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Biochim Biophys Acta Mol Cell Biol Lipids. 2019 Apr;1864(4):512-521. doi: 10.1016/j.bbalip.2018.12.013. Epub 2019 Jan 6.

1-Deoxysphingolipids.

Author information

1
Institute for Clinical Chemistry, University Hospital Zurich, Switzerland; Zurich Center for Integrative Human Physiology (ZIHP), University of Zurich, Switzerland.
2
Institute for Clinical Chemistry, University Hospital Zurich, Switzerland; Zurich Center for Integrative Human Physiology (ZIHP), University of Zurich, Switzerland; iMed.ULisboa - Research Institute for Medicines, Faculdade de Farmácia, Universidade de Lisboa, Av. Prof. Gama Pinto, 1649-003 Lisboa, Portugal; Centro de Química-Física Molecular and IN-Institute of Nanoscience and Nanotechnology, Instituto Superior Técnico, Universidade de Lisboa, Lisboa, Portugal.
3
Neural Regeneration Laboratory, India Taylor Lipidomic Research Platform, Department of Biochemistry, Microbiology and Immunology, Ottawa Institute of Systems Biology, Ottawa Brain and Mind Research Institute, University of Ottawa, Canada.
4
iMed.ULisboa - Research Institute for Medicines, Faculdade de Farmácia, Universidade de Lisboa, Av. Prof. Gama Pinto, 1649-003 Lisboa, Portugal; Centro de Química-Física Molecular and IN-Institute of Nanoscience and Nanotechnology, Instituto Superior Técnico, Universidade de Lisboa, Lisboa, Portugal.
5
Institute for Clinical Chemistry, University Hospital Zurich, Switzerland; Zurich Center for Integrative Human Physiology (ZIHP), University of Zurich, Switzerland. Electronic address: thorsten.hornemann@usz.ch.

Abstract

Sphingolipids (SLs) are fundamental components of eukaryotic cells. 1-Deoxysphingolipids differ structurally from canonical SLs as they lack the essential C1-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.

KEYWORDS:

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

PMID:
30625374
DOI:
10.1016/j.bbalip.2018.12.013
[Indexed for MEDLINE]

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