دورية أكاديمية
أعرض في EDS

TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: From pathomechanisms to therapeutic strategies.

التفاصيل البيبلوغرافية
العنوان: TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: From pathomechanisms to therapeutic strategies.
المؤلفون: Ho PC; Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan., Hsieh TC; Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan., Tsai KJ; Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Research Center of Clinical Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan. Electronic address: kjtsai@mail.ncku.edu.tw.
المصدر: Ageing research reviews [Ageing Res Rev] 2024 Sep; Vol. 100, pp. 102441. Date of Electronic Publication: 2024 Jul 27.
نوع المنشور: Journal Article; Review
اللغة: English
بيانات الدورية: Publisher: Elsevier Science Country of Publication: England NLM ID: 101128963 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1872-9649 (Electronic) Linking ISSN: 15681637 NLM ISO Abbreviation: Ageing Res Rev Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Oxford, UK : Elsevier Science, c2002-
مواضيع طبية MeSH: Amyotrophic Lateral Sclerosis*/metabolism , Amyotrophic Lateral Sclerosis*/pathology , Amyotrophic Lateral Sclerosis*/genetics , Amyotrophic Lateral Sclerosis*/therapy , Frontotemporal Lobar Degeneration*/metabolism , Frontotemporal Lobar Degeneration*/pathology , Frontotemporal Lobar Degeneration*/therapy , Frontotemporal Lobar Degeneration*/genetics , TDP-43 Proteinopathies*/metabolism , TDP-43 Proteinopathies*/pathology , TDP-43 Proteinopathies*/genetics , DNA-Binding Proteins*/metabolism , DNA-Binding Proteins*/genetics, Humans ; Animals ; Autophagy/physiology
مستخلص: Proteostasis failure is a common pathological characteristic in neurodegenerative diseases. Revitalizing clearance systems could effectively mitigate these diseases. The transactivation response (TAR) DNA-binding protein 43 (TDP-43) plays a critical role as an RNA/DNA-binding protein in RNA metabolism and synaptic function. Accumulation of TDP-43 aggregates in the central nervous system is a hallmark of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Autophagy, a major and highly conserved degradation pathway, holds the potential for degrading aggregated TDP-43 and alleviating FTLD/ALS. This review explores the causes of TDP-43 aggregation, FTLD/ALS-related genes, key autophagy factors, and autophagy-based therapeutic strategies targeting TDP-43 proteinopathy. Understanding the underlying pathological mechanisms of TDP-43 proteinopathy can facilitate therapeutic interventions.
Competing Interests: Declaration of Competing Interest The authors declare no conflict of interests.
(Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)
فهرسة مساهمة: Keywords: Amyotrophic lateral sclerosis; Autophagy; Frontotemporal lobar degeneration; TDP-43
المشرفين على المادة: 0 (DNA-Binding Proteins)
0 (TARDBP protein, human)
تواريخ الأحداث: Date Created: 20240728 Date Completed: 20240909 Latest Revision: 20240909
رمز التحديث: 20240910
DOI: 10.1016/j.arr.2024.102441
PMID: 39069095
قاعدة البيانات: MEDLINE
الوصف
تدمد:1872-9649
DOI:10.1016/j.arr.2024.102441