Abstract
Sphingoid bases (also known as long-chain bases) form the backbone of sphingolipids. Sphingolipids comprise a large group of lipid molecules, which function as the building blocks of biological membranes and play important signaling and regulatory roles within cells. The roles of sphingoid bases in neurotoxicity and neurodegeneration have yet to be fully elucidated, as they are complex and multi-faceted. This comprises a new frontier of research that may provide us with important clues regarding their involvement in neurological health and disease. This paper explores various neurological diseases and conditions which result when the metabolism of sphingoid bases and some of their derivatives, such as sphingosine-1-phosphate and psychosine, becomes compromised due to the inhibition or mutation of key enzymes. Dysregulation of sphingoid base metabolism very often manifests with neurological symptoms, as sphingolipids are highly enriched in the nervous system, where they play important signaling and regulatory roles.
Original language | English |
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Pages (from-to) | 65-73 |
Number of pages | 9 |
Journal | Advances in Biological Regulation |
Volume | 70 |
DOIs | |
State | Published - Dec 2018 |
Bibliographical note
Publisher Copyright:© 2018 Elsevier Ltd
Funding
This work was the supported in part by the National Institutes of Health grants NIH R01AG034389 and R01NS095215. The authors also acknowledge support by the Department of Physiology (Chair Dr. Alan Daugherty), University of Kentucky, Lexington, KY. This work was the supported in part by the National Institutes of Health grants NIH R01AG034389 and R01NS095215 . The authors also acknowledge support by the Department of Physiology (Chair Dr. Alan Daugherty), University of Kentucky , Lexington, KY.
Funders | Funder number |
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Department of Physiology | |
National Institutes of Health (NIH) | R01AG034389 |
National Institute of Neurological Disorders and Stroke | R01NS095215 |
University of Kentucky | |
Department of Physiology and Biophysics, University at Buffalo |
Keywords
- Long-chain bases
- Lysosomal storage disorders
- Neurodegeneration
- Neuropathy
- Sphingoid bases
ASJC Scopus subject areas
- Molecular Medicine
- Molecular Biology
- Genetics
- Cancer Research