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).
about
Different human copper-zinc superoxide dismutase mutants, SOD1G93A and SOD1H46R, exert distinct harmful effects on gross phenotype in miceALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomesTrophic factors as modulators of motor neuron physiology and survival: implications for ALS therapyComparative Magnetic Resonance Imaging and Histopathological Correlates in Two SOD1 Transgenic Mouse Models of Amyotrophic Lateral SclerosisA splicing mutation in the novel mitochondrial protein DNAJC11 causes motor neuron pathology associated with cristae disorganization, and lymphoid abnormalities in miceIron insufficiency compromises motor neurons and their mitochondrial function in Irp2-null miceHuman neural stem cell replacement therapy for amyotrophic lateral sclerosis by spinal transplantationLoss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal traffickingBax and Bcl-2 interaction in a transgenic mouse model of familial amyotrophic lateral sclerosisActivated alpha 2-macroglobulin reverses the immunosuppressive activity in human breast cancer cell-conditioned medium by selectively neutralizing transforming growth factor-beta in the presence of interleukin-2.Genome-wide association analysis reveals a SOD1 mutation in canine degenerative myelopathy that resembles amyotrophic lateral sclerosis.A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron diseaseIdentification of B6SJL mSOD1(G93A) mouse subgroups with different disease progression rates.Neuroprotection from NMDA excitotoxic lesion by Cu/Zn superoxide dismutase gene delivery to the postnatal rat brain by a modular protein vector.Proteasomes remain intact, but show early focal alteration in their composition in a mouse model of amyotrophic lateral sclerosis.Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.Postneonatal Mortality and Liver Changes in Cloned Pigs Associated with Human Tumor Necrosis Factor Receptor I-Fc and Human Heme Oxygenase-1 Overexpression.Neurotoxic mechanisms of degeneration in motor neuron diseases.Comparison of Sirtuin 3 Levels in ALS and Huntington's Disease-Differential Effects in Human Tissue Samples vs. Transgenic Mouse Models.Nerve injection of viral vectors efficiently transfers transgenes into motor neurons and delivers RNAi therapy against ALS.Phenotype of transgenic mice carrying a very low copy number of the mutant human G93A superoxide dismutase-1 gene associated with amyotrophic lateral sclerosisWhat transgenic mice tell us about neurodegenerative disease.A high-throughput screen to identify inhibitors of SOD1 transcription.Complex genetics of amyotrophic lateral sclerosis.Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS).Reduction in hSOD1 copy number significantly impacts ALS phenotype presentation in G37R (line 29) mice: implications for the assessment of putative therapeutic agentsA review of the neural mechanisms of action and clinical efficiency of riluzole in treating amyotrophic lateral sclerosis: what have we learned in the last decade?Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation.Screening for inhibitors of the SOD1 gene promoter: pyrimethamine does not reduce SOD1 levels in cell and animal modelsPersistent inward currents in spinal motoneurons: important for normal function but potentially harmful after spinal cord injury and in amyotrophic lateral sclerosis.Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondriaEmerging strategies for drug development in motor neuron disease.Programmed cell death in amyotrophic lateral sclerosisMitochondrial abnormalities and low grade inflammation are present in the skeletal muscle of a minority of patients with amyotrophic lateral sclerosis; an observational myopathology study.A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivoMotor neuron trophic factors: therapeutic use in ALS?A Ufd2/D4Cole1e chimeric protein and overexpression of Rbp7 in the slow Wallerian degeneration (WldS) mouse.Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signalingAdaptive and maladaptive motor axonal sprouting in aging and motoneuron disease.Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis.
P2860
Q21560962-FCD99A32-0975-4CEF-8A8D-C900CDD925EFQ24792140-363CB8BD-04B7-43BB-AD86-E268FEB7B04BQ26999881-5CFCA089-9B5B-4B19-AD25-E43858187D78Q27303080-BD5C79D9-5091-4D7B-A569-AF07AD6167D0Q27316380-82737C05-7A13-48AA-8E07-CBA93632432EQ27318323-22A90E47-B3AD-4111-82D1-09396F147CE1Q27324779-1865E522-DDDE-41D9-B3F5-57EC3CF2928AQ27332255-5D7A3DC2-7BEE-4133-ABEB-CD72A170D1C4Q28585525-53F5E99B-F317-4599-98C7-AEBB257C16F9Q30471974-E6267293-61CB-4EDB-B6F0-58EA04060089Q30485762-2B8F81F2-D573-441D-84F1-C4C773F0C66AQ30496374-0D9ECE45-DBCB-48ED-9AA4-ECC4FDD28B00Q30668718-8441B2F6-4D22-4FC1-82D9-0E59501D324DQ33241058-1E3E8ADB-38AA-4609-8653-3FC27812E43EQ33322191-268A484E-0D3F-4B3C-8C06-0534EE09CE7FQ33570225-6DAE5D30-D015-4937-8F0A-A0E920D61B85Q33630950-3DFAC01B-E8E6-4337-8579-3DEC5AF7BEF6Q33719819-7FEEEA1D-0BF1-4C8A-95CC-BBC913AA6884Q33729341-0C866658-4AB9-4015-8D7F-B69F128CD884Q33742711-8FC6DE9C-7386-4B6F-BAF1-CCE90CE1D795Q33779002-7129ED2B-ABA5-42CB-9ED3-0B8F369E2868Q33843472-9EDB5CB2-50C7-4F45-AC00-464AE8EE75E0Q33850895-CD8EE399-DE73-41FC-A3F9-6EE372C09A43Q33910597-34358698-F3B6-4800-921A-8F8B3B8DD359Q34010341-7369825B-4D1F-4AAE-8171-6941406BE451Q34051858-DC876806-9A7C-4C4D-B59C-2193982D55C5Q34104378-AAA7F99F-A006-42C2-AFB0-14E22122DA2CQ34220144-39ACA183-1C96-4A8C-8D8D-26449FE9D450Q34234969-2B0EE878-B605-4C67-9201-BB6B0E1370A9Q34399203-76BEBC37-6F4F-476C-89F3-FAEDC7B81A09Q34565165-6A7D51CB-2DA1-419D-92BC-4520BBECD07AQ34581485-9C22D161-72A7-494E-AFFC-22FE88742611Q34831541-4B9967B8-3216-418A-8A1A-E9E991D0E9A5Q34978980-828840F4-0E29-4DEA-92D8-264F743047DCQ35007685-26161B27-2247-4C55-A25F-F07931C3938EQ35027903-63CD0417-9F15-4CB0-90F0-F0B930D8AC9DQ35286946-E2CCBBF0-6732-43E1-A5F1-0EC390EADB33Q35701794-DDFB6300-052B-4D51-8A73-5C973B6965F1Q35741414-06653BF6-79DB-4976-AF49-D74D52AB8E78Q35763845-A52D805B-E95D-4705-BE84-BD1E84AC2497
P2860
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).
description
1995 nî lūn-bûn
@nan
1995年の論文
@ja
1995年論文
@yue
1995年論文
@zh-hant
1995年論文
@zh-hk
1995年論文
@zh-mo
1995年論文
@zh-tw
1995年论文
@wuu
1995年论文
@zh
1995年论文
@zh-cn
name
Neuropathological changes in t ...... phic lateral sclerosis (FALS).
@en
type
label
Neuropathological changes in t ...... phic lateral sclerosis (FALS).
@en
prefLabel
Neuropathological changes in t ...... phic lateral sclerosis (FALS).
@en
P1433
P1476
Neuropathological changes in t ...... phic lateral sclerosis (FALS).
@en
P2093
Dal Canto MC
P356
10.1016/0006-8993(95)00063-V
P577
1995-04-01T00:00:00Z