About: Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1

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About: Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 Goto Sponge NotDistinct Permalink

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scientific article published on 01 May 1998

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rdf:type	Item
description	wetenschappelijk artikel (nl) наукова стаття, опублікована в травні 1998 (uk) article scientifique publié en 1998 (fr) im Mai 1998 veröffentlichter wissenschaftlicher Artikel (de) artículu científicu espublizáu en mayu de 1998 (ast) scientific article published on 01 May 1998 (en)
publication date	wds:Q74454204-F80FFB78-F138-4A87-92C8-EE9E5A69228B
publication date	1998-05-01 00:00:00Z (xsd:dateTime)
cites work	wds:Q74454204-0B2870B7-9FFB-4461-9634-72E4B68A65B3 wds:Q74454204-0E53D325-76D0-446B-ABE1-66C77079028D wds:Q74454204-14817C89-E9D4-4FE0-AD34-0B52006DC910 wds:Q74454204-1E952BB6-8A7E-4EAF-9474-B41918EAC5B7 wds:Q74454204-2801B349-1F23-4645-9176-48B8DEDCEB3C wds:Q74454204-2DDAE531-E772-4020-89B4-48129D9F8DAF wds:Q74454204-2F26C264-932C-4824-A64C-7E981858AB80 wds:Q74454204-38DB0098-DEE8-47C0-A251-F0B24CA20DCA wds:Q74454204-486341CA-ED0F-461C-A726-CD28F920336C wds:Q74454204-4E60DD79-B76D-4F84-A939-64820A1C2F71 wds:Q74454204-573B8CE3-D39D-4837-9EF1-BE07FCB7FE0D wds:Q74454204-59AE532A-B37B-4A16-9317-9B39D1E8D652 wds:Q74454204-8CFBA89C-DFA9-4AB2-850F-5F98D0880968 wds:Q74454204-8F1BBFF3-09EA-4520-8D7C-DC6B37233BBB wds:Q74454204-913F71E9-CCE6-435E-BC1A-E3BC71468165 wds:Q74454204-B4487E0D-68B3-45F4-977F-53776E22C73A wds:Q74454204-C756BCD5-0C62-4C4C-AC90-04C4CE527844 wds:Q74454204-D9FD3591-C43B-4D12-8E9B-E6E9D96FB52D wds:Q74454204-E2B1F24E-3C6B-4F57-B9C9-CE279B8E2284 wds:Q74454204-E9B9CF65-997B-4A81-9722-62D28D44589D wds:Q74454204-F5B5E214-8D30-4014-A031-A466362A6152
cites work	Mitochondria, free radicals, and neurodegeneration Prognosis in familial amyotrophic lateral sclerosis: progression and survival in patients with glu100gly and ala4val mutations in Cu,Zn superoxide dismutase Familial adult motor neuron disease: amyotrophic lateral sclerosis The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice Epidemiology of mutations in superoxide dismutase in amyotrophic lateral sclerosis Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria ALS, SOD and peroxynitrite Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions Mechanisms of selective motor neuron death in ALS: insights from transgenic mouse models of motor neuron disease Expression of a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis induces mitochondrial alteration and increase of cytosolic Ca2+ concentration in transfected neuroblastoma SH-SY5Y cells Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS). Quantitative immunocytochemical analysis of the spinal cord in G86R superoxide dismutase transgenic mice: neurochemical correlates of selective vulnerability Oxidative stress, mutant SOD1, and neurofilament pathology in transgenic mouse models of human motor neuron disease Cytopathology of amyotrophic lateral sclerosis
author name string	wds:Q74454204-8F567B26-836A-4AD9-9D75-F0B832381AC0 wds:Q74454204-90D4E6E1-71B4-4138-A459-FF18BAC55DFE
author name string	Z Xu J Kong
rdfs:label	Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (en) Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (nl)
skos:prefLabel	Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (en) Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (nl)
name	Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (en) Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (nl)
title	wds:Q74454204-0E9EC8DE-0B98-4C29-9D75-B44BF1384D37
title	Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 (en)
page(s)	wds:Q74454204-E999A393-1696-41C4-9AC6-48C420F10B5A
page(s)	3241-3250
instance of	wds:Q74454204-953B7207-675F-418F-A4CB-69C32A5BDAE3
instance of	scholarly article
main subject	wds:Q74454204-BCE1E840-7C77-4CEA-B478-32FB28B365CB
main subject	amyotrophic lateral sclerosis
PubMed ID	wds:Q74454204-781F761C-804D-4942-A1EE-D36B894A6EED
PubMed ID	http://rdf.ncbi.nlm.nih.gov/pubchem/reference/9547233
PubMed ID	9547233
published in	wds:Q74454204-4548545D-6FE0-40D0-803A-2D81AF21DC5E
published in	Journal of Neuroscience
issue	wds:Q74454204-7DDF7D77-16D9-4407-AE57-F8E7661079D4
volume	wds:Q74454204-E4DC5E86-573B-4857-8037-9B3694F6DEE8
issue	9
volume	18
DOI	wds:Q74454204-CC5E8C1F-7ADC-4ECB-A15D-AD7869114FB6
DOI	http://dx.doi.org/10.1523/JNEUROSCI.18-09-03241.1998
DOI	10.1523/JNEUROSCI.18-09-03241.1998
PMCID	wds:Q74454204-A5F2F6A5-9540-4CE4-A685-F67871E29D75
PMCID	6792665
is about of	https://www.wikidata.org/wiki/Special:EntityData/Q74454204
is cites work of	Imaging and neurochemical markers for diagnosis and disease progression in ALS. Cerebral microvascular endothelium and the pathogenesis of neurodegenerative diseases The "dying-back" phenomenon of motor neurons in ALS. A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS). Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS. Mitochondrial pathobiology in ALS. Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity. Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS Olesoxime, a cholesterol-like neuroprotectant for the potential treatment of amyotrophic lateral sclerosis Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis Miro1 deficiency in amyotrophic lateral sclerosis Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SOD(G93A) mice co-expressing the Copper-Chaperone-for-SOD

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