期刊
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
卷 112, 期 -, 页码 82-91出版社
ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD
DOI: 10.1016/j.semcdb.2020.10.008
关键词
Amyotrophic lateral sclerosis; Sphingolipids; Glucosylceramide; Glucocerebrosidase; Therapeutic target; Ambroxol
资金
- AREMANE
- FightMND
- Target ALS
- Andre combat la SLA
- Association pour la Recherche et le Developpement de Moyens de Lutte contre les Maladies Neurodegeneratives (AREMANE)
- Association francaise contre les myopathies (AFM)
- Association pour la Recherche et le Developpement de Moyens de Lutte contre les Maladies Neurodegeneratives
- Fight MND foundation
- FightMND Mid-Career Research Fellowship
- Australian NHMRC-ARC Dementia Research Leadership Fellowship
Sphingolipids play crucial roles in the development and maintenance of the nervous system, with dysregulated metabolism being linked to specific neurodegenerative diseases. Current research primarily focuses on ALS, seeking potential treatments that target sphingolipid metabolism in neurodegenerative diseases.
Sphingolipids are complex lipids. They play a structural role in neurons, but are also involved in regulating cellular communication, and neuronal differentiation and maturation. There is increasing evidence to suggest that dysregulated metabolism of sphingolipids is linked to neurodegenerative processes in amyotrophic lateral sclerosis (ALS), Parkinson?s disease and Gaucher?s disease. In this review, we provide an overview of the role of sphingolipids in the development and maintenance of the nervous system. We describe the implications of altered metabolism of sphingolipids in the pathophysiology of certain neurodegenerative diseases, with a primary focus on ALS. Finally, we provide an update of potential treatments that could be used to target the metabolism of sphingolipids in neurodegenerative diseases.
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