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Defining the contribution of these cells to disease can inform cell-type-specific effects and uncover new disease-modifying therapeutic targets. These cells express integrin (ITG) adhesion receptors that anchor the cells to the extracellular matrix (ECM) to maintain the integrity of the BBB. We used HD patient-derived induced pluripotent stem cell (iPSC) modeling to study the ECM\u2013ITG interface in astrocytes and brain microvascular endothelial cells and found ECM\u2013ITG dysregulation in human iPSC-derived cells that may contribute to the dysfunction of the BBB in HD. This disruption has functional consequences since reducing ITG expression in glia in an HD Drosophila model suppressed disease-associated CNS dysfunction. Since ITGs can be targeted therapeutically and manipulating ITG signaling prevents neurodegeneration in other diseases, defining the role of ITGs in HD may provide a novel strategy of intervention to slow CNS pathophysiology to treat HD.<\/jats:p>","DOI":"10.1093\/hmg\/ddac303","type":"journal-article","created":{"date-parts":[[2022,12,22]],"date-time":"2022-12-22T09:59:20Z","timestamp":1671703160000},"page":"1483-1496","source":"Crossref","is-referenced-by-count":15,"title":["An altered extracellular matrix\u2013integrin interface contributes to Huntington\u2019s disease-associated CNS dysfunction in glial and vascular cells"],"prefix":"10.1093","volume":"32","author":[{"given":"Sarah J","family":"Hernandez","sequence":"first","affiliation":[{"name":"University of California Irvine Department of Neurobiology and Behavior, , Irvine, CA 92697 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan G","family":"Lim","sequence":"additional","affiliation":[{"name":"University of California Irvine Institute for Memory Impairments and Neurological Disorders, , Irvine, CA 92697 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tarik","family":"Onur","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine Department of Molecular and Human Genetics, , Houston, TX , USA"},{"name":"Jan and Dan Duncan Neurological Research Institute at Texas Children\u2019s Hospital , Houston, TX , USA"},{"name":"Baylor College of Medicine Genetics & Genomics Graduate Program, , Houston, TX 77030 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark A","family":"Dane","sequence":"additional","affiliation":[{"name":"OHSU Department of Biomedical Engineering, , Portland, OR 97239 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Smith","sequence":"additional","affiliation":[{"name":"OHSU Department of Biomedical Engineering, , Portland, OR 97239 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keona","family":"Wang","sequence":"additional","affiliation":[{"name":"University of California Irvine Department of Neurobiology and Behavior, , Irvine, CA 92697 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grace En-Hway","family":"Jean","sequence":"additional","affiliation":[{"name":"University of California Department of Developmental and Cell Biology, , Irvine, CA 92697 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Reyes-Ortiz","sequence":"additional","affiliation":[{"name":"University of California Irvine Department of Biological Chemistry, , Irvine, CA 92697 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaylyn","family":"Devlin","sequence":"additional","affiliation":[{"name":"OHSU Department of Biomedical Engineering, , Portland, OR 97239 , 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USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura M","family":"Heiser","sequence":"additional","affiliation":[{"name":"OHSU Department of Biomedical Engineering, , Portland, OR 97239 , USA"},{"name":"OHSU Knight Cancer Institute , Portland, OR 97239 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James E","family":"Korkola","sequence":"additional","affiliation":[{"name":"OHSU Department of Biomedical Engineering, , Portland, OR 97239 , USA"},{"name":"OHSU Knight Cancer Institute , Portland, OR 97239 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Van Vactor","sequence":"additional","affiliation":[{"name":"Harvard Medical School Department of Cell Biology, , Boston, MA 02115 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Botas","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine Department of Molecular and Human Genetics, , Houston, TX , USA"},{"name":"Jan and Dan Duncan Neurological Research Institute at Texas Children\u2019s Hospital , Houston, TX , USA"},{"name":"Baylor College of Medicine Genetics & Genomics Graduate Program, , Houston, TX 77030 , USA"},{"name":"Baylor College of Medicine Quantitative & Computational Biosciences, , Houston, TX 77030 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine L","family":"Thompson-Peer","sequence":"additional","affiliation":[{"name":"University of California Department of Developmental and Cell Biology, , Irvine, CA 92697 , USA"},{"name":"University of California Reeve-Irvine Research Center, , Irvine, CA 92697 , USA"},{"name":"University of California Center for the Neurobiology of Learning and Memory, , Irvine, CA 92697 , USA"},{"name":"University of California Irvine Sue and Bill Gross Stem Cell Research Center, , Irvine, CA 92697 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie 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Proteome Res."},{"key":"2023042013363212500_ref30","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s40478-020-0880-6","article-title":"Single-nucleus RNA-seq identifies Huntington disease astrocyte states","volume":"8","author":"Al-Dalahmah","year":"2020","journal-title":"Acta Neuropathol. Commun."},{"key":"2023042013363212500_ref31","doi-asserted-by":"crossref","first-page":"3005","DOI":"10.1007\/s00216-022-03929-4","article-title":"Matrisome changes in Parkinson\u2019s disease","volume":"414","author":"Downs","year":"2022","journal-title":"Anal. Bioanal. Chem."},{"key":"2023042013363212500_ref32","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1038\/ncb0803-694","article-title":"Talin forges the links between integrins and actin","volume":"5","author":"Calderwood","year":"2003","journal-title":"Nat. Cell Biol."},{"key":"2023042013363212500_ref33","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1016\/S0166-2236(98)01298-3","article-title":"Perineuronal nets: past and present","volume":"21","author":"Celio","year":"1998","journal-title":"Trends Neurosci."},{"key":"2023042013363212500_ref34","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pcbi.0030039","article-title":"Discovering motifs in ranked lists of DNA sequences","volume":"3","author":"Eden","year":"2007","journal-title":"PLoS Comput. Biol."},{"key":"2023042013363212500_ref35","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/1471-2105-10-48","article-title":"GOrilla: a tool for discovery and visualization of enriched GO terms in ranked gene lists","volume":"10","author":"Eden","year":"2009","journal-title":"BMC Bioinform."},{"key":"2023042013363212500_ref36","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.nbd.2009.07.030","article-title":"Structure and function of the blood\u2013brain barrier","volume":"37","author":"Abbott","year":"2010","journal-title":"Neurobiol. Dis."},{"key":"2023042013363212500_ref37","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1016\/j.biocel.2011.05.002","article-title":"The impact of pericytes on the blood\u2013brain barrier integrity depends critically on the pericyte differentiation stage","volume":"43","author":"Thanabalasundaram","year":"2011","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"2023042013363212500_ref38","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1016\/S1534-5807(02)00188-0","article-title":"The IGF-1\/Akt pathway is neuroprotective in Huntington\u2019s disease and involves huntingtin phosphorylation by Akt","volume":"2","author":"Humbert","year":"2002","journal-title":"Dev. Cell"},{"key":"2023042013363212500_ref39","doi-asserted-by":"crossref","first-page":"2124","DOI":"10.1038\/emboj.2008.133","article-title":"Huntingtin phosphorylation acts as a molecular switch for anterograde\/retrograde transport in neurons","volume":"27","author":"Colin","year":"2008","journal-title":"EMBO J."},{"key":"2023042013363212500_ref40","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1523\/JNEUROSCI.3706-05.2006","article-title":"Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421","volume":"26","author":"Pardo","year":"2006","journal-title":"J. Neurosci."},{"key":"2023042013363212500_ref41","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1111\/j.0953-816X.2003.03131.x","article-title":"The serum-and glucocorticoid-induced kinase SGK inhibits mutant huntingtin-induced toxicity by phosphorylating serine 421 of huntingtin","volume":"19","author":"Rangone","year":"2004","journal-title":"Eur. J. Neurosci."},{"key":"2023042013363212500_ref42","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.mcn.2008.09.007","article-title":"Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments","volume":"40","author":"Warby","year":"2009","journal-title":"Mol. Cell. Neurosci."},{"key":"2023042013363212500_ref43","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a033993","article-title":"The autophagy lysosomal pathway and neurodegeneration","volume":"12","author":"Finkbeiner","year":"2020","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"2023042013363212500_ref44","doi-asserted-by":"crossref","DOI":"10.1016\/j.mbplus.2021.100089","article-title":"Cooperation of cell adhesion and autophagy in the brain: functional roles in development and neurodegenerative disease","volume":"12","author":"Hernandez","year":"2021","journal-title":"Matrix Biol. Plus"},{"key":"2023042013363212500_ref45","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.yexcr.2019.01.027","article-title":"Integrin signaling and mechanotransduction in regulation of somatic stem cells","volume":"378","author":"Isomursu","year":"2019","journal-title":"Exp. Cell Res."},{"key":"2023042013363212500_ref46","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.1701679","article-title":"Directed differentiation of human pluripotent stem cells to blood-brain barrier endothelial cells","volume":"3","author":"Qian","year":"2017","journal-title":"Sci. Adv."},{"key":"2023042013363212500_ref47","doi-asserted-by":"crossref","first-page":"25","DOI":"10.3233\/JHD-210502","article-title":"Diminished LC3-associated phagocytosis by Huntington\u2019s disease striatal astrocytes","volume":"11","author":"Wakida","year":"2022","journal-title":"J. Huntingtons Dis."},{"key":"2023042013363212500_ref48","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1006\/excr.2000.5040","article-title":"Multiple roles of integrins in cell motility","volume":"261","author":"Holly","year":"2000","journal-title":"Exp. Cell Res."},{"key":"2023042013363212500_ref49","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1111\/j.1469-7793.2001.0785e.x","article-title":"Integrin binding to fibronectin and vitronectin maintains the barrier function of isolated porcine coronary venules","volume":"532","author":"Wu","year":"2001","journal-title":"J. Physiol."},{"key":"2023042013363212500_ref50","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1177\/0271678X17722108","article-title":"\u03b21-integrin\u2013matrix interactions modulate cerebral microvessel endothelial cell tight junction expression and permeability","volume":"38","author":"Izawa","year":"2018","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"2023042013363212500_ref51","article-title":"Using microarrays to interrogate microenvironmental impact on cellular phenotypes in cancer","volume":"147","author":"Smith","year":"2019","journal-title":"J. Vis. Exp."},{"key":"2023042013363212500_ref52","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/j.cels.2018.02.001","article-title":"Microenvironment-mediated mechanisms of resistance to HER2 inhibitors differ between HER2+ breast cancer subtypes","volume":"6","author":"Watson","year":"2018","journal-title":"Cell Syst."},{"key":"2023042013363212500_ref53","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1016\/S0092-8674(00)81618-9","article-title":"Extensive vasculogenesis, angiogenesis, and organogenesis precede lethality in mice lacking all \u03b1v integrins","volume":"95","author":"Bader","year":"1998","journal-title":"Cell"},{"key":"2023042013363212500_ref54","doi-asserted-by":"crossref","first-page":"7667","DOI":"10.1128\/MCB.22.21.7667-7677.2002","article-title":"Defective associations between blood vessels and brain parenchyma lead to cerebral hemorrhage in mice lacking \u03b1v integrins","volume":"22","author":"McCarty","year":"2002","journal-title":"Mol. Cell. Biol."},{"key":"2023042013363212500_ref55","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1242\/dev.01551","article-title":"Selective ablation of \u03b1v integrins in the central nervous system leads to cerebral hemorrhage, seizures, axonal degeneration and premature death","volume":"132","author":"McCarty","year":"2005","journal-title":"Development"},{"key":"2023042013363212500_ref56","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1093\/ilar.48.4.356","article-title":"Animal models of Huntington\u2019s disease","volume":"48","author":"Ramaswamy","year":"2007","journal-title":"ILAR J."},{"key":"2023042013363212500_ref57","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.64564","article-title":"Downregulation of glial genes involved in synaptic function mitigates Huntington's disease pathogenesis","volume-title":"Elife","author":"Onur","year":"2021"},{"key":"2023042013363212500_ref58","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.cels.2018.05.010","article-title":"High-throughput functional analysis distinguishes pathogenic, nonpathogenic, and compensatory transcriptional changes in neurodegeneration","volume":"7","author":"Al-Ramahi","year":"2018","journal-title":"Cell Syst."},{"key":"2023042013363212500_ref59","doi-asserted-by":"crossref","first-page":"2204","DOI":"10.1126\/science.287.5461.2204","article-title":"Comparative genomics of the eukaryotes","volume":"287","author":"Rubin","year":"2000","journal-title":"Science"},{"key":"2023042013363212500_ref60","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1093\/hmg\/ddm315","article-title":"Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases","volume":"17","author":"Branco","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"2023042013363212500_ref61","doi-asserted-by":"crossref","first-page":"top067587","DOI":"10.1101\/pdb.top067587","article-title":"Analysis of glial cell development and function in Drosophila","volume":"2012","author":"Stork","year":"2012","journal-title":"Cold Spring Harb. Protoc."},{"key":"2023042013363212500_ref62","volume-title":"Madame Curie Bioscience Database","author":"Narasimha","year":"2013"},{"key":"2023042013363212500_ref63","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1126\/science.aax3338","article-title":"Huntington\u2019s disease alters human neurodevelopment","volume":"369","author":"Barnat","year":"2020","journal-title":"Science"},{"key":"2023042013363212500_ref64","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1002\/mds.25591","article-title":"Elevated arteriolar cerebral blood volume in prodromal Huntington's disease","volume":"29","author":"Hua","year":"2014","journal-title":"Mov. Disord."},{"key":"2023042013363212500_ref65","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1212\/WNL.36.2.244","article-title":"Huntington\u2019s disease in Venezuela: neurologic features and functional decline","volume":"36","author":"Young","year":"1986","journal-title":"Neurology"},{"key":"2023042013363212500_ref66","doi-asserted-by":"crossref","first-page":"1783","DOI":"10.1161\/STROKEAHA.110.586537","article-title":"Mechanisms of osteopontin-induced stabilization of blood-brain barrier disruption after subarachnoid hemorrhage in rats","volume":"41","author":"Suzuki","year":"2010","journal-title":"Stroke"},{"key":"2023042013363212500_ref67","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1002\/ana.22102","article-title":"Recombinant osteopontin in cerebral vasospasm after subarachnoid hemorrhage","volume":"68","author":"Suzuki","year":"2010","journal-title":"Ann. Neurol."},{"key":"2023042013363212500_ref68","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuron.2022.02.017","article-title":"Pericyte-to-endothelial cell signaling via vitronectin-integrin regulates blood-CNS barrier","volume":"110","author":"Ayloo","year":"2022","journal-title":"Neuron"},{"key":"2023042013363212500_ref69","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1002\/glia.23115","article-title":"The glia of the adult Drosophila nervous system","volume":"65","author":"Kremer","year":"2017","journal-title":"Glia"},{"key":"2023042013363212500_ref70","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1038\/nn.4532","article-title":"Developmental alterations in Huntington\u2019s disease neural cells and pharmacological rescue in cells and mice","volume":"20","author":"Consortium","year":"2017","journal-title":"Nat. Neurosci."},{"key":"2023042013363212500_ref71","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1080\/10618600.2020.1741379","article-title":"Automatic transformation and integration to improve visualization and discovery of latent effects in imaging data","volume":"29","author":"Hunt","year":"2020","journal-title":"J. Comput. Graph. 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