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論文 - 一覧

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  • 検索条件 : 絞込み (MeSH = Amyotrophic Lateral Sclerosis / genetics*)
生物種 リソース名 タイトル
実験動物マウス RBRCGD000108 , RBRCGD000110 Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
遺伝子材料 pcDNA5-FRT-TO-EGFP-Mo DP1 (RDB19223) , pcDNA5-FRT-TO-EGFP-Mo Climp63 (RDB19222) Proteomics-Based Approach Identifies Altered ER Domain Properties by ALS-Linked VAPB Mutation.
ヒト・動物細胞 SK-N-SH(RCB0426) An in vitro model for Lewy body-like hyaline inclusion/astrocytic hyaline inclusion: induction by ER stress with an ALS-linked SOD1 mutation.
ショウジョウバエ 4887R-3 , 4901R-3 , 4901R-4 , 5013R-2 , 5013R-3 , 6013R-2 , 7069R-1 , 7069R-2 , 7470R-2 , 7470R-3 , ... ZNF598 co-translationally titrates poly(GR) protein implicated in the pathogenesis of C9ORF72-associated ALS/FTD.
ラット W-Tg(CAG-GFP)184Ys (StrainID=525) Intravenous infusion of mesenchymal stem cells delays disease progression in the SOD1G93A transgenic amyotrophic lateral sclerosis rat model.
ゼブラフィッシュ Tg(hb9:Venus) Aberrant axon branching via Fos-B dysregulation in FUS-ALS motor neurons.
線虫 tm776 Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration.
ショウジョウバエ TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response.
線虫 tm776 A V-to-F substitution in SK2 channels causes Ca2+ hypersensitivity and improves locomotion in a C. elegans ALS model.
線虫 tm3659 A C9orf72 ALS/FTD Ortholog Acts in Endolysosomal Degradation and Lysosomal Homeostasis.
ショウジョウバエ Self-assembly of FUS through its low-complexity domain contributes to neurodegeneration.
ゼブラフィッシュ pT2KXIGdeltaIN Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration.
ゼブラフィッシュ Tg(Huc:Kaede) ALS-linked protein disulfide isomerase variants cause motor dysfunction.
ショウジョウバエ 5495R-1 , 5445R-4 Ubiquitin-Binding Protein CG5445 Suppresses Aggregation and Cytotoxicity of Amyotrophic Lateral Sclerosis-Linked TDP-43 in Drosophila.
ショウジョウバエ 9553R-3 Familial Amyotrophic Lateral Sclerosis-linked Mutations in Profilin 1 Exacerbate TDP-43-induced Degeneration in the Retina of Drosophila melanogaster through an Increase in the Cytoplasmic Localization of TDP-43.
ヒト・動物細胞 SK-N-SH(RCB0426) Familial amyotrophic lateral sclerosis (FALS)-linked SOD1 mutation accelerates neuronal cell death by activating cleavage of caspase-4 under ER stress in an in vitro model of FALS.
ショウジョウバエ Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration.
ヒト・動物細胞 GI-1(RCB0763) VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death.