{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T02:53:07Z","timestamp":1772160787150,"version":"3.50.1"},"reference-count":38,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,5,22]],"date-time":"2025-05-22T00:00:00Z","timestamp":1747872000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100014440","name":"Ministerio de Ciencia, Innovaci\u00f3n y Universidades","doi-asserted-by":"publisher","award":["PID2021\u2010125041OB\u2010I00"],"award-info":[{"award-number":["PID2021\u2010125041OB\u2010I00"]}],"id":[{"id":"10.13039\/100014440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","award":["FPU21\/00912"],"award-info":[{"award-number":["FPU21\/00912"]}],"id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003989","name":"Ikerbasque, Basque Foundation for Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003989","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI22\/01382"],"award-info":[{"award-number":["PI22\/01382"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>This study aims to develop a quantitative method for assessing collagen VI expression in cell cultures, which is crucial for the diagnosis and treatment of collagen VI\u2010related dystrophies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We developed a combined in\u2010cell western (ICW) and on\u2010cell western (OCW) assay, which we have called \u2018collablot\u2019, to quantify collagen VI and its organisation in the extracellular matrix of cell cultures from patients and healthy controls. To optimise it, we optimised cell density and the protocols to induce collagen expression in cultures, as well as the cell fixation and permeabilisation methods. This was completed with a thorough selection of collagen antibodies and a collagen\u2010hybridising peptide (CHP). We then used collablots to compare cultures from patients and controls and evaluate therapeutic interventions in the cultures.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Collablots enabled the quantification of collagen VI expression in both control and patient cells, aligning with immunocytochemistry findings and detecting variations in collagen VI expression following treatment of the cultures. Additionally, CHP analysis revealed a marked increase in collagen network disruption in patients compared to the controls.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The collablot assay represents a suitable method for quantifying collagen VI expression and its organisation in culture and assessing the effect of therapies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/nan.70020","type":"journal-article","created":{"date-parts":[[2025,5,22]],"date-time":"2025-05-22T03:49:34Z","timestamp":1747885774000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Collablots: Quantification of Collagen VI Levels and Its Structural Disorganisation in Cell Cultures From Patients With Collagen VI\u2010Related Dystrophies"],"prefix":"10.1111","volume":"51","author":[{"given":"Nadia","family":"Osegui\u2010Barcenilla","sequence":"first","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Sendino","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sergio","family":"Mart\u00edn\u2010Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Itziar","family":"Gonz\u00e1lez\u2010Moro","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ainhoa","family":"Benito\u2010Agustino","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Noemi","family":"Torres\u2010Conde","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"L\u00f3pez\u2010Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cecilia","family":"Jim\u00e9nez\u2010Mallebrera","sequence":"additional","affiliation":[{"name":"Laboratory of Applied Research in Neuromuscular Diseases, Neuromuscular Pathology Unit, Neuropediatric Service Institut de Recerca Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"},{"name":"Rare Diseases Network Biomedical Research Center (CIBERER)  Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ar\u00edstides","family":"L\u00f3pez\u2010M\u00e1rquez","sequence":"additional","affiliation":[{"name":"Laboratory of Applied Research in Neuromuscular Diseases, Neuromuscular Pathology Unit, Neuropediatric Service Institut de Recerca Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"},{"name":"Rare Diseases Network Biomedical Research Center (CIBERER)  Madrid Spain"},{"name":"Department of Genetics, Microbiology and Statistics Faculty of Biology, University of Barcelona  Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7703-3255","authenticated-orcid":false,"given":"Virginia","family":"Arechavala\u2010Gomeza","sequence":"additional","affiliation":[{"name":"Nucleic Acid Therapeutics for Rare Disorders (NAT\u2010RD) Biobizkaia Health Research Institute  Barakaldo Spain"},{"name":"Ikerbasque, Basque Foundation for Science  Bilbao Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,5,22]]},"reference":[{"key":"e_1_2_11_2_1","unstructured":"A. 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