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Short definitions for reading the structure, metabolism, and disease guides.
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- Amide bond
- The linkage formed between a carboxyl-derived acyl group and nitrogen, joining a fatty acyl chain to the sphingoid base in ceramide.
- Amphipathic
- Having both hydrophilic and hydrophobic regions within one molecule.
- Arylsulfatase A
- A lysosomal sulfatase involved in sulfatide breakdown whose deficiency commonly causes metachromatic leukodystrophy.
- Ceramide
- An N-acyl sphingoid base that forms the core of many complex sphingolipids.
- Ceramide-1-phosphate
- A phosphorylated ceramide that retains its N-acyl chain and is distinct from S1P.
- Ceramide synthase
- An enzyme that N-acylates a sphingoid base using a fatty acyl-CoA substrate.
- Ceramidase
- An enzyme that hydrolyzes the amide bond of ceramide to release a sphingoid base and fatty acid.
- Cerebroside
- A glycosphingolipid with one hexose head group, commonly glucose or galactose.
- CERT
- The ceramide transfer protein that supports nonvesicular ceramide transport from the ER to the Golgi.
- Cholesterol
- A sterol that interacts with membrane lipids but is not a sphingolipid.
- De novo synthesis
- Production of a new sphingoid backbone from precursors such as serine and palmitoyl-CoA.
- Dihydroceramide
- An N-acylsphinganine that precedes sphingosine-based ceramide in the canonical de novo pathway.
- Ester bond
- A carboxylic-acid-derived linkage to oxygen, commonly attaching fatty acyl chains to glycerol.
- Galactosylceramide
- A monohexosylceramide with galactose as its head group and an important component of myelin.
- Ganglioside
- A glycosphingolipid containing one or more sialic acid residues.
- Globoside
- A neutral oligosaccharide-containing glycosphingolipid of the globo-series.
- Globotriaosylceramide
- The glycosphingolipid Gb3, a substrate of α-galactosidase A.
- Glucocerebrosidase
- The lysosomal acid β-glucosidase that cleaves glucosylceramide to ceramide and glucose.
- Glucosylceramide
- A ceramide bearing a glucose residue and a precursor of many complex glycosphingolipids.
- Glycosidic bond
- A bond from a sugar anomeric carbon to another group, including the linkage joining a sugar to ceramide.
- Glycosphingolipid
- A sphingolipid with a carbohydrate head group linked to a ceramide core.
- GM1
- A monosialylated ganglioside with a defined glycan that can bind cholera toxin.
- GM2
- A monosialylated ganglioside whose lysosomal degradation requires β-hexosaminidase A and GM2 activator protein.
- Lactosylceramide
- A glycosphingolipid with a galactose–glucose disaccharide linked to ceramide.
- Lipid raft
- A term for dynamic membrane assemblies enriched in sterols and sphingolipids in particular biological contexts.
- Lysosome
- An acidic cellular organelle involved in degradation and recycling of macromolecules and membrane components.
- Myelin
- A specialized multilayered membrane sheath that supports rapid nerve impulse conduction.
- Phosphatidylcholine
- A glycerophospholipid with a phosphocholine head group, commonly called lecithin.
- Phytosphingosine
- A sphingoid base with an additional hydroxyl group relative to sphinganine.
- Psychosine
- Galactosylsphingosine, a deacylated sphingolipid that accumulates in Krabbe disease.
- Salvage pathway
- Recycling of recovered sphingoid bases into ceramide and more complex sphingolipids.
- Saposin
- One of a family of small lipid-binding proteins that facilitate lysosomal sphingolipid degradation.
- Serine palmitoyltransferase
- The enzyme complex that catalyzes the initial condensation in canonical de novo sphingoid-base synthesis.
- Sialic acid
- A member of a family of acidic nine-carbon sugars present in gangliosides and other glycoconjugates.
- Sphinganine
- A saturated sphingoid base also called dihydrosphingosine.
- Sphingoid base
- A long-chain amino alcohol forming the defining backbone of a sphingolipid.
- Sphingomyelin
- A ceramide-based phospholipid commonly bearing a phosphocholine head group.
- Sphingosine
- A commonly 18-carbon sphingoid base with a trans double bond at C4–C5.
- Sphingosine-1-phosphate
- The phosphorylated sphingoid base S1P, with intracellular functions and extracellular receptor-mediated actions.
- Sulfatide
- A sulfated glycosphingolipid, commonly 3-O-sulfogalactosylceramide in mammals.
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References
Sources for further reading and verification. The update date records page revisions. No independent scientific peer review is claimed.
- Berg JM, Tymoczko JL, Stryer L. Biochemistry. 5th ed. W. H. Freeman; 2002. Chapters 12 and 26: lipids, membranes, and membrane lipid biosynthesis.
- Nelson DL, Cox MM. Lehninger Principles of Biochemistry. 8th ed. Macmillan Learning; 2021. Chapters 10 (Lipids) and 21 (Lipid Biosynthesis).
- Hannun YA, Obeid LM. Sphingolipids and their metabolism in physiology and disease. Nature Reviews Molecular Cell Biology. 2018;19:175–191. doi:10.1038/nrm.2017.107.
- Merrill AH Jr. Sphingolipid and glycosphingolipid metabolic pathways in the era of sphingolipidomics. Chemical Reviews. 2011;111:6387–6422. doi:10.1021/cr2002917.
- Schnaar RL, Sandhoff R, Tiemeyer M, Kinoshita T. Glycosphingolipids. In: Essentials of Glycobiology. 4th ed. Cold Spring Harbor Laboratory Press; 2022. Chapter 11.