{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T13:46:59Z","timestamp":1790257619140,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,2,22]],"date-time":"2017-02-22T00:00:00Z","timestamp":1487721600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005758","name":"Universit\u00e0 Politecnica delle Marche","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005758","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2017,3]]},"abstract":"Abstract<\/jats:title>\n \n Introduction<\/jats:title>\n \n Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (\n CADASIL<\/jats:styled-content>\n ) is a hereditary systemic vascular disorder. Granular osmiophilic material (\n GOM<\/jats:styled-content>\n ) is its ultrastructural marker. We reviewed tissue biopsies from\n CADASIL<\/jats:styled-content>\n patients to establish whether ultrastructural observations help clarify the pathogenic mechanism of\n CADASIL<\/jats:styled-content>\n . Given the resemblance of the\n GOM<\/jats:styled-content>\n deposits to the immunoglobulin deposits seen in glomerulonephritis and focal segmental glomerulosclerosis (\n FSGS<\/jats:styled-content>\n ), their morphologies were investigated and compared.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Skin, skeletal muscle, kidney, and pericardium tissue biopsies from 13 patients with a clinical and molecular diagnosis of\n CADASIL<\/jats:styled-content>\n , and kidney biopsies from five patients with IgA nephropathy and five patients with primary\n FSGS<\/jats:styled-content>\n were subjected to ultrastructural examination.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n In\n CADASIL<\/jats:styled-content>\n patients, several\n GOM<\/jats:styled-content>\n deposits from all sites were partially or totally surrounded by an electron\u2010lucent halo. The deposits frequently had a more electron\u2010dense portion with a regular outline on the inner side and a less osmiophilic, looser outer side displaying a less regular profile. The uniformly dense deposits tended to be more osmiophilic if located close to the cell membrane and less osmiophilic if laid farther away from it. The immunoglobulin deposits from the glomerulonephritis and\n FSGS<\/jats:styled-content>\n patients lacked both the granular pattern and the halo.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n This study demonstrates that\n GOM<\/jats:styled-content>\n deposits may have a nonuniform morphology and describes in detail an electron\u2010lucent halo surrounding several of them. It is conceivable that the halo is the morphological evidence and possibly the cause of an aberrant\n NOTCH<\/jats:styled-content>\n 3 processing, already suspected to be involved in\n CADASIL<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/brb3.624","type":"journal-article","created":{"date-parts":[[2017,2,22]],"date-time":"2017-02-22T00:08:43Z","timestamp":1487722123000},"source":"Crossref","is-referenced-by-count":17,"title":["CADASIL<\/scp>\n : Ultrastructural insights into the morphology of granular osmiophilic material"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3723-5378","authenticated-orcid":false,"given":"Teresa","family":"Lorenzi","sequence":"first","affiliation":[{"name":"Section of Neuroscience and Cell Biology Department of Experimental and Clinical Medicine School of Medicine Universit\u00e0 Politecnica delle Marche Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Ragno","sequence":"additional","affiliation":[{"name":"Division of Neurology Madonna del Soccorso Hospital San Benedetto del Tronto Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Paolinelli","sequence":"additional","affiliation":[{"name":"Section of Neuroscience and Cell Biology Department of Experimental and Clinical Medicine School of Medicine Universit\u00e0 Politecnica delle Marche Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"Castellucci","sequence":"additional","affiliation":[{"name":"Division of Pathology Department of Clinical and Molecular Sciences School of Medicine Universit\u00e0 Politecnica delle Marche Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Scarpelli","sequence":"additional","affiliation":[{"name":"Section of Pathological Anatomy Department of Biomedical Sciences and Public Health School of Medicine Universit\u00e0 Politecnica delle Marche United Hospitals Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manrico","family":"Morroni","sequence":"additional","affiliation":[{"name":"Section of Neuroscience and Cell Biology Department of Experimental and Clinical Medicine School of Medicine Universit\u00e0 Politecnica delle Marche Ancona Italy"},{"name":"Electron Microscopy Unit United Hospitals Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,2,22]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1101964108"},{"key":"e_1_2_8_3_1","first-page":"879","article-title":"Aggregation of alpha\u2010synuclein in Lewy bodies of sporadic Parkinson's disease and dementia with Lewy bodies","volume":"152","author":"Baba M.","year":"1998","journal-title":"American Journal of 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