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論文 - 一覧

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  • 検索条件 : 絞込み (MeSH = Amyotrophic Lateral Sclerosis* / metabolism)
生物種 リソース名 タイトル
実験動物マウス RBRC01828 A mouse model with widespread expression of the C9orf72-linked glycine-arginine dipeptide displays non-lethal ALS/FTD-like phenotypes.
ゼブラフィッシュ UAS:GFP-LC3-RFP-LC3∆G, SAGFF(LF)73A, SAIGFF213A, mnr2b-hs:Gal4, TDP-43DKO, UAS:hmCP2AGd410mT436A, UAS:mCherry-P2A-EGFP-d410m-T436A Intrinsically accelerated cellular degradation is amplified by TDP-43 loss in ALS-vulnerable motor neurons in a zebrafish model.
ゼブラフィッシュ UAS:RFP Poly-GP accumulation due to C9orf72 loss of function induces motor neuron apoptosis through autophagy and mitophagy defects.
ゼブラフィッシュ mnr2b-hs:opTDP-43h, mnr2b-hs:EGFP-TDP43z Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology.
ショウジョウバエ DGRC#118679 , DGRC#118682 , DGRC#118681 Neuronal polyunsaturated fatty acids are protective in ALS/FTD.
ショウジョウバエ 3016R-2 Activation of the Keap1/Nrf2 pathway suppresses mitochondrial dysfunction, oxidative stress, and motor phenotypes in C9orf72 ALS/FTD models.
線虫 tm544 Analysis of proteome-wide degradation dynamics in ALS SOD1 iPSC-derived patient neurons reveals disrupted VCP homeostasis.
ヒト・動物細胞 NIH/3T3(RCB2767) Valosin-containing protein regulates the stability of fused in sarcoma granules in cells by changing ATP concentrations.
ゼブラフィッシュ hspGFFDMC130A, UAS:GFP, UAS:GCaMP7a, SAIGFF213A Dysregulated TDP-43 proteostasis perturbs excitability of spinal motor neurons during brainstem-mediated fictive locomotion in zebrafish.
ゼブラフィッシュ SAGFF(LF)73A , SAIGFF213A , UAS:mRFP1-TDP-43z , UAS:mRFP1-CRY2olig , UAS:EGFP , mnr2b-hs:Gal4 Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
ゼブラフィッシュ SAGFF(LF)73 , SAIGFF213A , UAS:mRFP1-TDP-43z , UAS:mRFP1-CRY2olig , UAS:EGFP , mnr2b-hs:Gal4 Optogenetic interrogation of TDP-43 cytotoxicity in a zebrafish ALS model
ショウジョウバエ Loss of amyotrophic lateral sclerosis risk factor SCFD1 causes motor dysfunction in Drosophila.
ヒト・動物細胞 B16F10(RCB2630) Molecular Analysis of the Melanogenesis Inhibitory Effect of Saponins-Rich Fraction of Argania spinosa Leaves Extract.
ヒト・動物細胞 C6(RCB2854) , RCR-1(RCB1456) Central nervous system specific high molecular weight ALS2/alsin homophilic complex is enriched in mouse brain synaptosomes.
実験動物マウス RBRC01828 NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility.
ショウジョウバエ Caspar, an adapter for VAPB and TER94, modulates the progression of ALS8 by regulating IMD/NFκB-mediated glial inflammation in a Drosophila model of human disease.