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This changed three decades ago when lipids were found to regulate cell polarity and vesicle transport, and the \u201clipid raft\u201d concept took shape. The lipid\u2010driven membrane anisotropy in form of \u201crafts\u201d that associate with proteins led to the view that organized complexes of lipids and proteins regulate various cell functions. Disturbance of this organization can lead to cellular, tissue, and organ malfunction. Sphingolipidoses, lysosomal storage diseases that are caused by enzyme deficiencies in the sphingolipid degradation pathway, were found to be particularly detrimental to the brain. These enzyme deficiencies result in accumulation of sphingolipid metabolites in lysosomes, although it is not yet clear how this accumulation affects the organization of lipids in cellular membranes. Krabbe's disease (KD), or globoid cell leukodystrophy, was one of the first sphingolipidosis for which the raft concept offered a potential mechanism. KD is caused by mutations in the enzyme \u03b2\u2010galactocerebrosidase; however, elevation of its substrate, galactosylceramide, is not observed or considered detrimental. Instead, it was found that a byproduct of galactosylceramide metabolism, the lysosphingolipid psychosine, is accumulated. The \u201cpsychosine hypothesis\u201d has been refined by showing that psychosine disrupts lipid rafts and vesicular transport critical for the function of glia and neurons. The role of psychosine in KD is an example of how the disruption of sphingolipid metabolism can lead to elevation of a toxic lysosphingolipid, resulting in disruption of cellular membrane organization and neurotoxicity. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.23888","type":"journal-article","created":{"date-parts":[[2016,9,17]],"date-time":"2016-09-17T11:22:31Z","timestamp":1474111351000},"page":"974-981","source":"Crossref","is-referenced-by-count":42,"title":["Lysosphingolipids and sphingolipidoses: Psychosine in Krabbe's disease"],"prefix":"10.1002","volume":"94","author":[{"given":"Stefka","family":"Spassieva","sequence":"first","affiliation":[{"name":"Department of Molecular and Cellular Medicine Texas A&amp;M Health Science Center College Station Texas"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erhard","family":"Bieberich","sequence":"additional","affiliation":[{"name":"Department of Neuroscience and Regenerative Medicine Medical 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