{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:40:06Z","timestamp":1791330006315,"version":"4.3.1"},"reference-count":43,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2004,7,1]],"date-time":"2004-07-01T00:00:00Z","timestamp":1088640000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2013,7,17]],"date-time":"2013-07-17T00:00:00Z","timestamp":1374019200000},"content-version":"vor","delay-in-days":3303,"URL":"https:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuron"],"published-print":{"date-parts":[[2004,7]]},"DOI":"10.1016\/j.neuron.2004.06.021","type":"journal-article","created":{"date-parts":[[2004,7,8]],"date-time":"2004-07-08T11:14:16Z","timestamp":1089285256000},"page":"19-30","source":"Crossref","is-referenced-by-count":442,"title":["Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria"],"prefix":"10.1016","volume":"43","author":[{"given":"Piera","family":"Pasinelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary Elizabeth","family":"Belford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niall","family":"Lennon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian J","family":"Bacskai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bradley T","family":"Hyman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Trotti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Robert H","family":"Brown","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.neuron.2004.06.021_BIB1","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1080\/14660820310011700","article-title":"Sixteen novel mutations in the Cu\/Zn superoxide dismutase gene in amyotrophic lateral sclerosis","volume":"4","author":"Andersen","year":"2003","journal-title":"Amyotroph. Lateral Scler. Other Motor Neuron Disord."},{"issue":"Suppl 1","key":"10.1016\/j.neuron.2004.06.021_BIB2","doi-asserted-by":"crossref","first-page":"S47","DOI":"10.1080\/146608201300079418","article-title":"Calcium","volume":"2","author":"Appel","year":"2001","journal-title":"Amyotroph. Lateral Scler. Other Motor Neuron Disord."},{"key":"10.1016\/j.neuron.2004.06.021_BIB3","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1117\/1.1584442","article-title":"Fluorescence resonance energy transfer determinations using multiphoton fluorescence lifetime imaging microscopy to characterize amyloid-beta plaques","volume":"8","author":"Bacskai","year":"2003","journal-title":"J. Biomed. Opt."},{"key":"10.1016\/j.neuron.2004.06.021_BIB4","doi-asserted-by":"crossref","first-page":"2322","DOI":"10.1111\/j.1471-4159.1993.tb07478.x","article-title":"Superoxide dismutase activity, oxidative damage and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis","volume":"61","author":"Bowling","year":"1993","journal-title":"J. Neurochem."},{"key":"10.1016\/j.neuron.2004.06.021_BIB5","doi-asserted-by":"crossref","first-page":"1851","DOI":"10.1126\/science.281.5384.1851","article-title":"Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1","volume":"281","author":"Bruijn","year":"1998","journal-title":"Science"},{"key":"10.1016\/j.neuron.2004.06.021_BIB6","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1038\/35097565","article-title":"From Charcot to Lou Gehrig","volume":"2","author":"Cleveland","year":"2001","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.neuron.2004.06.021_BIB7","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/0006-8993(95)00063-V","article-title":"Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu, Zn SOD, and in mice overexpressing wild type human SOD","volume":"676","author":"Dal Canto","year":"1995","journal-title":"Brain Res."},{"key":"10.1016\/j.neuron.2004.06.021_BIB8","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/S0092-8674(04)00046-7","article-title":"Cell death. Critical control points","volume":"116","author":"Danial","year":"2004","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2004.06.021_BIB9","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1097\/00005072-199705000-00008","article-title":"Aggregation of mutant Cu\/Zn superoxide dismutase proteins in a culture model of ALS","volume":"56","author":"Durham","year":"1997","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.neuron.2004.06.021_BIB10","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1111\/j.1600-0404.1999.tb00403.x","article-title":"Upregulation of Bax protein and increased DNA degradation in ALS spinal cord motor neurons","volume":"100","author":"Ekegren","year":"1999","journal-title":"Acta Neurol. Scand."},{"key":"10.1016\/j.neuron.2004.06.021_BIB11","doi-asserted-by":"crossref","first-page":"2498","DOI":"10.1126\/science.286.5449.2498","article-title":"Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase","volume":"286","author":"Estevez","year":"1999","journal-title":"Science"},{"key":"10.1016\/j.neuron.2004.06.021_BIB12","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1038\/40299","article-title":"Inhibition of ICE slows ALS in mice","volume":"388","author":"Friedlander","year":"1997","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2004.06.021_BIB13","doi-asserted-by":"crossref","first-page":"8756","DOI":"10.1523\/JNEUROSCI.17-22-08756.1997","article-title":"Mutant superoxide dismutase-1-linked familial amyotrophic lateral sclerosis","volume":"17","author":"Ghadge","year":"1997","journal-title":"J. Neurosci."},{"key":"10.1016\/j.neuron.2004.06.021_BIB14","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/0896-6273(95)90287-2","article-title":"Superoxide dismutase delays neuronal apoptosis","volume":"14","author":"Greenlund","year":"1995","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2004.06.021_BIB15","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1172\/JCI200317610","article-title":"Programmed cell death in amyotrophic lateral sclerosis","volume":"111","author":"Guegan","year":"2003","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.neuron.2004.06.021_BIB16","doi-asserted-by":"crossref","first-page":"6569","DOI":"10.1523\/JNEUROSCI.21-17-06569.2001","article-title":"Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis","volume":"21","author":"Guegan","year":"2001","journal-title":"J. Neurosci."},{"key":"10.1016\/j.neuron.2004.06.021_BIB17","doi-asserted-by":"crossref","first-page":"RC215","DOI":"10.1523\/JNEUROSCI.22-06-j0001.2002","article-title":"Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS","volume":"22","author":"Higgins","year":"2002","journal-title":"J. Neurosci."},{"key":"10.1016\/j.neuron.2004.06.021_BIB18","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1034\/j.1600-0404.2001.103004255.x","article-title":"Interleukin-1beta converting enzyme\/Caspase-1 (ICE\/Caspase-1) and soluble APO-1\/Fas\/CD 95 receptor in amyotrophic lateral sclerosis patients","volume":"103","author":"Ilzecka","year":"2001","journal-title":"Acta Neurol. Scand."},{"key":"10.1016\/j.neuron.2004.06.021_BIB19","doi-asserted-by":"crossref","first-page":"3241","DOI":"10.1523\/JNEUROSCI.18-09-03241.1998","article-title":"Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1","volume":"18","author":"Kong","year":"1998","journal-title":"J. Neurosci."},{"key":"10.1016\/j.neuron.2004.06.021_BIB20","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1126\/science.277.5325.559","article-title":"Bcl-2","volume":"277","author":"Kostic","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.neuron.2004.06.021_BIB21","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1126\/science.288.5464.335","article-title":"Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model","volume":"288","author":"Li","year":"2000","journal-title":"Science"},{"key":"10.1016\/j.neuron.2004.06.021_BIB22","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/S0092-8674(04)00162-X","article-title":"Conversion of Bcl-2 from protector to killer by interaction with nuclear orphan receptor Nur77\/TR3","volume":"116","author":"Lin","year":"2004","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2004.06.021_BIB23","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1097\/00005072-199905000-00005","article-title":"Neuronal death in amyotrophic lateral sclerosis is apoptosis","volume":"58","author":"Martin","year":"1999","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.neuron.2004.06.021_BIB24","doi-asserted-by":"crossref","first-page":"29626","DOI":"10.1074\/jbc.M203065200","article-title":"Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice","volume":"277","author":"Mattiazzi","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.neuron.2004.06.021_BIB25","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/S0896-6273(00)80282-2","article-title":"Inactivation of bcl-2 results in progressive degeneration of motoneurons, sympathetic and sensory neurons during early postnatal development","volume":"17","author":"Michaelidis","year":"1996","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2004.06.021_BIB26","doi-asserted-by":"crossref","first-page":"9010","DOI":"10.1073\/pnas.132260399","article-title":"Amyotrophic lateral sclerosis","volume":"99","author":"Okado-Matsumoto","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB27","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1038\/534","article-title":"Extension of Drosophila lifespan by overexpression of human SOD1 in motorneurons","volume":"19","author":"Parkes","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/j.neuron.2004.06.021_BIB28","doi-asserted-by":"crossref","first-page":"15763","DOI":"10.1073\/pnas.95.26.15763","article-title":"Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase","volume":"95","author":"Pasinelli","year":"1998","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB29","doi-asserted-by":"crossref","first-page":"13901","DOI":"10.1073\/pnas.240305897","article-title":"Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis","volume":"97","author":"Pasinelli","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB30","doi-asserted-by":"crossref","first-page":"3024","DOI":"10.1073\/pnas.92.7.3024","article-title":"Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene","volume":"92","author":"Rabizadeh","year":"1995","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB31","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1038\/362059a0","article-title":"Mutations in Cu\/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis","volume":"362","author":"Rosen","year":"1993","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2004.06.021_BIB32","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1002\/ana.410280106","article-title":"Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis","volume":"28","author":"Rothstein","year":"1990","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.neuron.2004.06.021_BIB33","doi-asserted-by":"crossref","first-page":"4155","DOI":"10.1073\/pnas.91.10.4155","article-title":"Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons","volume":"91","author":"Rothstein","year":"1994","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB34","doi-asserted-by":"crossref","first-page":"3541","DOI":"10.1182\/blood.V98.13.3541","article-title":"Receptor- and mitochondrial-mediated apoptosis in acute leukemia","volume":"98","author":"Schimmer","year":"2001","journal-title":"Blood"},{"key":"10.1016\/j.neuron.2004.06.021_BIB35","doi-asserted-by":"crossref","first-page":"7834","DOI":"10.1073\/pnas.92.17.7834","article-title":"Multiple Bcl-2 family members demonstrate selective dimerizations with Bax","volume":"92","author":"Sedlak","year":"1995","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB36","first-page":"38084","article-title":"A fraction of yeast Cu,Zn-superoxide dismutase and its metallochaperone, CCS, localize to the intermembrane space of mitochondria","volume":"1","author":"Sturtz","year":"2001","journal-title":"A physiological role for SOD"},{"key":"10.1016\/j.neuron.2004.06.021_BIB37","doi-asserted-by":"crossref","first-page":"50966","DOI":"10.1074\/jbc.M209356200","article-title":"Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis","volume":"277","author":"Takeuchi","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.neuron.2004.06.021_BIB38","doi-asserted-by":"crossref","first-page":"6384","DOI":"10.1073\/pnas.91.14.6384","article-title":"Down-regulation of copper\/zinc superoxide dismutase causes apoptotic death in PC12 neuronal cells","volume":"91","author":"Troy","year":"1994","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2004.06.021_BIB39","doi-asserted-by":"crossref","first-page":"2460","DOI":"10.1046\/j.1471-4159.1999.0732460.x","article-title":"Bax and Bcl-2 intercation in a transgenic mouse model of familial amyotrophic lateral sclerosis","volume":"73","author":"Vukosavic","year":"1999","journal-title":"J. Neurochem."},{"key":"10.1016\/j.neuron.2004.06.021_BIB40","doi-asserted-by":"crossref","first-page":"9119","DOI":"10.1523\/JNEUROSCI.20-24-09119.2000","article-title":"Delaying caspase activation by Bcl-2","volume":"20","author":"Vukosavic","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.neuron.2004.06.021_BIB41","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1016\/0896-6273(95)90259-7","article-title":"An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria","volume":"14","author":"Wong","year":"1995","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2004.06.021_BIB42","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1006\/exnr.1997.6746","article-title":"Developmental patterns of BCL-2 and BCL-X polypeptide expression in the human spinal cord","volume":"150","author":"Yachnis","year":"1998","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.neuron.2004.06.021_BIB43","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1038\/369321a0","article-title":"BH1 and BH2 domains of Bcl-2 are required for inhibition of apoptosis and heterodimerization with Bax","volume":"369","author":"Yin","year":"1994","journal-title":"Nature"}],"container-title":["Neuron"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896627304003915?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896627304003915?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,2,5]],"date-time":"2019-02-05T20:53:08Z","timestamp":1549399988000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0896627304003915"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,7]]},"references-count":43,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2004,7]]}},"alternative-id":["S0896627304003915"],"URL":"https:\/\/doi.org\/10.1016\/j.neuron.2004.06.021","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.1022215.251769","asserted-by":"object"},{"id-type":"doi","id":"10.3410\/f.1022215.14073074","asserted-by":"object"}]},"ISSN":["0896-6273"],"issn-type":[{"value":"0896-6273","type":"print"}],"subject":[],"published":{"date-parts":[[2004,7]]}}}