Lipids, lysosomes and mitochondria: insights into Lewy body formation from rare monogenic disorders
| العنوان: | Lipids, lysosomes and mitochondria: insights into Lewy body formation from rare monogenic disorders |
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| المؤلفون: | Daniel Erskine, David J. Koss, Johannes Attems, Viktor I. Korolchuk, Ian G. McKeith, Tiago F. Outeiro |
| المصدر: | Acta Neuropathologica Acta neuropathologica 141(4), 511-526 (2021). doi:10.1007/s00401-021-02266-7 |
| بيانات النشر: | Springer Berlin Heidelberg, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Mitochondrial Diseases, Context (language use), Disease, Review, Biology, Mitochondrion, pathology [Mitochondria], Pathology and Forensic Medicine, metabolism [Lysosomes], Alpha-synuclein, Cellular and Molecular Neuroscience, chemistry.chemical_compound, medicine, Autophagy, Dementia, Humans, ddc:610, metabolism [alpha-Synuclein], pathology [Lysosomal Storage Diseases], Synucleinopathies, pathology [Lewy Bodies], Lewy body, Catabolism, pathology [Hemochromatosis], metabolism [Lewy Bodies], metabolism [Mitochondria], medicine.disease, Lipid Metabolism, Cell biology, Mitochondria, Lysosomal Storage Diseases, metabolism [Lysosomal Storage Diseases], pathology [Mitochondrial Diseases], Lipid metabolism, chemistry, metabolism [Mitochondrial Diseases], Lewy Bodies, Neurology (clinical), Hemochromatosis, physiology [Lipid Metabolism], Lysosomes, metabolism [Hemochromatosis], pathology [Lysosomes] |
| الوصف: | Accumulation of the protein α-synuclein into insoluble intracellular deposits termed Lewy bodies (LBs) is the characteristic neuropathological feature of LB diseases, such as Parkinson’s disease (PD), Parkinson’s disease dementia (PDD) and dementia with LB (DLB). α-Synuclein aggregation is thought to be a critical pathogenic event in the aetiology of LB disease, based on genetic analyses, fundamental studies using model systems, and the observation of LB pathology in post-mortem tissue. However, some monogenic disorders not traditionally characterised as synucleinopathies, such as lysosomal storage disorders, iron storage disorders and mitochondrial diseases, appear disproportionately vulnerable to the deposition of LBs, perhaps suggesting the process of LB formation may be a result of processes perturbed as a result of these conditions. The present review discusses biological pathways common to monogenic disorders associated with LB formation, identifying catabolic processes, particularly related to lipid homeostasis, autophagy and mitochondrial function, as processes that could contribute to LB formation. These findings are discussed in the context of known mediators of α-synuclein aggregation, highlighting the potential influence of impairments to these processes in the aetiology of LB formation. |
| اللغة: | English |
| تدمد: | 1432-0533 0001-6322 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::43306bec4e1cde40566d68af6e3b9796 http://europepmc.org/articles/PMC7952289 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....43306bec4e1cde40566d68af6e3b9796 |
| قاعدة البيانات: | OpenAIRE |
Find this article in full text from Springer Verlag. | تدمد: | 14320533 00016322 |
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