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{"title":"Disrupted endoplasmic reticulum-mediated autophagosomal biogenesis in a <i>Drosophila</i> model of C9-ALS-FTD.","authors":"Hyun Sung, Thomas E Lloyd","doi":"10.1080/15548627.2023.2249750","DOIUrl":null,"url":null,"abstract":"<p><strong>Abbreviations: </strong>3R: UAS construct expressing 3 G<sub>4</sub>C<sub>2</sub> repeats (used as control); 3WJ: three-way junction; 12R: UAS construct expressing leader sequence and 12 G<sub>4</sub>C<sub>2</sub> repeats; 30R: UAS construct expressing 30 G<sub>4</sub>C<sub>2</sub> repeats; 36R: UAS construct expressing 36 G<sub>4</sub>C<sub>2</sub> repeats; 44R: UAS construct expressing leader sequence and 44 G<sub>4</sub>C<sub>2</sub> repeats; ALS: amyotrophic lateral sclerosis; Atg: autophagy related; atl: atlastin; C9-ALS-FTD: ALS or FTD caused by hexanuleotide repeat expansion in <i>C9orf72</i>; ER: endoplasmic reticulum; FTD: frontotemporal dementia; HRE: GGGGCC hexanucleotide repeat expansion; HSP: hereditary spastic paraplegia; Lamp1: lysosomal associated membrane protein 1; MT: microtubule; NMJ: neuromuscular junction; Rab: Ras-associated binding GTPase; RAN: repeat associated non-AUG (RAN) translation; RO-36: UAS construct expression \"RNA-only\" version of 36 G<sub>4</sub>C<sub>2</sub> repeats in which stop codons in all six reading frames are inserted.; Rtnl1: Reticulon-like 1; SN: segmental nerve; TFEB/Mitf: transcription factor EB/microphthalmia associated transcription factor (<i>Drosophila</i> ortholog of TFEB); TrpA1: transient receptor potential cation channel A1; VAPB: VAMP associated protein B and C; VNC: ventral nerve cord (spinal cord in <i>Drosophila</i> larvae).</p>","PeriodicalId":8722,"journal":{"name":"Autophagy","volume":" ","pages":"94-113"},"PeriodicalIF":14.6000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10761023/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Autophagy","FirstCategoryId":"99","ListUrlMain":"https://doi.org/10.1080/15548627.2023.2249750","RegionNum":1,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/8/27 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"CELL BIOLOGY","Score":null,"Total":0}
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Abstract
Abbreviations: 3R: UAS construct expressing 3 G4 C2 repeats (used as control); 3WJ: three-way junction; 12R: UAS construct expressing leader sequence and 12 G4 C2 repeats; 30R: UAS construct expressing 30 G4 C2 repeats; 36R: UAS construct expressing 36 G4 C2 repeats; 44R: UAS construct expressing leader sequence and 44 G4 C2 repeats; ALS: amyotrophic lateral sclerosis; Atg: autophagy related; atl: atlastin; C9-ALS-FTD: ALS or FTD caused by hexanuleotide repeat expansion in C9orf72 ; ER: endoplasmic reticulum; FTD: frontotemporal dementia; HRE: GGGGCC hexanucleotide repeat expansion; HSP: hereditary spastic paraplegia; Lamp1: lysosomal associated membrane protein 1; MT: microtubule; NMJ: neuromuscular junction; Rab: Ras-associated binding GTPase; RAN: repeat associated non-AUG (RAN) translation; RO-36: UAS construct expression "RNA-only" version of 36 G4 C2 repeats in which stop codons in all six reading frames are inserted.; Rtnl1: Reticulon-like 1; SN: segmental nerve; TFEB/Mitf: transcription factor EB/microphthalmia associated transcription factor (Drosophila ortholog of TFEB); TrpA1: transient receptor potential cation channel A1; VAPB: VAMP associated protein B and C; VNC: ventral nerve cord (spinal cord in Drosophila larvae).
C9-ALS-FTD果蝇模型中内质网介导的自噬体生物发生紊乱
缩写:3R:表达 3 个 G4C2 重复序列的 UAS 构建物(用作对照);3WJ:三向连接;12R:表达领导序列和 12 个 G4C2 重复序列的 UAS 构建物;30R:表达 30 个 G4C2 重复序列的 UAS 构建物;36R:表达 36 个 G4C2 重复序列的 UAS 构建物;44R:表达领导序列和 44 个 G4C2 重复序列的 UAS 构建物;ALS:肌萎缩侧索硬化症;Atg:自噬相关;atl:atlastin;ALS:肌萎缩侧索硬化症:ALS:肌萎缩性脊髓侧索硬化症;Atg:自噬相关;atl:atlastin;C9-ALS-FTD:由 C9orf72 中六位核苷酸重复扩增引起的 ALS 或 FTD;ER:内质网;FTD:额颞叶痴呆症;HRE:GGGGCC:六核苷酸重复扩增;HSP:遗传性痉挛性截瘫;Lamp1:溶酶体相关膜蛋白 1;MT:微管;NMJ:神经肌肉接头;Rab:RAN:重复相关非 AUG(RAN)翻译;RO-36:UAS 构建表达 36 个 G4C2 重复序列的 "纯 RNA "版本,其中插入了所有六个阅读框中的终止密码子;Rtnl1:TFEB/Mitf:转录因子 EB/小眼症相关转录因子(果蝇 TFEB 的直向同源物);TrpA1:瞬时受体电位阳离子通道 A1;VAPB:VAMP 相关蛋白 B 和 C;VNC:腹侧神经索(果蝇幼虫的脊髓)。
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