{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:39:19Z","timestamp":1791329959470,"version":"4.3.1"},"reference-count":34,"publisher":"National Academy of Sciences","issue":"30","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2007,7,24]]},"abstract":"<jats:p>Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron degenerative disease whose etiology and pathogenesis remain poorly understood. Most cases of ALS (\u224890%) are sporadic (SALS), occurring in the absence of genetic associations. Approximately 20% of familial ALS (FALS) cases are due to known mutations in the copper, zinc superoxide dismutase (SOD1) gene. Molecular evidence for a common pathogenesis of SALS and FALS has remained elusive. Here we use covalent chemical modification to reveal an attribute of spinal cord SOD1 common to both SOD1-linked FALS and SALS, but not present in normal or disease-affected tissues from other neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's diseases and spinal muscular atrophy, a non-ALS motor neuron disease. Biotinylation reveals a 32-kDa, covalently cross-linked SOD1-containing protein species produced not only in FALS caused by SOD1 mutation, but also in SALS. These studies use chemical modification as a novel tool for the detection of a disease-associated biomarker. Our results identify a shared molecular event involving a known target gene and suggest a common step in the pathogenesis between SALS and FALS.<\/jats:p>","DOI":"10.1073\/pnas.0705044104","type":"journal-article","created":{"date-parts":[[2007,7,16]],"date-time":"2007-07-16T20:44:52Z","timestamp":1184618692000},"page":"12524-12529","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":156,"title":["Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis"],"prefix":"10.1073","volume":"104","author":[{"given":"Arie","family":"Gruzman","sequence":"first","affiliation":[{"name":"*Bioconformatics Laboratory and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William L.","family":"Wood","sequence":"additional","affiliation":[{"name":"Department of Chemistry, Natural Sciences Complex, University at Buffalo, State University of New York, Buffalo, NY 14260;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evgenia","family":"Alpert","sequence":"additional","affiliation":[{"name":"*Bioconformatics Laboratory and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Dharma","family":"Prasad","sequence":"additional","affiliation":[{"name":"*Bioconformatics Laboratory and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert G.","family":"Miller","sequence":"additional","affiliation":[{"name":"Department of Neurology, California Pacific Medical Center, San Francisco, CA 94115;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffery D.","family":"Rothstein","sequence":"additional","affiliation":[{"name":"Department of Neurology, Johns Hopkins University, Baltimore, MD 21287;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Bowser","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald","family":"Hamilton","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Troy D.","family":"Wood","sequence":"additional","affiliation":[{"name":"Department of Chemistry, Natural Sciences Complex, University at Buffalo, State University of New York, Buffalo, NY 14260;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Don W.","family":"Cleveland","sequence":"additional","affiliation":[{"name":"Ludwig Institute for Cancer Research, Departments of Medicine and Neuroscience, University of California at San Diego, La Jolla, CA 92093; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vishwanath R.","family":"Lingappa","sequence":"additional","affiliation":[{"name":"*Bioconformatics Laboratory and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, California Pacific Medical Center Research Institute, San Francisco, CA 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