[Molecular mechanisms of amyotrophic lateral sclerosis: recent contributions from studies in animal models]

scientific article published on 01 January 2004

[Molecular mechanisms of amyotrophic lateral sclerosis: recent contributions from studies in animal models] is …
instance of (P31):
scholarly articleQ13442814
review articleQ7318358

External links are
P356DOI10.1016/S0035-3787(04)70845-0
P698PubMed publication ID14978392

P2093author name stringV Meininger
A Muller
L Dupuis
J P Loeffler
P2860cites workAggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1Q22299419
A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2Q24291755
Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosisQ24306886
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosisQ28131805
The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosisQ28190263
Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosisQ28203381
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injuryQ28511628
Bax and Bcl-2 interaction in a transgenic mouse model of familial amyotrophic lateral sclerosisQ28585525
Deregulation of Cdk5 in a mouse model of ALS: toxicity alleviated by perikaryal neurofilament inclusionsQ28591963
Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNSQ28610671
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutationQ29547561
Loss of prion protein in a transgenic model of amyotrophic lateral sclerosisQ32063679
Familial amyotrophic lateral sclerosisQ34547620
Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosisQ34718757
Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosisQ34736976
Reduction of axonal caliber does not alleviate motor neuron disease caused by mutant superoxide dismutase 1Q35425101
Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosisQ35580300
Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutaseQ36270615
Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxQ36662645
Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis.Q38291855
Neuronal-specific expression of human copper-zinc superoxide dismutase gene in transgenic mice: animal model of gene dosage effects in Down's syndromeQ41140051
Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosisQ41237348
Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neuronsQ41644415
Transgenic ALS mice show increased vulnerability to the mitochondrial toxins MPTP and 3-nitropropionic acid.Q43547870
Phase III randomized trial of gabapentin in patients with amyotrophic lateral sclerosisQ43569621
A double-blind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study GroupQ43683334
Effects of creatine supplementation on exercise performance and muscular strength in amyotrophic lateral sclerosis: preliminary results.Q43777132
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patientsQ43883813
High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulationQ43918294
Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in miceQ43977943
Minocycline delays disease onset and mortality in a transgenic model of ALS.Q44024931
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding siteQ44070818
Survival in transgenic ALS mice does not vary with CNS glutathione peroxidase activity.Q44131398
Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration.Q45877621
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.Q46135324
Familial amyotrophic lateral sclerosis-associated mutations decrease the thermal stability of distinctly metallated species of human copper/zinc superoxide dismutaseQ46566731
Beneficial effects of lysine acetylsalicylate, a soluble salt of aspirin, on motor performance in a transgenic model of amyotrophic lateral sclerosisQ48266693
Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment.Q48874060
A quantitative histochemical assay for activities of mitochondrial electron transport chain complexes in mouse spinal cord sections.Q50501359
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.Q51714635
Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration.Q52546347
Survival in patients with amyotrophic lateral sclerosis, treated with an array of antioxidants.Q52908162
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondriaQ54965159
Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis.Q55032889
Inhibition of ICE slows ALS in miceQ59081638
Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse ModelQ60144092
Familial adult motor neuron disease: amyotrophic lateral sclerosisQ70035464
Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosisQ70633178
Induction of nitrotyrosine-like immunoreactivity in the lower motor neuron of amyotrophic lateral sclerosisQ70958165
A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study GroupQ72759362
Blood oxidative stress in amyotrophic lateral sclerosisQ73039021
Differential vulnerability of oculomotor, facial, and hypoglossal nuclei in G86R superoxide dismutase transgenic miceQ73219169
A mouse model of familial amyotrophic lateral sclerosis expressing a mutant superoxide dismutase 1 shows evidence of disordered transport in the vasopressin hypothalamo-neurohypophysial axisQ73265105
Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutaseQ73316561
Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degenerationQ73348009
A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosisQ73440820
Bcl-2: prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosisQ73520806
Increased 3-nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosisQ73907503
Differential screening of mutated SOD1 transgenic mice reveals early up-regulation of a fast axonal transport component in spinal cord motor neuronsQ74240941
Alteration of the Bcl-x/Bax ratio in a transgenic mouse model of amyotrophic lateral sclerosis: evidence for the implication of the p53 signaling pathwayQ74240982
Early and selective pathology of light chain neurofilament in the spinal cord and sciatic nerve of G86R mutant superoxide dismutase transgenic miceQ74332165
Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron diseaseQ74334654
Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosisQ74411605
Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1Q74454204
CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutationsQ74625760
Instrumental activation of bid by caspase-1 in a transgenic mouse model of ALSQ74733872
The antioxidant N-acetylcysteine does not delay disease onset and death in a transgenic mouse model of amyotrophic lateral sclerosisQ77107922
Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loadingQ77754821
P433issue1
P921main subjectamyotrophic lateral sclerosisQ206901
P304page(s)35-43
P577publication date2004-01-01
P1433published inRevue neurologiqueQ7318987
P1476title[Molecular mechanisms of amyotrophic lateral sclerosis: recent contributions from studies in animal models]
P478volume160

Reverse relations

cites work (P2860)
Q38160421Amyotrophic lateral sclerosis and skeletal muscle: an update.
Q33294971Microarray analysis of the cellular pathways involved in the adaptation to and progression of motor neuron injury in the SOD1 G93A mouse model of familial ALS.
Q47155349Specific Physical Exercise Improves Energetic Metabolism in the Skeletal Muscle of Amyotrophic-Lateral- Sclerosis Mice

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