A novel human iPSC model of COL4A1/A2 small vessel disease unveils a key pathogenic role of matrix metalloproteinases
Al-Thani M, Goodwin-Trotman M, Bell S, Patel K, Fleming L, Vilain C, Abramowicz M, Allan S, Wang T, Cader M, Horsburgh K, Van Agtmael T, Sinha S, Markus H, Granata A. A novel human iPSC model of COL4A1/A2 small vessel disease unveils a key pathogenic role of matrix metalloproteinases. Stem Cell Reports 2023, 18: 2386-2399. PMID: 37977146, PMCID: PMC10724071, DOI: 10.1016/j.stemcr.2023.10.014.Peer-Reviewed Original ResearchConceptsSmall vessel diseaseMatrix metalloproteinasesVessel diseaseCerebral small vessel diseaseKey pathogenic roleEndothelial cell tight junctionsHuman iPSC modelSVD pathologyCo-culture modelLeading causePathogenic roleCell phenotypic changesExtracellular matrixParacrine actionCell tight junctionsMMP activityTherapeutic opportunitiesECM abnormalitiesCell defectsEndothelial cellsMural cellsSmall vesselsTight junctionsIPSC modelsDisease
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