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Bull."},{"key":"10.1016\/j.ejphar.2020.173737_bib94","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0068312","article-title":"Evidence for glutamate as a neuroglial transmitter within sensory ganglia","volume":"8","author":"Kung","year":"2013","journal-title":"PloS One"},{"key":"10.1016\/j.ejphar.2020.173737_bib95","doi-asserted-by":"crossref","first-page":"3766","DOI":"10.1096\/fj.09-130047","article-title":"Neuroprotective and neuritogenic activities of novel multimodal iron-chelating drugs in motor-neuron-like NSC-34 cells and transgenic mouse model of amyotrophic lateral sclerosis","volume":"23","author":"Kupershmidt","year":"2009","journal-title":"Faseb. 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Mol. Genet."},{"key":"10.1016\/j.ejphar.2020.173737_bib105","doi-asserted-by":"crossref","first-page":"4296","DOI":"10.1073\/pnas.1200658109","article-title":"Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis","volume":"109","author":"Marinkovi\u0107","year":"2012","journal-title":"Proc. Natl. Acad. Sci. U. S. A"},{"key":"10.1016\/j.ejphar.2020.173737_bib106","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1006\/neur.1995.0046","article-title":"Neutron activation analysis of trace elements in motor neuron disease spinal cord","volume":"4","author":"Markesbery","year":"1995","journal-title":"Neurodegeneration"},{"key":"10.1016\/j.ejphar.2020.173737_bib107","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1016\/j.freeradbiomed.2013.04.018","article-title":"S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis","volume":"61","author":"Mead","year":"2013","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.ejphar.2020.173737_bib108","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.tox.2015.11.001","article-title":"Mechanisms of divalent metal toxicity in affective disorders","volume":"339","author":"Menon","year":"2016","journal-title":"Toxicology"},{"key":"10.1016\/j.ejphar.2020.173737_bib109","first-page":"CD001447","article-title":"Riluzole for amyotrophic lateral sclerosis (ALS)\/motor neuron disease (MND)","volume":"2","author":"Miller","year":"2002","journal-title":"Cochrane Database Syst. Rev."},{"key":"10.1016\/j.ejphar.2020.173737_bib110","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1159\/000116626","article-title":"Manganese, selenium and other trace elements in spinal cord, liver and bone in motor neurone disease","volume":"31","author":"Mitchell","year":"1991","journal-title":"Eur. 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Drug Alcohol Res."},{"key":"10.1016\/j.ejphar.2020.173737_bib115","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.3390\/ph7121028","article-title":"Role of microglial M1\/M2 polarization in relapse and remission of psychiatric disorders and diseases","volume":"7","author":"Nakagawa","year":"2014","journal-title":"Pharmaceuticals"},{"key":"10.1016\/j.ejphar.2020.173737_bib116","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.pharmthera.2015.06.010","article-title":"Diversity and plasticity of microglial cells in psychiatric and neurological disorders","volume":"154","author":"Nakagawa","year":"2015","journal-title":"Pharmacol. 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