title: [Sphingolipids, vehicle for pathogenic agents and cause of genetic diseases].
abstract: Sphingolipids are present in all eukaryotic cells and share a sphingoid base : sphingosine. They were first discovered in 1884 and for a long time they were thought to participate to membrane structure only. Recently it has been established that they are mainly located in particular areas of the membrane called rafts which are signalling platforms. It has also been demonstrated that sphingolipids are receptors and second messengers. They play a crucial role in cellular functioning and are necessary to maintenance and developing of living organisms. However due to their receptor properties, they are also gateway for penetration of pathogenic agents such as virus (Ebola, HIV) or toxins (botulinium, tetanus). These agents first bind to glycosphingolipids or proteins mainly located in rafts. The complex so formed is required for the crossing of the membrane by the pathogenic agent. Sphingolipids metabolism is regulated by numerous enzymes. A failure in the activity of one of them induces an accumulation of sphingolipids known as sphingolipidoses. These are genetic diseases having severe consequences for the survival of the organism. The precise mechanisms of the sphingolipidoses are still mainly unknown which explains why few therapeutic strategies are available. These particular properties of lipids rafts and sphingolipids explain why a growing number of studies in the medical and scientific fields are devoted to them.
authors.affiliation: Département de Pharmacologie, Faculté de Médecine, Université de Montréal, Québec, Canada.
authors.forename: Caroline
authors.initials: C
authors.lastname: Fasano
authors.forename: Abel
authors.initials: A
authors.lastname: Hiol
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Miolan
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Niel
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000502:physiology; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology; D002462:Cell Membrane / Q000648:ultrastructure / Q000821:virology; D020275:Guillain-Barre Syndrome / Q000276:immunology; D006801:Humans; D008563:Membrane Lipids / Q000502:physiology; D021962:Membrane Microdomains; D009498:Neurotoxins / Q000493:pharmacokinetics; D051381:Rats; D011924:Rats, Zucker; D011956:Receptors, Cell Surface / Q000235:genetics / Q000502:physiology; D011991:Receptors, Virus / Q000502:physiology; D015290:Second Messenger Systems / Q000502:physiology; D013106:Sphingolipidoses / Q000145:classification / Q000235:genetics / Q000378:metabolism*; D013107:Sphingolipids / Q000276:immunology / Q000378:metabolism / Q000502:physiology*
chemical_list: D001324:Autoantigens; D008563:Membrane Lipids; D009498:Neurotoxins; D011956:Receptors, Cell Surface; D011991:Receptors, Virus; D013107:Sphingolipids