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論文 - 一覧

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  • 検索条件 : 絞込み (MeSH = Motor Neurons / pathology)
生物種 リソース名 タイトル
実験動物マウス RBRC01386 Involvement of neuronal and muscular Trk-fused gene (TFG) defects in the development of neurodegenerative diseases.
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.
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 UAS:RFP Functional Characterization of Neurofilament Light Splicing and Misbalance in Zebrafish.
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
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.
ヒト・動物細胞 409B2(HPS0076) A widespread family of heat-resistant obscure (Hero) proteins protect against protein instability and aggregation.
線虫 tm700 Disruption of genes associated with Charcot-Marie-Tooth type 2 lead to common behavioural, cellular and molecular defects in Caenorhabditis elegans.
ゼブラフィッシュ Tg(isl1:GFP)rw0 Exosc2 deficiency leads to developmental disorders by causing a nucleotide pool imbalance in zebrafish.
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.
実験動物マウス RBRC02192 , RBRC05147 Involvement of p38 in Age-Related Decline in Adult Neurogenesis via Modulation of Wnt Signaling.
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.
線虫 tm1086 The C. elegans BRCA2-ALP/Enigma Complex Regulates Axon Regeneration via a Rho GTPase-ROCK-MLC Phosphorylation Pathway.
線虫 tm4255 Neuron-specific knock-down of SMN1 causes neuron degeneration and death through an apoptotic mechanism.
ショウジョウバエ 6117R-1 , 6117R-2 The PKA-C3 catalytic subunit is required in two pairs of interneurons for successful mating of Drosophila.
ゼブラフィッシュ Tg(CM-isl1:GFP) Altered RNA metabolism due to a homozygous RBM7 mutation in a patient with spinal motor neuropathy.
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.
線虫 tm2484 Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons.
ゼブラフィッシュ Tg(brn3a-hsp70:GFP)/rw0110b The cellular and molecular progression of mitochondrial dysfunction induced by 2,4-dinitrophenol in developing zebrafish embryos.