{"PubmedArticle":{"MedlineCitation":{"@attributes":{"Status":"MEDLINE","Owner":"NLM","IndexingMethod":"Curated"},"PMID":{"@attributes":{"Version":"1"},"@text":"33247858"},"DateCompleted":{"Year":"2022","Month":"02","Day":"07"},"DateRevised":{"Year":"2022","Month":"02","Day":"07"},"Article":{"@attributes":{"PubModel":"Print-Electronic"},"Journal":{"ISSN":{"@attributes":{"IssnType":"Electronic"},"@text":"1098-1136"},"JournalIssue":{"@attributes":{"CitedMedium":"Internet"},"Volume":"69","Issue":"4","PubDate":{"Year":"2021","Month":"Apr"}},"Title":"Glia","ISOAbbreviation":"Glia"},"ArticleTitle":"Dp71 contribution to the molecular scaffold anchoring aquaporine-4 channels in brain macroglial cells.","Pagination":{"StartPage":"954","EndPage":"970","MedlinePgn":"954-970"},"ELocationID":[{"@attributes":{"EIdType":"doi","ValidYN":"Y"},"@text":"10.1002\/glia.23941"}],"Abstract":{"AbstractText":["Intellectual disability in Duchenne muscular dystrophy has been associated with the loss of dystrophin-protein 71, Dp71, the main dystrophin-gene product in the adult brain. Dp71 shows major expression in perivascular macroglial endfeet, suggesting that dysfunctional glial mechanisms contribute to cognitive impairments. In the present study, we investigated the molecular alterations induced by a selective loss of Dp71 in mice, using semi-quantitative immunogold analyses in electron microscopy and immunofluorescence confocal analyses in brain sections and purified gliovascular units. In macroglial pericapillary endfeet of the cerebellum and hippocampus, we found a drastic reduction (70%) of the polarized distribution of aquaporin-4 (AQP4) channels, a 50% reduction of \u03b2-dystroglycan, and a complete loss of \u03b11-syntrophin. Interestingly, in the hippocampus and cortex, these effects were not homogeneous: AQP4 and AQP4ex isoforms were mostly lost around capillaries but preserved in large vessels corresponding to pial arteries, penetrating cortical arterioles, and arterioles of the hippocampal fissure, indicating the presence of Dp71-independent pools of AQP4 in these vascular structures. In conclusion, the depletion of Dp71 strongly alters the distribution of AQP4 selectively in macroglial perivascular endfeet surrounding capillaries. This effect likely affects water homeostasis and blood-brain barrier functions and may thus contribute to the synaptic and cognitive defects associated with Dp71 deficiency."],"CopyrightInformation":"\u00a9 2020 Wiley Periodicals LLC."},"AuthorList":{"@attributes":{"CompleteYN":"Y"},"Author":[{"@attributes":{"ValidYN":"Y"},"LastName":"Belmaati Cherkaoui","ForeName":"Mehdi","Initials":"M","AffiliationInfo":[{"Affiliation":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay, Gif-sur-Yvette, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Vacca","ForeName":"Oph\u00e9lie","Initials":"O","AffiliationInfo":[{"Affiliation":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay, Gif-sur-Yvette, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Izabelle","ForeName":"Charlotte","Initials":"C","AffiliationInfo":[{"Affiliation":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay, Gif-sur-Yvette, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Boulay","ForeName":"Anne-C\u00e9cile","Initials":"AC","AffiliationInfo":[{"Affiliation":"Physiology and Physiopathology of the Gliovascular Unit Research Group, Center for Interdisciplinary Research in Biology (CIRB), Coll\u00e8ge de France, Unit\u00e9 Mixte de Recherche 7241CNRS, Unit\u00e9 1050 INSERM, PSL Research University, Paris, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Boulogne","ForeName":"Claire","Initials":"C","AffiliationInfo":[{"Affiliation":"Institute for Integrative Biology of the Cell (I2BC), CEA, CNRS, Univ Paris-Sud, Universit\u00e9 Paris-Saclay, Gif-sur-Yvette, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Gillet","ForeName":"Cynthia","Initials":"C","AffiliationInfo":[{"Affiliation":"Institute for Integrative Biology of the Cell (I2BC), CEA, CNRS, Univ Paris-Sud, Universit\u00e9 Paris-Saclay, Gif-sur-Yvette, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Barnier","ForeName":"Jean-Vianney","Initials":"JV","AffiliationInfo":[{"Affiliation":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay, Gif-sur-Yvette, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Rendon","ForeName":"Alvaro","Initials":"A","AffiliationInfo":[{"Affiliation":"UPMC Universit\u00e9 Paris 06, INSERM, CNRS, Institut de la Vision, Sorbonne Universit\u00e9s, Paris, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Cohen-Salmon","ForeName":"Martine","Initials":"M","Identifier":[{"@attributes":{"Source":"ORCID"},"@text":"0000-0002-5312-8476"}],"AffiliationInfo":[{"Affiliation":"Physiology and Physiopathology of the Gliovascular Unit Research Group, Center for Interdisciplinary Research in Biology (CIRB), Coll\u00e8ge de France, Unit\u00e9 Mixte de Recherche 7241CNRS, Unit\u00e9 1050 INSERM, PSL Research University, Paris, France."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Vaillend","ForeName":"Cyrille","Initials":"C","Identifier":[{"@attributes":{"Source":"ORCID"},"@text":"0000-0002-8783-8185"}],"AffiliationInfo":[{"Affiliation":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay, Gif-sur-Yvette, France."}]}]},"Language":["eng"],"PublicationTypeList":{"PublicationType":[{"@attributes":{"UI":"D016428"},"@text":"Journal Article"},{"@attributes":{"UI":"D013485"},"@text":"Research Support, Non-U.S. Gov't"}]},"ArticleDate":[{"@attributes":{"DateType":"Electronic"},"Year":"2020","Month":"11","Day":"28"}]},"MedlineJournalInfo":{"Country":"United States","MedlineTA":"Glia","NlmUniqueID":"8806785","ISSNLinking":"0894-1491"},"ChemicalList":{"Chemical":[{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D051401"},"@text":"Aquaporin 4"}},{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D016189"},"@text":"Dystrophin"}},{"RegistryNumber":"146888-27-9","NameOfSubstance":{"@attributes":{"UI":"D049030"},"@text":"Dystroglycans"}}]},"CitationSubset":["IM"],"MeshHeadingList":{"MeshHeading":[{"DescriptorName":{"@attributes":{"UI":"D000818","MajorTopicYN":"N"},"@text":"Animals"}},{"DescriptorName":{"@attributes":{"UI":"D051401","MajorTopicYN":"N"},"@text":"Aquaporin 4"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"},{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D001253","MajorTopicYN":"N"},"@text":"Astrocytes"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D001921","MajorTopicYN":"N"},"@text":"Brain"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D049030","MajorTopicYN":"Y"},"@text":"Dystroglycans"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"}]},{"DescriptorName":{"@attributes":{"UI":"D016189","MajorTopicYN":"Y"},"@text":"Dystrophin"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"}]},{"DescriptorName":{"@attributes":{"UI":"D051379","MajorTopicYN":"N"},"@text":"Mice"}},{"DescriptorName":{"@attributes":{"UI":"D009457","MajorTopicYN":"N"},"@text":"Neuroglia"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]}]},"KeywordList":[{"@attributes":{"Owner":"NOTNLM"},"Keyword":[{"@attributes":{"MajorTopicYN":"N"},"@text":"blood-brain barrier"},{"@attributes":{"MajorTopicYN":"N"},"@text":"dystrophin"},{"@attributes":{"MajorTopicYN":"N"},"@text":"gliovascular unit"},{"@attributes":{"MajorTopicYN":"N"},"@text":"glymphatic system"},{"@attributes":{"MajorTopicYN":"N"},"@text":"intellectual disability"}]}]},"PubmedData":{"History":{"PubMedPubDate":[{"@attributes":{"PubStatus":"received"},"Year":"2020","Month":"4","Day":"23"},{"@attributes":{"PubStatus":"revised"},"Year":"2020","Month":"11","Day":"13"},{"@attributes":{"PubStatus":"accepted"},"Year":"2020","Month":"11","Day":"17"},{"@attributes":{"PubStatus":"pubmed"},"Year":"2020","Month":"11","Day":"29","Hour":"6","Minute":"0"},{"@attributes":{"PubStatus":"medline"},"Year":"2022","Month":"2","Day":"8","Hour":"6","Minute":"0"},{"@attributes":{"PubStatus":"entrez"},"Year":"2020","Month":"11","Day":"28","Hour":"12","Minute":"5"}]},"PublicationStatus":"ppublish","ArticleIdList":{"ArticleId":[{"@attributes":{"IdType":"pubmed"},"@text":"33247858"},{"@attributes":{"IdType":"doi"},"@text":"10.1002\/glia.23941"}]},"ReferenceList":[{"Title":"REFERENCES","Reference":[{"Citation":"Abbott, N. J., Pizzo, M. E., Preston, J. E., Janigro, D., & Thorne, R. G. (2018). The role of brain barriers in fluid movement in the CNS: Is there a 'glymphatic' system? Acta Neuropathologica, 135(3), 387-407. https:\/\/doi.org\/10.1007\/s00401-018-1812-4"},{"Citation":"Adams, M. E., Mueller, H. A., & Froehner, S. C. (2001). In vivo requirement of the alpha-syntrophin PDZ domain for the sarcolemmal localization of nNOS and aquaporin-4. The Journal of Cell Biology, 155(1), 113-122. https:\/\/doi.org\/10.1083\/jcb.200106158"},{"Citation":"Amiry-Moghaddam, M., Frydenlund, D. S., & Ottersen, O. P. (2004). Anchoring of aquaporin-4 in brain: Molecular mechanisms and implications for the physiology and pathophysiology of water transport. Neuroscience, 129(4), 999-1010. https:\/\/doi.org\/10.1016\/j.neuroscience.2004.08.049"},{"Citation":"Amiry-Moghaddam, M., Hoddevik, E. H., & Ottersen, O. P. (2010). Aquaporins: multifarious roles in brain. Neuroscience, 168(4), 859-861. https:\/\/doi.org\/10.1016\/j.neuroscience.2010.04.071"},{"Citation":"Amiry-Moghaddam, M., Otsuka, T., Hurn, P. D., Traystman, R. J., Haug, F. M., Froehner, S. C., \u2026 Bhardwaj, A. (2003). An alpha-syntrophin-dependent pool of AQP4 in astroglial end-feet confers bidirectional water flow between blood and brain. Proceedings of the National Academy of Sciences of the United States of America, 100(4), 2106-2111. https:\/\/doi.org\/10.1073\/pnas.0437946100"},{"Citation":"Amiry-Moghaddam, M., Williamson, A., Palomba, M., Eid, T., de Lanerolle, N. C., Nagelhus, E. A., \u2026 Ottersen, O. P. (2003). Delayed K+ clearance associated with aquaporin-4 mislocalization: Phenotypic defects in brains of alpha-syntrophin-null mice. Proceedings of the National Academy of Sciences of the United States of America, 100(23), 13615-13620. https:\/\/doi.org\/10.1073\/pnas.2336064100"},{"Citation":"Arag\u00f3n, J., Gonz\u00e1lez-Reyes, M., Romo-Y\u00e1\u00f1ez, J., Vacca, O., Aguilar-Gonz\u00e1lez, G., Rend\u00f3n, A., \u2026 Monta\u00f1ez, C. (2018). Dystrophin Dp71 isoforms are differentially expressed in the mouse brain and retina: Report of new alternative splicing and a novel nomenclature for Dp71 isoforms. Molecular Neurobiology, 55(2), 1376-1386. https:\/\/doi.org\/10.1007\/s12035-017-0405-x"},{"Citation":"Austin, R. C., Morris, G. E., Howard, P. L., Klamut, H. J., & Ray, P. N. (2000). Expression and synthesis of alternatively spliced variants of Dp71 in adult human brain. Neuromuscular Disorders, 10(3), 187-193. https:\/\/doi.org\/10.1016\/s0960-8966(99)00105-4"},{"Citation":"Barboni, M. T. S., Vaillend, C., Joachimsthaler, A., Liber, A. M. P., Khabou, H., Roux, M. J., \u2026 Kremers, J. (2020). Rescue of defective electroretinographic responses in Dp71-null mice with AAV-mediated reexpression of Dp71. Investigative Ophthalmology & Visual Science, 61(2), 11. https:\/\/doi.org\/10.1167\/iovs.61.2.11"},{"Citation":"Benabdesselam, R., Dorbani-Mamine, L., Benmessaoud-Mesbah, O., Rendon, A., Mhaouty-Kodja, S., & Hardin-Pouzet, H. (2012). Dp71 gene disruption alters the composition of the dystrophin-associated protein complex and neuronal nitric oxide synthase expression in the hypothalamic supraoptic and paraventricular nuclei. The Journal of Endocrinology, 213(3), 239-249. https:\/\/doi.org\/10.1530\/JOE-12-0066"},{"Citation":"Benabdesselam, R., Sene, A., Raison, D., Benmessaoud-Mesbah, O., Ayad, G., Mornet, D., \u2026 Dorbani-Mamine, L. (2010). A deficit of brain dystrophin 71 impairs hypothalamic osmostat. Journal of Neuroscience Research, 88(2), 324-334. https:\/\/doi.org\/10.1002\/jnr.22198"},{"Citation":"Blauth, K., Soltys, J., Matschulat, A., Reiter, C. R., Ritchie, A., Baird, N. L., \u2026 Owens, G. P. (2015). Antibodies produced by clonally expanded plasma cells in multiple sclerosis cerebrospinal fluid cause demyelination of spinal cord explants. Acta Neuropathologica, 130(6), 765-781. https:\/\/doi.org\/10.1007\/s00401-015-1500-6"},{"Citation":"Boulay, A. C., Saubam\u00e9a, B., Decl\u00e8ves, X., & Cohen-Salmon, M. (2015). Purification of mouse brain vessels. Journal of Visualized Experiments, 105, e53208. https:\/\/doi.org\/10.3791\/53208"},{"Citation":"Bragg, A. D., Das, S. S., & Froehner, S. C. (2010). Dystrophin-associated protein scaffolding in brain requires alpha-dystrobrevin. Neuroreport, 21(10), 695-699. https:\/\/doi.org\/10.1097\/WNR.0b013e32833b0a3b"},{"Citation":"Chaussenot, R., Amar, M., Fossier, P., & Vaillend, C. (2019). Dp71-dystrophin deficiency alters prefrontal cortex excitation-inhibition balance and executive functions. Molecular Neurobiology, 56(4), 2670-2684. https:\/\/doi.org\/10.1007\/s12035-018-1259-6"},{"Citation":"Culligan, K., Glover, L., Dowling, P., & Ohlendieck, K. (2001). Brain dystrophin-glycoprotein complex: Persistent expression of beta-dystroglycan, impaired oligomerization of Dp71 and up-regulation of utrophins in animal models of muscular dystrophy. BMC Cell Biology, 2, 2. https:\/\/doi.org\/10.1186\/1471-2121-2-2"},{"Citation":"Dalloz, C., Sarig, R., Fort, P., Yaffe, D., Bordais, A., Pannicke, T., \u2026 Rendon, A. (2003). Targeted inactivation of dystrophin gene product Dp71: Phenotypic impact in mouse retina. Human Molecular Genetics, 12(13), 1543-1554. https:\/\/doi.org\/10.1093\/hmg\/ddg170"},{"Citation":"Daoud, F., Angeard, N., Demerre, B., Martie, I., Benyaou, R., Leturcq, F., \u2026 Chelly, J. (2009). Analysis of Dp71 contribution in the severity of mental retardation through comparison of Duchenne and Becker patients differing by mutation consequences on Dp71 expression. Human Molecular Genetics, 18(20), 3779-3794. https:\/\/doi.org\/10.1093\/hmg\/ddp320"},{"Citation":"Daoud, F., Candelario-Mart\u00ednez, A., Billard, J. M., Avital, A., Khelfaoui, M., Rozenvald, Y., \u2026 Vaillend, C. (2009). Role of mental retardation associated dystrophin-gene product Dp71 in excitatory synapse organization, synaptic plasticity and behavioral functions. PLoS One, 4(8), e6574. https:\/\/doi.org\/10.1371\/journal.pone.0006574"},{"Citation":"De Bellis, M., Pisani, F., Mola, M. G., Rosito, S., Simone, L., Buccoliero, C., \u2026 Frigeri, A. (2017). Translational readthrough generates new astrocyte AQP4 isoforms that modulate supramolecular clustering, glial endfeet localization, and water transport. Glia, 65(5), 790-803. https:\/\/doi.org\/10.1002\/glia.23126"},{"Citation":"De Brouwer, A. P., Nabuurs, S. B., Verhaart, I. E., Oudakker, A. R., Hordijk, R., Yntema, H. G., \u2026 Kleefstra, T. (2014). A 3-base pair deletion, c.9711_9713del, in DMD results in intellectual disability without muscular dystrophy. European Journal of Human Genetics, 22(4), 480-485. https:\/\/doi.org\/10.1038\/ejhg.2013.169"},{"Citation":"Desguerre, I., Christov, C., Mayer, M., Zeller, R., Becane, H. M., Bastuji-Garin, S., \u2026 Gherardi, R. K. (2009). Clinical heterogeneity of Duchenne muscular dystrophy (DMD): Definition of sub-phenotypes and predictive criteria by long-term follow-up. PLoS One, 4(2), e4347. https:\/\/doi.org\/10.1371\/journal.pone.0004347"},{"Citation":"Eid, T., Lee, T. S., Thomas, M. J., Amiry-Moghaddam, M., Bj\u00f8rnsen, L. P., Spencer, D. D., \u2026 de Lanerolle, N. C. (2005). Loss of perivascular aquaporin 4 may underlie deficient water and K+ homeostasis in the human epileptogenic hippocampus. Proceedings of the National Academy of Sciences of the United States of America, 102(4), 1193-1198. https:\/\/doi.org\/10.1073\/pnas.0409308102"},{"Citation":"Eidsvaag, V. A., Enger, R., Hansson, H. A., Eide, P. K., & Nagelhus, E. A. (2017). Human and mouse cortical astrocytes differ in aquaporin-4 polarization toward microvessels. Glia, 65(6), 964-973. https:\/\/doi.org\/10.1002\/glia.23138"},{"Citation":"El Mathari, B., Sene, A., Charles-Messance, H., Vacca, O., Guillonneau, X., Grepin, C., \u2026 Tadayoni, R. (2015). Dystrophin Dp71 gene deletion induces retinal vascular inflammation and capillary degeneration. Human Molecular Genetics, 24(14), 3939-3947. https:\/\/doi.org\/10.1093\/hmg\/ddv132"},{"Citation":"Enger, R., Gundersen, G. A., Haj-Yasein, N. N., Eilert-Olsen, M., Thoren, A. E., Vindedal, G. F., \u2026 Nagelhus, E. A. (2012). Molecular scaffolds underpinning macroglial polarization: An analysis of retinal M\u00fcller cells and brain astrocytes in mouse. Glia, 60(12), 2018-2026. https:\/\/doi.org\/10.1002\/glia.22416"},{"Citation":"Farmer, W. T., Abrahamsson, T., Chierzi, S., Lui, C., Zaelzer, C., Jones, E. V., \u2026 Murai, K. K. (2016). Neurons diversify astrocytes in the adult brain through sonic hedgehog signaling. Science, 351(6275), 849-854. https:\/\/doi.org\/10.1126\/science.aab3103"},{"Citation":"Frigeri, A., Nicchia, G. P., Nico, B., Quondamatteo, F., Herken, R., Roncali, L., & Svelto, M. (2001). Aquaporin-4 deficiency in skeletal muscle and brain of dystrophic mdx mice. The FASEB Journal, 15(1), 90-98. https:\/\/doi.org\/10.1096\/fj.00-0260com"},{"Citation":"Frydenlund, D. S., Bhardwaj, A., Otsuka, T., Mylonakou, M. N., Yasumura, T., Davidson, K. G., \u2026 Amiry-Moghaddam, M. (2006). Temporary loss of perivascular aquaporin-4 in neocortex after transient middle cerebral artery occlusion in mice. Proceedings of the National Academy of Sciences of the United States of America, 103(36), 13532-13536. https:\/\/doi.org\/10.1073\/pnas.0605796103"},{"Citation":"Fukuda, A. M., & Badaut, J. (2012). Aquaporin 4: A player in cerebral edema and neuroinflammation. Journal of Neuroinflammation, 9, 279. https:\/\/doi.org\/10.1186\/1742-2094-9-279"},{"Citation":"Haenggi, T., Schaub, M. C., & Fritschy, J. M. (2005). Molecular heterogeneity of the dystrophin-associated protein complex in the mouse kidney nephron: Differential alterations in the absence of utrophin and dystrophin. Cell and Tissue Research, 319(2), 299-313. https:\/\/doi.org\/10.1007\/s00441-004-0999-y"},{"Citation":"Haenggi, T., Soontornmalai, A., Schaub, M. C., & Fritschy, J. M. (2004). The role of utrophin and Dp71 for assembly of different dystrophin-associated protein complexes (DPCs) in the choroid plexus and microvasculature of the brain. Neuroscience, 129(2), 403-413. https:\/\/doi.org\/10.1016\/j.neuroscience.2004.06.079"},{"Citation":"Helleringer, R., Le Verger, D., Li, X., Izabelle, C., Chaussenot, R., Belmaati-Cherkaoui, M., \u2026 Vaillend, C. (2018). Cerebellar synapse properties and cerebellum-dependent motor and non-motor performance in Dp71-null mice. Disease Models & Mechanisms, 11, 7. https:\/\/doi.org\/10.1242\/dmm.033258"},{"Citation":"Hoddevik, E. H., Khan, F. H., Rahmani, S., Ottersen, O. P., Boldt, H. B., & Amiry-Moghaddam, M. (2017). Factors determining the density of AQP4 water channel molecules at the brain-blood interface. Brain Structure & Function, 222(4), 1753-1766. https:\/\/doi.org\/10.1007\/s00429-016-1305-y"},{"Citation":"Hubbard, J. A., Hsu, M. S., Seldin, M. M., & Binder, D. K. (2015). Expression of the astrocyte water channel Aquaporin-4 in the mouse brain. ASN Neuro, 7, 5. https:\/\/doi.org\/10.1177\/1759091415605486"},{"Citation":"Hubbard, J. A., Szu, J. I., & Binder, D. K. (2018). The role of aquaporin-4 in synaptic plasticity, memory and disease. Brain Research Bulletin, 136, 118-129. https:\/\/doi.org\/10.1016\/j.brainresbull.2017.02.011"},{"Citation":"Inoue, M., Wakayama, Y., Liu, J. W., Murahashi, M., Shibuya, S., & Oniki, H. (2002). Ultrastructural localization of aquaporin 4 and alpha1-syntrophin in the vascular feet of brain astrocytes. The Tohoku Journal of Experimental Medicine, 197(2), 87-93. https:\/\/doi.org\/10.1620\/tjem.197.87"},{"Citation":"Kawashima, H., Sueyoshi, S., Li, H., Yamamoto, K., & Osawa, T. (1990). Carbohydrate binding specificities of several poly-N-acetyllactosamine-binding lectins. Glycoconjugate Journal, 7(4), 323-334. https:\/\/doi.org\/10.1007\/bf01073376"},{"Citation":"Knuesel, I., Bornhauser, B. C., Zuellig, R. A., Heller, F., Schaub, M. C., & Fritschy, J. M. (2000). Differential expression of utrophin and dystrophin in CNS neurons: An in situ hybridization and immunohistochemical study. The Journal of Comparative Neurology, 422(4), 594-611. https:\/\/doi.org\/10.1002\/1096-9861(20000710)422:4<594::aid-cne8>3.0.co;2-q"},{"Citation":"K\u00f6ferl, P., Hollborn, M., Rehak, J., Iandiev, I., Dukic-Stefanovic, S., Wiedemann, P., \u2026 Rehak, M. (2014). Effects of arteriolar constriction on retinal gene expression and M\u00fcller cell responses in a rat model of branch retinal vein occlusion. Graefe's Archive for Clinical and Experimental Ophthalmology, 252(2), 257-265. https:\/\/doi.org\/10.1007\/s00417-013-2532-z"},{"Citation":"Kofuji, P., & Newman, E. A. (2004). Potassium buffering in the central nervous system. Neuroscience, 129(4), 1045-1056. https:\/\/doi.org\/10.1016\/j.neuroscience.2004.06.008"},{"Citation":"Li, D., Yue, Y., & Duan, D. (2008). Preservation of muscle force in Mdx3cv mice correlates with low-level expression of a near full-length dystrophin protein. The American Journal of Pathology, 172(5), 1332-1341. https:\/\/doi.org\/10.2353\/ajpath.2008.071042"},{"Citation":"Li, D., Yue, Y., & Duan, D. (2010). Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin\/utrophin double knockout mice. PLoS One, 5(12), e15286. https:\/\/doi.org\/10.1371\/journal.pone.0015286"},{"Citation":"Lidov, H. G., Selig, S., & Kunkel, L. M. (1995). Dp140: A novel 140 kDa CNS transcript from the dystrophin locus. Human Molecular Genetics, 4(3), 329-335. https:\/\/doi.org\/10.1093\/hmg\/4.3.329"},{"Citation":"Lien, C. F., Mohanta, S. K., Frontczak-Baniewicz, M., Swinny, J. D., Zablocka, B., & G\u00f3recki, D. C. (2012). Absence of glial \u03b1-dystrobrevin causes abnormalities of the blood-brain barrier and progressive brain edema. The Journal of Biological Chemistry, 287(49), 41374-41385. https:\/\/doi.org\/10.1074\/jbc.M112.400044"},{"Citation":"Morris, G. E., Simmons, C., & Nguyen, T. M. (1995). Apo-dystrophins (Dp140 and Dp71) and dystrophin splicing isoforms in developing brain. Biochemical and Biophysical Research Communications, 215(1), 361-367. https:\/\/doi.org\/10.1006\/bbrc.1995.2474"},{"Citation":"Naidoo, M., & Anthony, K. (2020). Dystrophin Dp71 and the neuropathophysiology of Duchenne muscular dystrophy. Molecular Neurobiology, 57(3), 1748-1767. https:\/\/doi.org\/10.1007\/s12035-019-01845-w"},{"Citation":"Neely, J. D., Amiry-Moghaddam, M., Ottersen, O. P., Froehner, S. C., Agre, P., & Adams, M. E. (2001). Syntrophin-dependent expression and localization of Aquaporin-4 water channel protein. Proceedings of the National Academy of Sciences of the United States of America, 98(24), 14108-14113. https:\/\/doi.org\/10.1073\/pnas.241508198"},{"Citation":"Nicchia, G. P., Cogotzi, L., Rossi, A., Basco, D., Brancaccio, A., Svelto, M., & Frigeri, A. (2008). Expression of multiple AQP4 pools in the plasma membrane and their association with the dystrophin complex. Journal of Neurochemistry, 105(6), 2156-2165. https:\/\/doi.org\/10.1111\/j.1471-4159.2008.05302.x"},{"Citation":"Nicchia, G. P., Nico, B., Camassa, L. M., Mola, M. G., Loh, N., Dermietzel, R., \u2026 Frigeri, A. (2004). The role of aquaporin-4 in the blood-brain barrier development and integrity: Studies in animal and cell culture models. Neuroscience, 129(4), 935-945. https:\/\/doi.org\/10.1016\/j.neuroscience.2004.07.055"},{"Citation":"Nicchia, G. P., Rossi, A., Nudel, U., Svelto, M., & Frigeri, A. (2008). Dystrophin-dependent and -independent AQP4 pools are expressed in the mouse brain. Glia, 56(8), 869-876. https:\/\/doi.org\/10.1002\/glia.20661"},{"Citation":"Nico, B., Corsi, P., Ria, R., Crivellato, E., Vacca, A., Roccaro, A. M., \u2026 Roncali, L. (2006). Increased matrix-metalloproteinase-2 and matrix-metalloproteinase-9 expression in the brain of dystrophic mdx mouse. Neuroscience, 140(3), 835-848. https:\/\/doi.org\/10.1016\/j.neuroscience.2006.02.077"},{"Citation":"Nico, B., Frigeri, A., Nicchia, G. P., Corsi, P., Ribatti, D., Quondamatteo, F., \u2026 Roncali, L. (2003). Severe alterations of endothelial and glial cells in the blood-brain barrier of dystrophic mdx mice. Glia, 42(3), 235-251. https:\/\/doi.org\/10.1002\/glia.10216"},{"Citation":"Nico, B., Ribatti, D., Frigeri, A., Nicchia, G. P., Corsi, P., Svelto, M., & Roncali, L. (2002). Aquaporin-4 expression during development of the cerebellum. Cerebellum, 1(3), 207-212. https:\/\/doi.org\/10.1080\/14734220260418439"},{"Citation":"Noell, S., Wolburg-Buchholz, K., Mack, A. F., Beedle, A. M., Satz, J. S., Campbell, K. P., \u2026 Fallier-Becker, P. (2011). Evidence for a role of dystroglycan regulating the membrane architecture of astroglial endfeet. The European Journal of Neuroscience, 33, 2179-2186. https:\/\/doi.org\/10.1111\/j.1460-9568.2011.07688.x"},{"Citation":"Palazzo, C., Buccoliero, C., Mola, M. G., Abbrescia, P., Nicchia, G. P., Trojano, M., & Frigeri, A. (2019). AQP4ex is crucial for the anchoring of AQP4 at the astrocyte end-feet and for neuromyelitis optica antibody binding. Acta Neuropathologica Communications, 7(1), 51. https:\/\/doi.org\/10.1186\/s40478-019-0707-5"},{"Citation":"Palladino, M., Gatto, I., Neri, V., Straino, S., Smith, R. C., Silver, M., \u2026 Pola, R. (2013). Angiogenic impairment of the vascular endothelium: A novel mechanism and potential therapeutic target in muscular dystrophy. Arteriosclerosis, Thrombosis, and Vascular Biology, 33(12), 2867-2876. https:\/\/doi.org\/10.1161\/ATVBAHA.112.301172"},{"Citation":"Papadopoulos, M. C., & Verkman, A. S. (2005). Aquaporin-4 gene disruption in mice reduces brain swelling and mortality in pneumococcal meningitis. The Journal of Biological Chemistry, 280(14), 13906-13912. https:\/\/doi.org\/10.1074\/jbc.M413627200"},{"Citation":"Paxinos, G., & Franklin, K. B. J. (2001). The mouse brain in stereotaxic coordinates (2nd ed.). San Diego, CA: Academic Press."},{"Citation":"Perronnet, C., Chagneau, C., Le Blanc, P., Samson-Desvignes, N., Mornet, D., Laroche, S., \u2026 Vaillend, C. (2012). Upregulation of brain utrophin does not rescue behavioral alterations in dystrophin-deficient mice. Human Molecular Genetics, 21(10), 2263-2276. https:\/\/doi.org\/10.1093\/hmg\/dds047"},{"Citation":"Perronnet, C., & Vaillend, C. (2010). Dystrophins, utrophins, and associated scaffolding complexes: Role in mammalian brain and implications for therapeutic strategies. Journal of Biomedicine & Biotechnology, 2010, 849426-849419. https:\/\/doi.org\/10.1155\/2010\/849426"},{"Citation":"Porter, G. A., Palade, G. E., & Milici, A. J. (1990). Differential binding of the lectins Griffonia simplicifolia I and Lycopersicon esculentum to microvascular endothelium: Organ-specific localization and partial glycoprotein characterization. European Journal of Cell Biology, 51(1), 85-95."},{"Citation":"Puwarawuttipanit, W., Bragg, A. D., Frydenlund, D. S., Mylonakou, M. N., Nagelhus, E. A., Peters, M. F., \u2026 Amiry-Moghaddam, M. (2006). Differential effect of alpha-syntrophin knockout on aquaporin-4 and Kir4.1 expression in retinal macroglial cells in mice. Neuroscience, 137(1), 165-175. https:\/\/doi.org\/10.1016\/j.neuroscience.2005.08.051"},{"Citation":"Rauch, U., Shami, A., Zhang, F., Carmignac, V., Durbeej, M., & Hultg\u00e5rdh-Nilsson, A. (2012). Increased neointimal thickening in dystrophin-deficient mdx mice. PLoS One, 7(1), e29904. https:\/\/doi.org\/10.1371\/journal.pone.0029904"},{"Citation":"Ricotti, V., Mandy, W. P., Scoto, M., Pane, M., Deconinck, N., Messina, S., \u2026 Muntoni, F. (2016). Neurodevelopmental, emotional, and behavioural problems in Duchenne muscular dystrophy in relation to underlying dystrophin gene mutations. Developmental Medicine and Child Neurology, 58(1), 77-84. https:\/\/doi.org\/10.1111\/dmcn.12922"},{"Citation":"Ricotti, V., Roberts, R. G., & Muntoni, F. (2011). Dystrophin and the brain. Developmental Medicine and Child Neurology, 53(1), 12. https:\/\/doi.org\/10.1111\/j.1469-8749.2010.03836.x"},{"Citation":"Rosic, B., Dukefoss, D. B., \u00c5bj\u00f8rsbr\u00e5ten, K. S., Tang, W., Jensen, V., Ottersen, O. P., \u2026 Nagelhus, E. A. (2019). Aquaporin-4-independent volume dynamics of astroglial endfeet during cortical spreading depression. Glia, 67(6), 1113-1121. https:\/\/doi.org\/10.1002\/glia.23604"},{"Citation":"Sarig, R., Mezger-Lallemand, V., Gitelman, I., Davis, C., Fuchs, O., Yaffe, D., & Nudel, U. (1999). Targeted inactivation of Dp71, the major non-muscle product of the DMD gene: Differential activity of the Dp71 promoter during development. Human Molecular Genetics, 8(1), 1-10. https:\/\/doi.org\/10.1093\/hmg\/8.1.1"},{"Citation":"Sene, A., Tadayoni, R., Pannicke, T., Wurm, A., El Mathari, B., Benard, R., \u2026 Rendon, A. (2009). Functional implication of Dp71 in osmoregulation and vascular permeability of the retina. PLoS One, 4(10), e7329. https:\/\/doi.org\/10.1371\/journal.pone.0007329"},{"Citation":"Smith, A. J., Jin, B. J., Ratelade, J., & Verkman, A. S. (2014). Aggregation state determines the localization and function of M1- and M23-aquaporin-4 in astrocytes. Journal of Cell Biology, 204(4), 559-573. https:\/\/doi.org\/10.1083\/jcb.201308118"},{"Citation":"Souttou, S., Benabdesselam, R., Siqueiros-Marquez, L., Sifi, M., Deliba, M., Vacca, O., \u2026 Dorbani-Mamine, L. (2019). Expression and localization of dystrophins and \u03b2-dystroglycan in the hypothalamic supraoptic nuclei of rat from birth to adulthood. Acta Histochemica, 121(2), 218-226. https:\/\/doi.org\/10.1016\/j.acthis.2018.12.001"},{"Citation":"Stavale, L. M., Soares, E. S., Mendon\u00e7a, M. C., Irazusta, S. P., & da Cruz H\u00f6fling, M. A. (2013). Temporal relationship between aquaporin-4 and glial fibrillary acidic protein in cerebellum of neonate and adult rats administered a BBB disrupting spider venom. Toxicon, 66, 37-46. https:\/\/doi.org\/10.1016\/j.toxicon.2013.01.026"},{"Citation":"Tadayoni, R., Rendon, A., Soria-Jasso, L. E., & Cisneros, B. (2012). Dystrophin Dp71: The smallest but multifunctional product of the Duchenne muscular dystrophy gene. Molecular Neurobiology, 45(1), 43-60. https:\/\/doi.org\/10.1007\/s12035-011-8218-9"},{"Citation":"Vacca, O., Charles-Messance, H., El Mathari, B., Sene, A., Barbe, P., Fouquet, S., \u2026 Rendon, A. (2016). AAV-mediated gene therapy in dystrophin-Dp71 deficient mouse leads to blood-retinal barrier restoration and oedema reabsorption. Human Molecular Genetics, 25(14), 3070-3079. https:\/\/doi.org\/10.1093\/hmg\/ddw159"},{"Citation":"Vacca, O., Darche, M., Schaffer, D. V., Flannery, J. G., Sahel, J. A., Rendon, A., & Dalkara, D. (2014). AAV-mediated gene delivery in Dp71-null mouse model with compromised barriers. Glia, 62(3), 468-476. https:\/\/doi.org\/10.1002\/glia.22617"},{"Citation":"Vaillend, C., Perronnet, C., Ros, C., Gruszczynski, C., Goyenvalle, A., Laroche, S., \u2026 Peltekian, E. (2010). Rescue of a dystrophin-like protein by exon skipping in vivo restores GABAA-receptor clustering in the hippocampus of the mdx mouse. Molecular Therapy, 18(9), 1683-1688. https:\/\/doi.org\/10.1038\/mt.2010.134"},{"Citation":"Vaillend, C., & Ungerer, A. (1999). Behavioral characterization of mdx3cv mice deficient in C-terminal dystrophins. Neuromuscular Disorders, 9(5), 296-304. https:\/\/doi.org\/10.1016\/s0960-8966(99)00029-2"},{"Citation":"Wang, D. D., & Bordey, A. (2008). The astrocyte odyssey. Progress in Neurobiology, 86(4), 342-367. https:\/\/doi.org\/10.1016\/j.pneurobio.2008.09.015"},{"Citation":"Wen, H., Nagelhus, E. A., Amiry-Moghaddam, M., Agre, P., Ottersen, O. P., & Nielsen, S. (1999). Ontogeny of water transport in rat brain: Postnatal expression of the aquaporin-4 water channel. The European Journal of Neuroscience, 11(3), 935-945. https:\/\/doi.org\/10.1046\/j.1460-9568.1999.00502.x"},{"Citation":"Wolburg-Buchholz, K., Mack, A. F., Steiner, E., Pfeiffer, F., Engelhardt, B., & Wolburg, H. (2009). Loss of astrocyte polarity marks blood-brain barrier impairment during experimental autoimmune encephalomyelitis. Acta Neuropathologica, 118(2), 219-233. https:\/\/doi.org\/10.1007\/s00401-009-0558-4"},{"Citation":"Woo, J., Kim, J. E., Im, J. J., Lee, J., Jeong, H. S., Park, S., \u2026 Lee, C. J. (2018). Astrocytic water channel aquaporin-4 modulates brain plasticity in both mice and humans: A potential gliogenetic mechanism underlying language-associated learning. Molecular Psychiatry, 23(4), 1021-1030. https:\/\/doi.org\/10.1038\/mp.2017.113"},{"Citation":"Yang, J., Lunde, L. K., Nuntagij, P., Oguchi, T., Camassa, L. M., Nilsson, L. N., \u2026 Torp, R. (2011). Loss of astrocyte polarization in the tg-ArcSwe mouse model of Alzheimer's disease. Journal of Alzheimer's Disease, 27(4), 711-722. https:\/\/doi.org\/10.3233\/JAD-2011-110725"}]}]}}}