{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T19:03:21Z","timestamp":1792782201936,"version":"4.5.0"},"reference-count":50,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2014,10,26]],"date-time":"2014-10-26T00:00:00Z","timestamp":1414281600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Spanish Ministry of Health","award":["FIS-PI080894"],"award-info":[{"award-number":["FIS-PI080894"]}]},{"name":"Spanish Ministry of Health","award":["FIS-PI11-01418"],"award-info":[{"award-number":["FIS-PI11-01418"]}]},{"DOI":"10.13039\/100008054","name":"Fundaci\u00f3n Ram\u00f3n Areces","doi-asserted-by":"crossref","award":["2012-2015"],"award-info":[{"award-number":["2012-2015"]}],"id":[{"id":"10.13039\/100008054","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To define and characterize the progression of the spontaneous autoimmune disease that develops in mice in the absence of the leukocyte adhesion receptor P\u2010selectin glycoprotein ligand 1 (PSGL\u20101).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Skin\u2010resident immune cells from PSGL\u20101\u2013deficient mice and C57BL\/6 control mice of different ages were isolated and analyzed by flow cytometry. Biochemical parameters were analyzed in mouse serum and urine, and the presence of serum autoantibodies was investigated. Skin and internal organs were extracted, and their structure was analyzed histologically.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Skin\u2010resident innate and adaptive immune cells from PSGL\u20101\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      mice had a proinflammatory phenotype with an imbalanced T effector cell:Treg cell ratio. Sera from PSGL\u20101\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      mice had circulating autoantibodies commonly detected in connective tissue\u2013related human autoimmune diseases. Biochemical and histologic analysis of skin and internal organs revealed skin fibrosis and structural and functional abnormalities in the lungs and kidneys. Furthermore, PSGL\u20101\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      mice exhibited vascular alterations, showing loss of dermal vessels, small vessel medial layer remodeling in the lungs and kidneys, and ischemic processes in the kidney that promote renal infarcts.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our study demonstrates that immune system overactivation due to PSGL\u20101 deficiency triggers an autoimmune syndrome with characteristics similar to systemic sclerosis, including skin fibrosis, vascular alterations, and systemic organ involvement. These results suggest that PSGL\u20101 expression contributes to the maintenance of the homeostasis of the immune system and could act as a barrier for autoimmunity in mice.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.38808","type":"journal-article","created":{"date-parts":[[2014,8,6]],"date-time":"2014-08-06T15:52:11Z","timestamp":1407340331000},"page":"3178-3189","source":"Crossref","is-referenced-by-count":38,"title":["Development of an Autoimmune Syndrome Affecting the Skin and Internal Organs in P\u2010Selectin Glycoprotein Ligand 1 Leukocyte Receptor\u2013Deficient Mice"],"prefix":"10.1002","volume":"66","author":[{"given":"A.","family":"P\u00e9rez\u2010Fr\u00edas","sequence":"first","affiliation":[{"name":"Fundaci\u00f3n de Investigaci\u00f3n Biom\u00e9dica, Instituto de Investigaci\u00f3n Sanitaria\u2010Princesa, and Hospital de la Princesa Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Gonz\u00e1lez\u2010Tajuelo","sequence":"additional","affiliation":[{"name":"Fundaci\u00f3n de Investigaci\u00f3n Biom\u00e9dica, Instituto de Investigaci\u00f3n Sanitaria\u2010Princesa, and Hospital de la Princesa Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"N\u00fa\u00f1ez\u2010Andrade","sequence":"additional","affiliation":[{"name":"Fundaci\u00f3n de Investigaci\u00f3n Biom\u00e9dica, Instituto de Investigaci\u00f3n Sanitaria\u2010Princesa, and Hospital de la Princesa Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Tejedor","sequence":"additional","affiliation":[{"name":"Fundaci\u00f3n de Investigaci\u00f3n Biom\u00e9dica, Instituto de Investigaci\u00f3n Sanitaria\u2010Princesa, and Hospital de la Princesa Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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