4.6 Review

1-Deoxysphingolipids

Publisher

ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbalip.2018.12.013

Keywords

Serine; Alanine; Glycine; Sphingolipid metabolism; Atypical sphingolipids; HSAN1; T2DM

Funding

  1. Swiss National Foundation SNF [31003A3_53390/1]
  2. Herzog Egli Foundation
  3. Rare Disease Initiative Zurich (radiz, Clinical Research Priority Program for Rare Diseases, University of Zurich)
  4. Fundacao para a Ciencia e a Tecnologia (FCT), Portugal [PTDC/BBB-BQB/3710/2014, SFRH/BD/102933/2014, IF/00437/2014]
  5. Fundação para a Ciência e a Tecnologia [SFRH/BD/102933/2014] Funding Source: FCT

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Sphingolipids (SLs) are fundamental components of eukaryotic cells. 1-Deoxysphingolipids differ structurally from canonical SLs as they lack the essential Cl-OH group. Consequently, 1-deoxysphingolipids cannot be converted to complex sphingolipids and are not degraded over the canonical catabolic pathways. Pathologically elevated 1-deoxySLs are involved in several disease conditions. Within this review, we will provide an up-to-date overview on the metabolic, physiological and pathophysiological aspects of this enigmatic class of headless sphingolipids.

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