Sphingoid bases and their involvement in neurodegenerative diseases

Laura Goins, Stefka Spassieva

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

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 languageEnglish
Pages (from-to)65-73
Number of pages9
JournalAdvances in Biological Regulation
Volume70
DOIs
StatePublished - Dec 2018

Bibliographical note

Publisher Copyright:
© 2018 Elsevier Ltd

Keywords

  • Long-chain bases
  • Lysosomal storage disorders
  • Neurodegeneration
  • Neuropathy
  • Sphingoid bases

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Genetics
  • Cancer Research

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