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  • 検索条件 : 絞込み (MeSH = Amyotrophic Lateral Sclerosis / pathology)
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.
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.
ゼブラフィッシュ Multi-phaseted problems of TDP-43 in selective neuronal vulnerability in ALS.
ヒト・動物細胞 CiRA00026(HPS0294) TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS.
ゼブラフィッシュ Tg(hb9:Venus) Aberrant axon branching via Fos-B dysregulation in FUS-ALS motor neurons.
ゼブラフィッシュ Tg(HuC:GVP), UAS:GFP Myelin degeneration induced by mutant superoxide dismutase 1 accumulation promotes amyotrophic lateral sclerosis.
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 Hippo, Drosophila MST, is a novel modifier of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS.
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 tm7084 The THO Complex Coordinates Transcripts for Synapse Development and Dopamine Neuron Survival.
ショウジョウバエ Self-assembly of FUS through its low-complexity domain contributes to neurodegeneration.
ショウジョウバエ RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy.
線虫 tm1 , tm4439 FUS Regulates Activity of MicroRNA-Mediated Gene Silencing.
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.
遺伝子材料 pEF-BOS FLAG-mouse Rab29 (RDB14971) , pEF-BOS FLAG-mouse Rab29-Q67L (RDB14972) , pEF-BOS FLAG-mouse Rab29-T21N (RDB14973). C9orf72 and RAB7L1 regulate vesicle trafficking in amyotrophic lateral sclerosis and frontotemporal dementia.
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.
線虫 tm1411 VAPB/ALS8 MSP ligands regulate striated muscle energy metabolism critical for adult survival in caenorhabditis elegans.
ゼブラフィッシュ Tg(isl1:GFP)/rw0 Early interneuron dysfunction in ALS: insights from a mutant sod1 zebrafish model.