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Known genetic causes of PFBC include loss\u2010of\u2010function mutations in genes involved in either of three processes\u2014platelet\u2010derived growth factor (PDGF) signaling, phosphate homeostasis or protein glycosylation\u2014with unclear molecular links. To provide insight into the pathogenesis of PFBC, we analyzed murine models of PFBC for the first two of these processes in\n                    <jats:italic>\n                      Pdgfb\n                      <jats:sup>ret\/ret<\/jats:sup>\n                    <\/jats:italic>\n                    and\n                    <jats:italic>Slc20a2<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice with regard to the structure, molecular composition, development and distribution of perivascular calcified nodules. Analyses by transmission electron microscopy and immunofluorescence revealed that calcified nodules in both of these models have a multilayered ultrastructure and occur in direct contact with reactive astrocytes and microglia. However, whereas nodules in\n                    <jats:italic>\n                      Pdgfb\n                      <jats:sup>ret\/ret<\/jats:sup>\n                    <\/jats:italic>\n                    mice were large, solitary and smooth surfaced, the nodules in\n                    <jats:italic>Slc20a2<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice were multi\u2010lobulated and occurred in clusters. The regional distribution of nodules also differed between the two models. Proteomic analysis and immunofluorescence stainings revealed a common molecular composition of the nodules in the two models, involving proteins implicated in bone homeostasis, but also proteins not previously linked to tissue mineralization. While the brain vasculature of\n                    <jats:italic>\n                      Pdgfb\n                      <jats:sup>ret\/ret<\/jats:sup>\n                    <\/jats:italic>\n                    mice has been reported to display reduced pericyte coverage and abnormal permeability, we found that\n                    <jats:italic>Slc20a2<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice have a normal pericyte coverage and no overtly increased permeability.\u00a0Thus, lack of pericytes and increase in permeability of the blood\u2013brain barrier are likely not the causal triggers for PFBC pathogenesis. Instead, gene expression and spatial correlations suggest that astrocytes are intimately linked to the calcification process in PFBC.\n                  <\/jats:p>","DOI":"10.1111\/bpa.12787","type":"journal-article","created":{"date-parts":[[2019,9,27]],"date-time":"2019-09-27T23:55:34Z","timestamp":1569628534000},"page":"446-464","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Astrocyte\u2013microglial association and matrix composition are common events in the natural history of primary familial brain calcification"],"prefix":"10.1111","volume":"30","author":[{"given":"Khayrun","family":"Nahar","sequence":"first","affiliation":[{"name":"Department of Immunology, Genetics and Pathology Uppsala University Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thibaud","family":"Lebouvier","sequence":"additional","affiliation":[{"name":"Department of Immunology, Genetics and Pathology 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