C9-ALS-FTD 模型中内质网介导的自噬体生物发生障碍。
Disrupted endoplasmic reticulum-mediated autophagosomal biogenesis in a model of C9-ALS-FTD.
机构信息
Department of Neurology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
The Solomon H. Snyder Department of Neuroscience, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
出版信息
Autophagy. 2024 Jan;20(1):94-113. doi: 10.1080/15548627.2023.2249750. Epub 2023 Aug 27.
3R: UAS construct expressing 3 GC repeats (used as control); 3WJ: three-way junction; 12R: UAS construct expressing leader sequence and 12 GC repeats; 30R: UAS construct expressing 30 GC repeats; 36R: UAS construct expressing 36 GC repeats; 44R: UAS construct expressing leader sequence and 44 GC repeats; ALS: amyotrophic lateral sclerosis; Atg: autophagy related; atl: atlastin; C9-ALS-FTD: ALS or FTD caused by hexanuleotide repeat expansion in ; 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 GC 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 ( ortholog of TFEB); TrpA1: transient receptor potential cation channel A1; VAPB: VAMP associated protein B and C; VNC: ventral nerve cord (spinal cord in larvae).
3R：表达 3 个 GC 重复的 UAS 构建体（用作对照）；3WJ：三链结；12R：表达启动子序列和 12 个 GC 重复的 UAS 构建体；30R：表达 30 个 GC 重复的 UAS 构建体；36R：表达 36 个 GC 重复的 UAS 构建体；44R：表达启动子序列和 44 个 GC 重复的 UAS 构建体；ALS：肌萎缩性侧索硬化症；Atg：自噬相关；atl：atlastin；C9-ALS-FTD：由六核苷酸重复扩展引起的 ALS 或 FTD；ER：内质网；FTD：额颞叶痴呆；HRE：GGGGCC 六核苷酸重复扩展；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：Ras 相关结合 GTPase；RAN：重复相关非 AUG（RAN）翻译；RO-36：在 UAS 构建体中表达“仅 RNA”版本的 36 个 GC 重复，其中所有六个阅读框中的终止密码子都被插入；Rtnl1：网蛋白样 1；SN：节段神经；TFEB/Mitf：转录因子 EB/小眼相关转录因子（TFEB 的同源物）；TrpA1：瞬时受体电位阳离子通道 A1；VAPB：VAMP 相关蛋白 B 和 C；VNC：腹神经索（幼虫的脊髓）。
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