{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T23:13:40Z","timestamp":1793229220049,"version":"4.5.0"},"reference-count":34,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2004,2,1]],"date-time":"2004-02-01T00:00:00Z","timestamp":1075593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Experimental Neurology"],"published-print":{"date-parts":[[2004,2]]},"DOI":"10.1016\/j.expneurol.2003.10.004","type":"journal-article","created":{"date-parts":[[2004,1,16]],"date-time":"2004-01-16T05:15:36Z","timestamp":1074230136000},"page":"232-240","source":"Crossref","is-referenced-by-count":1235,"title":["Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man"],"prefix":"10.1016","volume":"185","author":[{"given":"Lindsey R.","family":"Fischer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah G.","family":"Culver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Tennant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert A.","family":"Davis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minsheng","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amilcar","family":"Castellano-Sanchez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaffar","family":"Khan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meraida A.","family":"Polak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Glass","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.expneurol.2003.10.004_BIB34","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1001\/archneur.1984.04050170073021","article-title":"Quantitative axon terminal and end-plate morphology in amyotrophic lateral sclerosis","volume":"41","author":"Bjornskov","year":"1984","journal-title":"Arch. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB1","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1083\/jcb.200208001","article-title":"Missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy, a model of human motoneuron disease","volume":"159","author":"Bommel","year":"2002","journal-title":"J. Cell Biol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB2","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1002\/ana.410140304","article-title":"Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis","volume":"14","author":"Bradley","year":"1983","journal-title":"Ann. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB3","first-page":"147","article-title":"Drug therapy for amyotrophic lateral sclerosis: where are we now?","volume":"6","author":"Carter","year":"2003","journal-title":"Investig. Drugs J"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB4","first-page":"219","article-title":"The significance of the \u201cdying back\u201d process in human and experimental neurological diseases","volume":"3","author":"Cavanagh","year":"1964","journal-title":"Int. Rev. Exp. Pathol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB5","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1006\/mcne.1995.1027","article-title":"Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis","volume":"6","author":"Chiu","year":"1995","journal-title":"Mol. Cell. Neurosci"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB6","doi-asserted-by":"crossref","first-page":"1439","DOI":"10.1093\/hmg\/11.12.1439","article-title":"Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model","volume":"11","author":"Cifuentes-Dias","year":"2002","journal-title":"Hum. Mol. Genet"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB7","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1002\/(SICI)1096-9861(19960101)364:1<6::AID-CNE2>3.0.CO;2-9","article-title":"Methods for determining numbers of cells and synapses: a case for more uniform standards of review","volume":"364","author":"Coggeshall","year":"1996","journal-title":"J. Comp. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB8","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1016\/S0166-2236(02)02255-5","article-title":"Axon pathology in neurological disease: a neglected therapeutic target","volume":"25","author":"Coleman","year":"2002","journal-title":"Trends Neurosci"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB9","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1006\/dbio.1994.1234","article-title":"Neurites can remain viable after destruction of the neuronal soma by programmed cell death (apoptosis)","volume":"165","author":"Deckwerth","year":"1994","journal-title":"Dev. Biol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB10","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1002\/ana.10374","article-title":"Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS","volume":"52","author":"Drachman","year":"2002","journal-title":"Ann. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB11","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1016\/S0960-9822(03)00206-9","article-title":"Inhibiting axon degeneration and synapse loss attenuates apoptosis and disease progression in a mouse model of motoneuron disease","volume":"13","author":"Ferri","year":"2003","journal-title":"Curr. Biol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB12","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1523\/JNEUROSCI.20-04-01333.2000","article-title":"Evidence that Wallerian degeneration and localized axon degeneration induced by local neurotrophin deprivation do not involve caspases","volume":"20","author":"Finn","year":"2000","journal-title":"J. Neurosci"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB13","doi-asserted-by":"crossref","first-page":"2534","DOI":"10.1523\/JNEUROSCI.20-07-02534.2000","article-title":"Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases","volume":"20","author":"Frey","year":"2000","journal-title":"J. Neurosci"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB14","first-page":"1075","article-title":"Pathophysiology of nerve and root disorders","volume":"vol. II","author":"Glass","year":"2002"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB15","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1002\/ana.410380510","article-title":"Immunocytochemical quantitation of human immunodeficiency virus in the brain: correlations with dementia","volume":"38","author":"Glass","year":"1995","journal-title":"Ann. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB16","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1002\/jnr.490210104","article-title":"Axotomy of the rat facial nerve leads to increased CR3 complement receptor expression by activated microglial cells","volume":"21","author":"Graeber","year":"1988","journal-title":"J. Neurosci. Res"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB17","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1002\/ana.410230103","article-title":"Axonal transport in peripheral neuropathies","volume":"23","author":"Griffin","year":"1988","journal-title":"Ann. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB18","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1126\/science.1083129","article-title":"Mutations in dynein link motor neuron degeneration to defects in retrograde transport","volume":"300","author":"Hafezparast","year":"2003","journal-title":"Science"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB19","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1002\/(SICI)1098-1136(199807)23:3<249::AID-GLIA7>3.0.CO;2-#","article-title":"Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS","volume":"23","author":"Hall","year":"1998","journal-title":"Glia"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB20","doi-asserted-by":"crossref","first-page":"12571","DOI":"10.1073\/pnas.220417997","article-title":"Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis","volume":"97","author":"Johnston","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U. S. A"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB21","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1097\/00001756-199605310-00021","article-title":"Neuromuscular function impairment is not caused by motor neuron loss in FALS mice: an electromyographic study","volume":"7","author":"Kennel","year":"1996","journal-title":"NeuroReport"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB22","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1016\/S0896-6273(02)00696-7","article-title":"Disruption of dynein\/dynactin inhibits axonal transport in motor neurons causing late-onset progressive deterioration","volume":"34","author":"LaMonte","year":"2002","journal-title":"Neuron"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB23","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1038\/ng1016","article-title":"A missense mutation in Tbce causes progressive motor neuronopathy in mice","volume":"32","author":"Martin","year":"2003","journal-title":"Nat. Genet"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB24","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1038\/ng1123","article-title":"Mutant dynactin in motor neuron disease","volume":"33","author":"Puls","year":"2003","journal-title":"Nat. Genet"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB25","doi-asserted-by":"crossref","first-page":"3305","DOI":"10.1152\/jn.00271.2002","article-title":"Reduced neuromuscular quantal content with normal synaptic release time course and depression in canine motor neuron disease","volume":"88","author":"Rich","year":"2002","journal-title":"J. Neurophysiol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB26","doi-asserted-by":"crossref","first-page":"6591","DOI":"10.1073\/pnas.90.14.6591","article-title":"Chronic inhibition of glutamate uptake produces a model of slow neurotoxicity","volume":"90","author":"Rothstein","year":"1993","journal-title":"Proc. Natl. Acad. Sci. U. S. A"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB27","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1016\/S0165-6147(97)01094-8","article-title":"Clinical and molecular aspects of motoneurone diseases: animal models, neurotrophic factors and Bcl-2 oncoprotein","volume":"18","author":"Sagot","year":"1997","journal-title":"Trends Pharmacol. Sci"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB28","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1001\/archneur.1983.04050050074011","article-title":"Amyotrophic lateral sclerosis: lack of central chromatolytic response of motor neurocytons corresponding to active axonal degeneration","volume":"40","author":"Sobue","year":"1983","journal-title":"Arch. Neurol"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB29","first-page":"253","article-title":"Central\u2013peripheral distal axonopathy\u2014The pathogenesis of dying-back polyneuropathies","volume":"vol. 3","author":"Spencer","year":"1976"},{"issue":"Supp. 13","key":"10.1016\/j.expneurol.2003.10.004_BIB30","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1007\/978-3-642-70699-8_1","article-title":"Reaction of motoneurons and their microenvironment to axotomy","author":"Tetzlaff","year":"1986","journal-title":"Exp. Brain Res"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB31","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1002\/ar.1092310411","article-title":"Unbiased stereological estimation of the total number of neurons in the subdivisions of the rat hippocampus using the optical fractionator","volume":"231","author":"West","year":"1991","journal-title":"Anat. Rec"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB32","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1038\/4553","article-title":"Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons","volume":"2","author":"Williamson","year":"1999","journal-title":"Nat. Neurosci"},{"key":"10.1016\/j.expneurol.2003.10.004_BIB33","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1083\/jcb.139.5.1307","article-title":"Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation","volume":"139","author":"Zhang","year":"1997","journal-title":"J. Cell Biol"}],"container-title":["Experimental Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488603004795?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488603004795?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,2,15]],"date-time":"2019-02-15T10:25:45Z","timestamp":1550226345000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0014488603004795"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,2]]},"references-count":34,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2004,2]]}},"alternative-id":["S0014488603004795"],"URL":"https:\/\/doi.org\/10.1016\/j.expneurol.2003.10.004","relation":{},"ISSN":["0014-4886"],"issn-type":[{"value":"0014-4886","type":"print"}],"subject":[],"published":{"date-parts":[[2004,2]]}}}