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Sphingolipids in neurodegenerative diseases
Neurodegenerative Diseases (NDDs) are a group of disorders that cause progressive deficits of neuronal function. Recent evidence argues that sphingolipid metabolism is affected in a surprisingly broad set of NDDs. These include some lysosomal storage diseases (LSDs), hereditary sensory and autonomou...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9978793/ https://www.ncbi.nlm.nih.gov/pubmed/36875645 http://dx.doi.org/10.3389/fnins.2023.1137893 |
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author | Pan, Xueyang Dutta, Debdeep Lu, Shenzhao Bellen, Hugo J. |
author_facet | Pan, Xueyang Dutta, Debdeep Lu, Shenzhao Bellen, Hugo J. |
author_sort | Pan, Xueyang |
collection | PubMed |
description | Neurodegenerative Diseases (NDDs) are a group of disorders that cause progressive deficits of neuronal function. Recent evidence argues that sphingolipid metabolism is affected in a surprisingly broad set of NDDs. These include some lysosomal storage diseases (LSDs), hereditary sensory and autonomous neuropathy (HSAN), hereditary spastic paraplegia (HSP), infantile neuroaxonal dystrophy (INAD), Friedreich’s ataxia (FRDA), as well as some forms of amyotrophic lateral sclerosis (ALS) and Parkinson’s disease (PD). Many of these diseases have been modeled in Drosophila melanogaster and are associated with elevated levels of ceramides. Similar changes have also been reported in vertebrate cells and mouse models. Here, we summarize studies using fly models and/or patient samples which demonstrate the nature of the defects in sphingolipid metabolism, the organelles that are implicated, the cell types that are initially affected, and potential therapeutics for these diseases. |
format | Online Article Text |
id | pubmed-9978793 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-99787932023-03-03 Sphingolipids in neurodegenerative diseases Pan, Xueyang Dutta, Debdeep Lu, Shenzhao Bellen, Hugo J. Front Neurosci Neuroscience Neurodegenerative Diseases (NDDs) are a group of disorders that cause progressive deficits of neuronal function. Recent evidence argues that sphingolipid metabolism is affected in a surprisingly broad set of NDDs. These include some lysosomal storage diseases (LSDs), hereditary sensory and autonomous neuropathy (HSAN), hereditary spastic paraplegia (HSP), infantile neuroaxonal dystrophy (INAD), Friedreich’s ataxia (FRDA), as well as some forms of amyotrophic lateral sclerosis (ALS) and Parkinson’s disease (PD). Many of these diseases have been modeled in Drosophila melanogaster and are associated with elevated levels of ceramides. Similar changes have also been reported in vertebrate cells and mouse models. Here, we summarize studies using fly models and/or patient samples which demonstrate the nature of the defects in sphingolipid metabolism, the organelles that are implicated, the cell types that are initially affected, and potential therapeutics for these diseases. Frontiers Media S.A. 2023-02-16 /pmc/articles/PMC9978793/ /pubmed/36875645 http://dx.doi.org/10.3389/fnins.2023.1137893 Text en Copyright © 2023 Pan, Dutta, Lu and Bellen. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neuroscience Pan, Xueyang Dutta, Debdeep Lu, Shenzhao Bellen, Hugo J. Sphingolipids in neurodegenerative diseases |
title | Sphingolipids in neurodegenerative diseases |
title_full | Sphingolipids in neurodegenerative diseases |
title_fullStr | Sphingolipids in neurodegenerative diseases |
title_full_unstemmed | Sphingolipids in neurodegenerative diseases |
title_short | Sphingolipids in neurodegenerative diseases |
title_sort | sphingolipids in neurodegenerative diseases |
topic | Neuroscience |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9978793/ https://www.ncbi.nlm.nih.gov/pubmed/36875645 http://dx.doi.org/10.3389/fnins.2023.1137893 |
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