{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T14:59:32Z","timestamp":1790521172629,"version":"4.1.0"},"reference-count":29,"publisher":"Rockefeller University Press","issue":"6","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2007,12,17]]},"abstract":"<jats:p>Increasing evidence suggests that neurotoxicity of secreted superoxide dismutase 1 (SOD1) mutants is associated with amyotrophic lateral sclerosis (ALS). We show here that mutant SOD1 protein activates microglia via a myeloid differentiation factor 88 (MyD88)\u2013dependent pathway. This inflammatory response is also associated with a marked recruitment of bone marrow\u2013derived microglia (BMDM) in the central nervous system. We then generated chimeric SOD1G37R and SOD1G93A mice by transplantation of bone marrow (BM) cells from MyD88-deficient or green fluorescent protein (GFP)\u2013expressing mice. SOD1G37R mice receiving MyD88\u2212\/\u2212 BM cells exhibit a significantly earlier disease onset and shorter lifespan compared with mice transplanted with control GFP cells. This compelling beneficial effect of MyD88-competent BMDM is a previously unrecognized natural innate immune mechanism of neuroprotection in a mouse model of late-onset motor neuron disease.<\/jats:p>","DOI":"10.1083\/jcb.200705046","type":"journal-article","created":{"date-parts":[[2007,12,17]],"date-time":"2007-12-17T20:43:29Z","timestamp":1197924209000},"page":"1219-1230","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":84,"title":["MyD88-deficient bone marrow cells accelerate onset and reduce survival in a mouse model of amyotrophic lateral sclerosis"],"prefix":"10.1083","volume":"179","author":[{"given":"Jihong","family":"Kang","sequence":"first","affiliation":[{"name":"Laboratory of Molecular Endocrinology, Centre hospitalier de l'Universite\u0301 Laval Research Center and Department of Anatomy and Physiology, Laval University, Que\u0301bec G1V 4G2, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serge","family":"Rivest","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Endocrinology, Centre hospitalier de l'Universite\u0301 Laval Research Center and Department of Anatomy and Physiology, Laval University, Que\u0301bec G1V 4G2, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2007,12,17]]},"reference":[{"key":"2023072909300745200_BIB1","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/S1074-7613(00)80596-8","volume":"9","year":"1998","journal-title":"Immunity."},{"key":"2023072909300745200_BIB2","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1016\/j.cell.2006.02.015","volume":"124","year":"2006","journal-title":"Cell."},{"key":"2023072909300745200_BIB3","doi-asserted-by":"crossref","first-page":"16021","DOI":"10.1073\/pnas.0607423103","volume":"103","year":"2006","journal-title":"Proc. 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