期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
卷 1864, 期 4, 页码 512-521出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbalip.2018.12.013
关键词
Serine; Alanine; Glycine; Sphingolipid metabolism; Atypical sphingolipids; HSAN1; T2DM
资金
- Swiss National Foundation SNF [31003A3_53390/1]
- Herzog Egli Foundation
- Rare Disease Initiative Zurich (radiz, Clinical Research Priority Program for Rare Diseases, University of Zurich)
- Fundacao para a Ciencia e a Tecnologia (FCT), Portugal [PTDC/BBB-BQB/3710/2014, SFRH/BD/102933/2014, IF/00437/2014]
- Fundação para a Ciência e a Tecnologia [SFRH/BD/102933/2014] Funding Source: FCT
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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