{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:05:16Z","timestamp":1792785916093,"version":"4.5.0"},"reference-count":34,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2021,12,17]],"date-time":"2021-12-17T00:00:00Z","timestamp":1639699200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P50AR065139"],"award-info":[{"award-number":["P50AR065139"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["U01NS101944"],"award-info":[{"award-number":["U01NS101944"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"MDA Clinical Research Network","award":["573783"],"award-info":[{"award-number":["573783"]}]},{"DOI":"10.13039\/100009824","name":"FSH Society","doi-asserted-by":"publisher","award":["52016"],"award-info":[{"award-number":["52016"]}],"id":[{"id":"10.13039\/100009824","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Chris Carinno Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,6,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Advances in understanding the pathophysiology of facioscapulohumeral dystrophy (FSHD) have led to several therapeutic approaches entering clinical trials and an increased need to develop biomarkers of disease activity and progression. Multiple prior studies have shown early elevation of RNAs encoding components of the complement pathways and relatively widespread activated complement complexes by immunodetection in FSHD muscle. The current study tested plasma from two independent cohorts of FSHD and control subjects and found elevated complement components in both FSHD cohorts. Combining subjects from both cohorts identified complement factors that best distinguished FSHD and controls. Within the FSHD group, a subset of subjects showed elevation in multiple complement components. Together these findings suggest the need for future studies to determine whether measurements of complement activation can be used as a non-invasive measurement of FSHD disease activity, progression and\/or response to therapies. In addition, with the ongoing expansion of complement therapeutic approaches, consideration for precision-based targeting of this pathway is appropriate.<\/jats:p>","DOI":"10.1093\/hmg\/ddab364","type":"journal-article","created":{"date-parts":[[2021,12,15]],"date-time":"2021-12-15T23:17:26Z","timestamp":1639610246000},"page":"1821-1829","source":"Crossref","is-referenced-by-count":24,"title":["Elevated plasma complement components in facioscapulohumeral dystrophy"],"prefix":"10.1093","volume":"31","author":[{"given":"Chao-Jen","family":"Wong","sequence":"first","affiliation":[{"name":"Division of Human Biology, Fred Hutchinson Cancer Research Center , Seattle, WA 98109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leo","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology , , Seattle, WA 98105 , USA"},{"name":"University of Washington , , Seattle, WA 98105 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V Michael","family":"Holers","sequence":"additional","affiliation":[{"name":"Division of Rheumatology , , Aurora, CO 80045 , USA"},{"name":"University of Colorado School of Medicine , , Aurora, CO 80045 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Frazer-Abel","sequence":"additional","affiliation":[{"name":"Exsera BioLabs , Division of Rheumatalogy, , Aurora, CO 80045 , USA"},{"name":"University of Colorado School of Medicine , Division of Rheumatalogy, , Aurora, CO 80045 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silv\u00e8re M","family":"van der Maarel","sequence":"additional","affiliation":[{"name":"Department of Human Genetics , , Leiden 2333 , The Netherlands"},{"name":"Leiden University Medical Center , , Leiden 2333 , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabi","family":"Tawil","sequence":"additional","affiliation":[{"name":"Department of Neurology , , Rochester, NY 14642 , USA"},{"name":"University of Rochester Medical Center , , Rochester, NY 14642 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey M","family":"Statland","sequence":"additional","affiliation":[{"name":"Department of Neurology , , Kansas City, KA 66160 , USA"},{"name":"University of Kansas Medical Center , , Kansas City, KA 66160 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0319-0968","authenticated-orcid":false,"given":"Stephen J","family":"Tapscott","sequence":"additional","affiliation":[{"name":"Division of Human Biology, Fred Hutchinson Cancer Research Center , Seattle, WA 98109 , USA"},{"name":"Department of Neurology , , Seattle, WA 98105 , USA"},{"name":"University of Washington , , Seattle, WA 98105 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"ReSolve Network","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,12,17]]},"reference":[{"key":"2022060611293919800_ref1","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1186\/2044-5040-4-12","article-title":"Facioscapulohumeral dystrophy: the path to consensus on pathophysiology","volume":"4","author":"Tawil","year":"2014","journal-title":"Skelet. 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