alpha-Synuclein dimerization in erythrocytes of Gaucher disease patients: correlation with lipid abnormalities and oxidative stress
| Autor: | Helen Michelakakis, Leonidas Stefanis, Nick Dekker, Georgia Dermentzaki, Ioannis Monopolis, Marina Moraitou, E. Dimitriou, Hans Aerts |
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| Přispěvatelé: | Amsterdam Cardiovascular Sciences, Amsterdam Gastroenterology Endocrinology Metabolism, Medical Biochemistry |
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
Adult
0301 basic medicine medicine.medical_specialty Ceramide Erythrocytes Parkinson's disease Adolescent Cell Plasmalogens Gaucher disease Ceramides medicine.disease_cause Young Adult 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Malondialdehyde Internal medicine medicine Humans Aged Alpha-synuclein General Neuroscience Middle Aged medicine.disease Lipids nervous system diseases Red blood cell 030104 developmental biology Endocrinology medicine.anatomical_structure Biochemistry chemistry nervous system Oxidative stress Case-Control Studies Child Preschool alpha-Synuclein Glucosylceramide Dimerization 030217 neurology & neurosurgery Homeostasis |
| Zdroj: | Neuroscience letters, 613, 1-5. Elsevier Ireland Ltd |
| ISSN: | 0304-3940 |
| Popis: | Several observations suggest that disturbed homeostasis of α-Synuclein (α-Syn) may provide a link between Gaucher disease (GD) and Parkinson's disease (PD). We recently reported increased dimerization of α-Syn in the red blood cell (RBC) membrane of patients with GD. Several studies indicate a crucial relationship between lipids, oxidative stress and α-Syn status. Here we investigated the relationship between the observed increased dimerization of α-Syn in the cell membranes of RBCs, cells devoid of lysosomes and lacking lysosomal enzyme synthesis, and the lipid abnormalities and oxidative stress already described in GD. Correlation studies showed that in GD the α-Syn dimer/monomer ratio is positively correlated with the levels of glucosylceramide (GlcCer) and the glucosylceramide/ceramide (GlcCer/Cer) ratio and negatively with the levels of malonyldialdehyde (MDA) and plasmalogens. In conclusion, we have shown that the increased tendency of α-Syn to form dimers in the RBC membrane of patients with GD, is correlated with both the level of lipids, including GlcCer, the primary lipid abnormality in GD, and the increased oxidative stress observed in this disorder. The study of other tissues, and in particular brain, will be important in order to elucidate the significance of these findings regarding the link between GD and PD. |
| Databáze: | OpenAIRE |
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