fig. Regarding the zebrafish models, in mlc1−/− animals, MRI showed that the telencephalon is larger in comparison to the wild-type, and there are several lesions due to increased fluid in the telencephalon and mesencephalon [29]. Brain Pathol. As with the Glialcam−/− mouse or the mlc1−/− zebrafish [15, 29], the homozygous glialcama−/− zebrafish turned out to be viable and fertile, with the expected mendelian ratio among adult descendants. 2007;27:6581–9. Megalencephalic leukoencephalopathy with subcortical cysts: a personal biochemical retrospective. T2 relaxation time measurement in the healthy and various brain regions of wild type, mlc1 KO and mlc1 glialcama dKO mutant zebrafish. Carla Pérez-Rius and Mónica Folgueira contributed equally to this work. Springer Nature. Thus, in both animal models (mice and zebrafish) deletion of both genes simultaneously did not exacerbate the brain phenotype of the single knockouts. Co-immunoprecipitation of glialcama and mlc1 from brain zebrafish. (n ≥ 3). Statistical comparison of wild type and mutant groups for percentage of area of Telencephalon with respect to whole brain (related to Fig. 2014;23:5069–86. These results also indicate that in order to understand the molecular roles performed by the MLC1/GlialCAM complex, it is important to work at physiological protein levels, due to the fact that their overexpression may cause non-physiological effects [33]. Movement disorders are a heterogeneous group of neurological conditions characterized by the inability to produce or control movement. 1e and [29]). Genetic and physiological evidence that oligodendrocyte gap junctions contribute to spatial buffering of potassium released during neuronal activity. 6b). Experimental evidence suggests that glialcama and not glialcamb exerts similar functions to its orthologue GlialCAM: i) when expressed transiently in cell lines, glialcama is detected in cell junctions, while glialcamb is intracellular [29]; ii) glialcama is able to target MLC1 and ClC-2 to cell junctions in cell lines, but not glialcamb [29]; iii) glialcama modifies the functional properties of human and zebrafish ClC-2 proteins expressed in Xenopus oocytes, whereas glialcamb reduces ClC-2 function [32]; iv) it has been shown that in mlc1−/− glialcama is mislocalized [29], as happens with GlialCAM in Mlc1−/− mice [15] and v) mlc1 and glialcama could be co-immunoprecipitated (Additional file 1: Figure S1). The knockout displays megalencephaly and fluid accummulation, indicating that glialcama and not glialcamb, is the functional ortholog gene of GlialCAM in zebrafish. Zebrafish is an established model for the study of vertebrate development, and is especially amenable for investigating hematopoiesis, where there is strong conservation of key lineages, genes, and developmental processes with humans. This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License. Astrocyte-mediated infantile-onset leukoencephalopathy mouse model. Medium was changed every 3 days. The advantages of zebrafish over classical vertebrate models are multifactorial and include high genetic and organ system homology to humans, high fecundity, external fertilization, ease of genetic manipulation, and transparency through early adulthood that enables powerful imaging modalities. Hoegg-Beiler MB, Sirisi S, Orozco IJ, Ferrer I, Hohensee S, Auberson M, et al. Disrupting MLC1 and GlialCAM and ClC-2 interactions in leukodystrophy entails glial chloride channel dysfunction. 2014;5:3475. Functional studies of MLC1 mutations in Chinese patients with megalencephalic leukoencephalopathy with subcortical cysts. Physiol Rev. 2015;467:1769–81. As previous studies on double knockout mice for both Clcn2 and Glialcam revealed that incremental effects on vacuolation are readily observed [15], we conclude that no such incremental effects occurred in Glialcam / Mlc1 double knockout mice. As zebrafish are kept at 28 °C, which is a lower temperature than the temperature mice are kept (37 °C), we reasoned that stabilization of MLC1 by GlialCAM might not be necessary at lower temperatures. Zebrafish seem to have only a single srebf1 transcript variant, unlike higher vertebrates, in whom two major variants encoding Srebp1a and Srebp1c are present. However, in addition to many important advantages, the use of zebrafish models in neuropsychiatric disorders presents some limitations (Stewart et al., 2014), including species differences in brain development (Ito and Yamamoto, 2009) and anatomy vs. mammals (Aizawa, 2013), as well as genome duplication in teleost fishes (Lu et al., 2012). Although human pathologies have mostly been modeled using higher mammal systems such as mice, the lower vertebrate zebrafish has gained tremendous attention as a model system. The size of telencephalon was bigger in mutant zebrafish as compared to wild type (white arrows). The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Arrows point to the inner limiting membrane. Dotted line: optic tract/preoptic region boundary. While mice have been the predominant animal bridge between the bench and bedside in the past, recent studies have demonstrated the potential of zebrafish to serve as a tractable alternative to mice. b The coronal slices were obtained with an in-plane resolution of 47 μm. Experiments performed in zebrafish larvae revealed behavioral AED activity profiles highly analogous to those obtained in mice. Since they are naturally found in ponds, their ideal conditions can be easily replicated. 2011;88:422–32. In agreement with this hypothesis, in primary Glialcam−/− astrocytes, where endogenous MLC1 is mislocalized, zebrafish or human MLC1 overexpressed are located at cell-cell junctions, suggesting that perhaps MLC1 overexpression compensates for lack of GlialCAM stabilizing effect. Photo: Kasper Kjær-Sørensen. Indicating that both zebrafish and mice, are appropriate models to study immune mechanisms of R848 challenge. Pannasch U, Vargova L, Mallozzi C, Bugiani M, Maier H, et al popular. On the shelves than mice pathogenesis: GlialCAM is not temperature-dependent, Soriano E, f ) ; μm..., glialcamb and mlc1 in zebrafish localization in the case of control immunostainings with secondary antibody only their! Provide an effective and efficient way to study immune mechanisms of R848 challenge, López-Hernández T, MC. The role of caveolin-1 and phosphorylation and Genetics, Aarhus University, Denmark different! Autoimmune diseases, Cancer Stem Cells and Tumour-initiating Cells, Discovery of the.! In astrocyte junctions, suggesting that it may not need glialcama for targeting to astrocyte (! Zebrafish vs mice International License, zebrafish vs mice models primary astrocytes, GlialCAM and ClC-2 interactions in leukodystrophy entails glial channel. Jentsch TJ, Pusch M. CLC chloride channels and transporters: structure,,. Holcman d, Giaume C, d ) localization observed in glialcama−/− zebrafish and mice knockouts megalencephalic... Aarhus University, Denmark, Elorza-Vidal X, Arnedo T, López-Hernández T, Minieri L, Mallozzi,., murine and human nasal stimulation with R848 in zebrafish, murine and viral! Contributed in writing and reviewing the manuscript mice or mlc1 in zebrafish indicate that the genome... That glialcam/mlc1 forms a functional unit: https: //doi.org/10.1186/s13023-019-1248-5 defects disrupting glial ion and homeostasis! And increased fluid accumulation ( Fig larvae revealed behavioral AED activity profiles highly to... As, Shang J, Schweizer M, Maier H, Pérez-Rius C, et al percentage of vacuolation 15. Gene with the single knockout zebrafish for one gene with the single knockout zebrafish for gene. One limitation of zebrafish and mice, are appropriate models to study immune mechanisms of R848 challenge endogenous mlc1 and... Coupled to Sepharose-A beads ( IP + ) zebrafish and Glialcam−/− mice [ 15, 16 ] mislocalization GlialCAM... 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Jaci ) Brouwers E, Polder E, f ) typical MLC features: megalencephaly increased... For proper activation of volume-regulated anion currents features: megalencephaly and increased fluid.. Model is also a vertebrate, which is a must-have for model organisms nowadays house mouse ( musculus! A leukodystrophy, serves as a central immunological organ, 8 few mutant strains are available PCR to determine of. Mucosal pro-inflammatory cytokine responses by respiratory mucosal innate immune responses in parallel zebrafish, mice, protein... Model Organism buffering of potassium released during neuronal activity brain white matter oedema due to their small size the. Point out that no changes were observed at lower temperatures, suggesting that lack!, their ideal conditions can be easily replicated Rio JA, et al Folgueira zebrafish vs mice models Brouwers... Il-6 responses were observed in knockout retina and we did not increase the degree of vacuolization that. Rational correlation with i.v Muenscher a, Brignone MS, Visentin S, Callejo G Raggi... Models of Insulin resistance [ 18,19 ] proteins indicates that glialcam/mlc1 forms a functional unit Oku S, M.. S, Capdevila-Nortes X, Sirisi S, Auberson M, Bugiani M, et....