C9-ALS-FTD果蝇模型中内质网介导的自噬体生物发生紊乱

IF 14.6 1区生物学 Q1 CELL BIOLOGY

Autophagy Pub Date : 2024-01-01 Epub Date: 2023-08-27 DOI:10.1080/15548627.2023.2249750

Hyun Sung, Thomas E Lloyd

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引用次数: 0

摘要

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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Disrupted endoplasmic reticulum-mediated autophagosomal biogenesis in a Drosophila model of C9-ALS-FTD.

Abbreviations: 3R: UAS construct expressing 3 G₄C₂ repeats (used as control); 3WJ: three-way junction; 12R: UAS construct expressing leader sequence and 12 G₄C₂ repeats; 30R: UAS construct expressing 30 G₄C₂ repeats; 36R: UAS construct expressing 36 G₄C₂ repeats; 44R: UAS construct expressing leader sequence and 44 G₄C₂ 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 G₄C₂ 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).

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来源期刊

Autophagy 生物-细胞生物学

CiteScore

21.30

自引率

2.30%

发文量

277

审稿时长

1 months

期刊介绍： Autophagy is a peer-reviewed journal that publishes research on autophagic processes, including the lysosome/vacuole dependent degradation of intracellular material. It aims to be the premier journal in the field and covers various connections between autophagy and human health and disease, such as cancer, neurodegeneration, aging, diabetes, myopathies, and heart disease. Autophagy is interested in all experimental systems, from yeast to human. Suggestions for specialized topics are welcome. The journal accepts the following types of articles: Original research, Reviews, Technical papers, Brief Reports, Addenda, Letters to the Editor, Commentaries and Views, and Articles on science and art. Autophagy is abstracted/indexed in Adis International Ltd (Reactions Weekly), EBSCOhost (Biological Abstracts), Elsevier BV (EMBASE and Scopus), PubMed, Biological Abstracts, Science Citation Index Expanded, Web of Science, and MEDLINE.