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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
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.
ヒト・動物細胞 , 遺伝子材料 293(RCB1637) , Achilles/pRSETB (RDB15982) , B6N Mouse BAC clone B6Ng01-222C01 , pCAG-Achilles-Tardbp 3' UTR (RDB21123) , pCAG-Achilles-Tardbp deltaUTR (RDB21124) , pCMV-TARDBP-pA (RDB21125) A partial deletion of the Tardbp 3´UTR affects TDP-43 regulation and leads to motor dysfunction in mice.
ショウジョウバエ DGRC#118679 , DGRC#118682 , DGRC#118681 Neuronal polyunsaturated fatty acids are protective in ALS/FTD.
遺伝子材料 pCAG-HIVgp (RDB04394) , pCMV-VSV-G-RSV-Rev (RDB04393) USP10 Inhibits Aberrant Cytoplasmic Aggregation of TDP-43 by Promoting Stress Granule Clearance.
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 tm4439 , tm776 Fused in sarcoma regulates glutamate signaling and oxidative stress response.
線虫 tm544 Analysis of proteome-wide degradation dynamics in ALS SOD1 iPSC-derived patient neurons reveals disrupted VCP homeostasis.
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.
ヒト・動物細胞 YST-1(RCB2136) Schwann cell derived-peroxiredoxin protects motor neurons against hydrogen peroxide-induced cell death in mouse motor neuron cell line NSC-34.
ヒト・動物細胞 , 遺伝子材料 201B7(HPS0063) , CSII-EF-MCS-IRES2-Venus (RDB04384) , pCMV-VSV-G-RSV-Rev (RDB04393) , pCAG-HIVgp (RDB04394) Monomerization of TDP-43 is a key determinant for inducing TDP-43 pathology in amyotrophic lateral sclerosis.
メダカ OK-Cab (MT830) Optineurin deficiency impairs autophagy to cause interferon beta overproduction and increased survival of mice following viral infection
線虫 tm1287 Targeting the NEDP1 enzyme to ameliorate ALS phenotypes through stress granule disassembly.
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.