Sphingoid bases and their involvement in neurodegenerative diseases

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.