{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:01:37Z","timestamp":1790834497721,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,10,30]],"date-time":"2020-10-30T00:00:00Z","timestamp":1604016000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000971","name":"Amyotrophic Lateral Sclerosis Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000971","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>An orally administered, fixed\u2010dose coformulation of sodium phenylbutyrate\u2010taurursodiol (PB\u2010TURSO) significantly slowed functional decline in a randomized, placebo\u2010controlled, phase 2 trial in ALS (CENTAUR). Herein we report results of a long\u2010term survival analysis of participants in CENTAUR. In CENTAUR, adults with ALS were randomized 2:1 to PB\u2010TURSO or placebo. Participants completing the 6\u2010month (24\u2010week) randomized phase were eligible to receive PB\u2010TURSO in the open\u2010label extension. An all\u2010cause mortality analysis (35\u2010month maximum follow\u2010up post\u2010randomization) incorporated all randomized participants. Participants and site investigators were blinded to treatment assignments through the duration of follow\u2010up of this analysis. Vital status was obtained for 135 of 137 participants originally randomized in CENTAUR. Median overall survival was 25.0 months among participants originally randomized to PB\u2010TURSO and 18.5 months among those originally randomized to placebo (hazard ratio, 0.56; 95% confidence interval, 0.34\u20100.92; <jats:italic>P<\/jats:italic> = .023). Initiation of PB\u2010TURSO treatment at baseline resulted in a 6.5\u2010month longer median survival as compared with placebo. Combined with results from CENTAUR, these results suggest that PB\u2010TURSO has both functional and survival benefits in ALS.<\/jats:p>","DOI":"10.1002\/mus.27091","type":"journal-article","created":{"date-parts":[[2020,10,16]],"date-time":"2020-10-16T14:19:07Z","timestamp":1602857947000},"page":"31-39","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":183,"title":["Long\u2010term survival of participants in the <scp>CENTAUR<\/scp> trial of sodium phenylbutyrate\u2010taurursodiol in <scp>amyotrophic lateral sclerosis<\/scp>"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0505-1168","authenticated-orcid":false,"given":"Sabrina","family":"Paganoni","sequence":"first","affiliation":[{"name":"Sean M. 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Healey &amp; AMG Center for ALS &amp; the Neurological Clinical Research Institute Massachusetts General Hospital, Harvard Medical School  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Elliott","sequence":"additional","affiliation":[{"name":"Swedish Neuroscience Institute  Seattle Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel","family":"Maiser","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Medicine Hennepin Healthcare  Minneapolis Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chafic","family":"Karam","sequence":"additional","affiliation":[{"name":"Department of Neurology Oregon Health &amp; Science University  Portland Oregon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James B.","family":"Caress","sequence":"additional","affiliation":[{"name":"Department of Neurology Wake Forest School of Medicine  Winston\u2010Salem North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret Ayo","family":"Owegi","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Massachusetts Memorial Medical Center  Worcester Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Quick","sequence":"additional","affiliation":[{"name":"Department of Neurology The Ohio State University College of Medicine  Columbus Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Wymer","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Florida College of Medicine  Gainesville Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8780-6637","authenticated-orcid":false,"given":"Stephen A.","family":"Goutman","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Michigan  Ann Arbor Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daragh","family":"Heitzman","sequence":"additional","affiliation":[{"name":"Texas Neurology  Dallas Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry D.","family":"Heiman\u2010Patterson","sequence":"additional","affiliation":[{"name":"Department of Neurology, Lewis Katz School of Medicine Temple University  Philadelphia Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlayne E.","family":"Jackson","sequence":"additional","affiliation":[{"name":"Department of Neurology The University of Texas Health Science Center at San Antonio  San Antonio Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin","family":"Quinn","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey D.","family":"Rothstein","sequence":"additional","affiliation":[{"name":"Brain Science Institute and Department of Neurology Johns Hopkins University  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward J.","family":"Kasarskis","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Kentucky College of Medicine  Lexington Kentucky"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Katz","sequence":"additional","affiliation":[{"name":"California Pacific Medical Center Research Institute and Forbes Norris MDA\/ALS Research and Treatment Center  San Francisco California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liberty","family":"Jenkins","sequence":"additional","affiliation":[{"name":"California Pacific Medical Center Research Institute and Forbes Norris MDA\/ALS Research and Treatment Center  San Francisco California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shafeeq","family":"Ladha","sequence":"additional","affiliation":[{"name":"Department of Neurology, Gregory W. 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