دورية أكاديمية
أعرض في EDS

Protein Disulfide Isomerase Endoplasmic Reticulum Protein 57 (ERp57) is Protective Against ALS-Associated Mutant TDP-43 in Neuronal Cells.

التفاصيل البيبلوغرافية
العنوان: Protein Disulfide Isomerase Endoplasmic Reticulum Protein 57 (ERp57) is Protective Against ALS-Associated Mutant TDP-43 in Neuronal Cells.
المؤلفون: Parakh S; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Perri ER; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Vidal M; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Takalloo Z; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Jagaraj CJ; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Mehta P; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Yang S; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Thomas CJ; Department of Microbiology, Anatomy, Physiology and Pharmacology, School of Agriculture, Biomedicine and Environment, La Trobe University, Melbourne, VIC, 3086, Australia.; Centre for Cardiovascular Biology and Disease Research, La Trobe University, Melbourne, VIC, 3086, Australia., Blair IP; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia., Hong Y; La Trobe Institute for Molecular Science, La Trobe University, Melbourne, VIC, 3086, Australia., Atkin JD; Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, 2109, Australia. julie.atkin@mq.edu.au.; La Trobe Institute for Molecular Science, La Trobe University, Melbourne, VIC, 3086, Australia. julie.atkin@mq.edu.au.
المصدر: Neuromolecular medicine [Neuromolecular Med] 2024 Jun 11; Vol. 26 (1), pp. 23. Date of Electronic Publication: 2024 Jun 11.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Humana Press Country of Publication: United States NLM ID: 101135365 Publication Model: Electronic Cited Medium: Internet ISSN: 1559-1174 (Electronic) Linking ISSN: 15351084 NLM ISO Abbreviation: Neuromolecular Med Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Totowa, NJ : Humana Press, c2002-
مواضيع طبية MeSH: Protein Disulfide-Isomerases*/genetics , Protein Disulfide-Isomerases*/metabolism , Amyotrophic Lateral Sclerosis*/genetics , Amyotrophic Lateral Sclerosis*/metabolism , DNA-Binding Proteins*/genetics , DNA-Binding Proteins*/metabolism , Inclusion Bodies*/metabolism , Inclusion Bodies*/genetics, Humans ; Animals ; Mice ; Cell Nucleus/metabolism ; Cytoplasm/metabolism ; Neurons/metabolism ; Neurons/drug effects ; Superoxide Dismutase-1/genetics ; Mutation
مستخلص: Amyotrophic Lateral Sclerosis (ALS) is a severe neurodegenerative disease affecting motor neurons. Pathological forms of Tar-DNA binding protein-43 (TDP-43), involving its mislocalisation to the cytoplasm and the formation of misfolded inclusions, are present in almost all ALS cases (97%), and ~ 50% cases of the related condition, frontotemporal dementia (FTD), highlighting its importance in neurodegeneration. Previous studies have shown that endoplasmic reticulum protein 57 (ERp57), a member of the protein disulphide isomerase (PDI) family of redox chaperones, is protective against ALS-linked mutant superoxide dismutase (SOD1) in neuronal cells and transgenic SOD1 G93A mouse models. However, it remains unclear whether ERp57 is protective against pathological TDP-43 in ALS. Here, we demonstrate that ERp57 is protective against key features of TDP-43 pathology in neuronal cells. ERp57 inhibited the mislocalisation of TDP-43 M337V from the nucleus to the cytoplasm. In addition, ERp57 inhibited the number of inclusions formed by ALS-associated variant TDP-43 M337V and reduced the size of these inclusions. ERp57 was also protective against ER stress and induction of apoptosis. Furthermore, ERp57 modulated the steady-state expression levels of TDP-43. This study therefore demonstrates a novel mechanism of action of ERp57 in ALS. It also implies that ERp57 may have potential as a novel therapeutic target to prevent the TDP-43 pathology associated with neurodegeneration.
(© 2024. The Author(s).)
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فهرسة مساهمة: Keywords: ALS—Amyotrophic lateral sclerosis; ER stress; ERp57—Endoplasmic reticulum protein 57; PDI—Protein disulphide isomerase; TDP-43 pathology
المشرفين على المادة: EC 5.3.4.1 (Protein Disulfide-Isomerases)
0 (DNA-Binding Proteins)
0 (TARDBP protein, human)
EC 5.3.4.1. (PDIA3 protein, human)
EC 1.15.1.1 (Superoxide Dismutase-1)
تواريخ الأحداث: Date Created: 20240611 Date Completed: 20240611 Latest Revision: 20240711
رمز التحديث: 20240712
مُعرف محوري في PubMed: PMC11166824
DOI: 10.1007/s12017-024-08787-0
PMID: 38861223
قاعدة البيانات: MEDLINE
الوصف
تدمد:1559-1174
DOI:10.1007/s12017-024-08787-0