{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T02:19:55Z","timestamp":1791080395351,"version":"4.1.0"},"reference-count":48,"publisher":"The Company of Biologists","issue":"Supplement_17","license":[{"start":{"date-parts":[[1993,12,1]],"date-time":"1993-12-01T00:00:00Z","timestamp":754704000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.biologists.com\/user-licence-1-1\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1993,12,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Motor neuron disease is clinically characterized by progressive muscle wasting leading to total muscle paralysis. A long history of pathological study of patients has firmly established that the primary lesion site is in spinal and cortical motor neurons. In addition to the widespread loss of these neurons, neuronal abnormalities including massive accumulation of neurofilaments in cell bodies and proximal axons have been also widely observed, particularly in the early stages of the disease. To test whether high accumulation of neurofilaments directly contributes to the pathogenic process, transgenic mice that produce high levels of neurofilaments in motor neurons have been generated. These transgenic mice show most of the hallmarks observed in motor neuron disease, including swollen perikarya with eccentrically localized nuclei, proximal axonal swellings, axonal degeneration and severe skeletal muscle atrophy. These data indicate that extensive accumulation of neurofilaments in motor neurons can trigger a neurodegenerative process and may be a key intermediate in the pathway of pathogenesis leading to neuronal loss.<\/jats:p>","DOI":"10.1242\/jcs.1993.supplement_17.15","type":"journal-article","created":{"date-parts":[[2013,5,23]],"date-time":"2013-05-23T12:48:12Z","timestamp":1369313292000},"page":"101-108","source":"Crossref","is-referenced-by-count":30,"title":["Involvement of neurofilaments in motor neuron disease"],"prefix":"10.1242","volume":"1993","author":[{"given":"Zuoshang","family":"Xu","sequence":"first","affiliation":[{"name":"Departments of Biological Chemistry, 725 North Wolfe Street, The Johns Hopkins University School of Medicine 1 , Baltimore, Maryland 21205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda C.","family":"Cork","sequence":"additional","affiliation":[{"name":"Departments of Comparative Medicine, 725 North Wolfe Street, The Johns Hopkins University School of Medicine 2 , Baltimore, Maryland 21205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John W.","family":"Griffin","sequence":"additional","affiliation":[{"name":"Departments of Neurology and 725 North Wolfe Street, The Johns Hopkins University School of Medicine 3 , Baltimore, Maryland 21205, USA"},{"name":"Neuroscience, 725 North Wolfe Street, The Johns Hopkins University School of Medicine 4 , Baltimore, Maryland 21205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Don W.","family":"Cleveland","sequence":"additional","affiliation":[{"name":"Departments of Biological Chemistry, 725 North Wolfe Street, The Johns Hopkins University School of Medicine 1 , Baltimore, Maryland 21205, USA"},{"name":"Neuroscience, 725 North Wolfe Street, The Johns Hopkins University School of Medicine 4 , Baltimore, Maryland 21205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[1993,12,1]]},"reference":[{"key":"2024071020071510400_JOCES_1993_S17_101C1","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1097\/00005072-198309000-00007","article-title":"The spatiotemporal pattern of the axonopathy associated wiht the neurotoxicity of 3, 4-dimethyl-2, 5-hexanedione in the rat.","volume":"42","author":"Anthony","year":"1983","journal-title":"J. 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