Abstract
Ceramides are a family of bioactive lipids belonging to the class of sphingolipids. Sphingolipidoses are a group of inherited genetic diseases characterized by the unmetabolized sphingolipids and the consequent reduction of ceramide pool in lysosomes. Sphingolipidoses include several disorders as Sandhoff disease, Fabry disease, Gaucher disease, metachromatic leukodystrophy, Krabbe disease, Niemann Pick disease, Farber disease, and GM2 gangliosidosis. In sphingolipidosis, lysosomal lipid storage occurs in both the central nervous system and visceral tissues, and central nervous system pathology is a common hallmark for all of them. Parkinson’s disease, the most common neurodegenerative movement disorder, is characterized by the accumulation and aggregation of misfolded α-synuclein that seem associated to some lysosomal disorders, in particular Gaucher disease. This review provides evidence into the role of ceramide metabolism in the pathophysiology of lysosomes, highlighting the more recent findings on its involvement in Parkinson’s disease.
Highlights
Ceramides (Cers) are a family of bioactive lipids belonging to the class of sphingolipids (SphLs)
It might be possible that the inhibition of aCerase, increases the levels of Cer and cathepsin D (CatD) activity. In line with this hypothesis, there are data from Heinrich et al, who described enhanced CatD activity in cells derived from patients affected by Farber disease, whereas in acid SMase (aSMase) deficient cells derived from Niemann-Pick disease “type A” (NPA) patients, reduced CatD activity was found
We described the association between the altered ceramide catabolism and PD pathogenesis
Summary
Ceramides (Cers) are a family of bioactive lipids belonging to the class of sphingolipids (SphLs). Cers differ in their fatty acid composition (medium chain (C12–C14), long chain (C16–C18), very-long chain (C20–C24), and ultra-long chain (≥C26) and in their saturation state. SMase is a family of enzymes that are named on the basis of their optimal pH activity as acid, neutral, and alkaline SMase and are located in different subcellular Jc.oCmlinp. Cer is the backbone to synthesize GSLs, consisting of cerebrosides (Crbs), the simplest GSLs, sulfatides characterized by the presence in the molecule of a sulphate group, globosides (GBs), and gangliosides (Gs), the most complex GSLs. In Crb, Cer is linked to a single monosaccharide commonly galactose (galactocerebroside, GalC) or glucose (glucocerebroside, GC) synthesized by galactosylceramide-synthase (GalCerS) and glucosylceramide-synthase (GluCerS), respectively. Among over 60 known natural Gs, monosialo-tetrahexosyl-ganglioside (GM1), GD1a, and GD1b are the most common ones [15]
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