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However, there is currently an incomplete understanding about the function of the multiple dystrophin isoforms in human brains. Here, we tested the hypothesis that dystrophin deficiency affects glial function in DMD and could therefore contribute to neural impairment. We investigated human dystrophin isoform expression with development and differentiation and response to damage in human astrocytes from control and induced pluripotent stem cells from DMD patients. In control cells, short dystrophin isoforms were up\u2010regulated with development and their expression levels changed differently upon neuronal and astrocytic differentiation, as well as in 2\u2010dimensional versus 3\u2010dimensional astrocyte cultures. All DMD\u2010astrocytes tested displayed altered morphology, proliferative activity and AQP4 expression. Furthermore, they did not show any morphological change in response to inflammatory stimuli and their number was significantly lower as compared to stimulated healthy astrocytes. Finally, DMD\u2010astrocytes appeared to be more sensitive than controls to oxidative damage as shown by their increased cell death. Behavioral and metabolic defects in DMD\u2010astrocytes were consistent with gene pathway dysregulation shared by lines with different mutations as demonstrated by bulk RNA\u2010seq analysis. Together, our DMD model provides evidence for altered astrocyte function in DMD suggesting that defective astrocyte responses may contribute to neural impairment and might provide additional potential therapeutic targets.<\/jats:p>","DOI":"10.1002\/glia.24116","type":"journal-article","created":{"date-parts":[[2021,11,13]],"date-time":"2021-11-13T08:27:02Z","timestamp":1636792022000},"page":"466-490","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Dystrophin deficiency affects human astrocyte properties and response to damage"],"prefix":"10.1002","volume":"70","author":[{"given":"Jenny","family":"Lange","sequence":"first","affiliation":[{"name":"Department of Developmental Biology and Cancer, Stem Cells and Regenerative Medicine Section UCL Great Ormond Street Institute of Child Health, University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivia","family":"Gillham","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology and Cancer, Stem Cells and Regenerative Medicine Section UCL Great Ormond Street Institute of Child Health, University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reem","family":"Alkharji","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology and Cancer, Stem Cells and Regenerative Medicine Section UCL Great Ormond Street Institute of Child Health, University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Eaton","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology and Cancer, Stem Cells and Regenerative Medicine Section UCL Great Ormond Street Institute of Child Health, University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Ferrari","sequence":"additional","affiliation":[{"name":"Department of Cell and Developmental Biology University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monika","family":"Madej","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases UCL Queen Square Institute of Neurology  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Flower","sequence":"additional","affiliation":[{"name":"UCL Queen Square Institute of Neurology, University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco Saverio","family":"Tedesco","sequence":"additional","affiliation":[{"name":"Department of Cell and Developmental Biology University College London  London UK"},{"name":"Dubowitz Neuromuscular Centre UCL Great Ormond Street Institute of Child Health, University College London  London UK"},{"name":"The Francis Crick Institute, 1 Midland Road  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Muntoni","sequence":"additional","affiliation":[{"name":"Dubowitz Neuromuscular Centre UCL Great Ormond Street Institute of Child Health, University College London  London UK"},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, Great Ormond Street Institute of Child Health University College London, &amp; 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