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15 results on '"Engelen-Lee, Joo-Yeon"'

5. Metabolic rerouting via SCD1 induction impacts X-linked adrenoleukodystrophy

Author

Raas, Quentin, van de Beek, Malu-Clair, Forss-Petter, Sonja, Dijkstra, Inge M.E., Deschiffart, Abigail, Freshner, Briana C., Stevenson, Tamara J., Jaspers, Yorrick R.J., Nagtzaam, Liselotte, Wanders, Ronald J.A., van Weeghel, Michel, Engelen-Lee, Joo-Yeon, Engelen, Marc, Eichler, Florian, Berger, Johannes, Bonkowsky, Joshua L., and Kemp, Stephan

Subjects
Adrenoleukodystrophy -- Genetic aspects -- Development and progression -- Care and treatment, Carrier proteins -- Health aspects, Cell metabolism -- Genetic aspects -- Health aspects, Oxidoreductases -- Health aspects, Health care industry
Abstract

X-linked adrenoleukodystrophy (ALD) is a progressive neurodegenerative disease caused by mutations in ABCD1, the peroxisomal very long-chain fatty acid (VLCFA) transporter. ABCD1 deficiency results in accumulation of saturated VLCFAs. A drug screen using a phenotypic motor assay in a zebrafish ALD model identified chloroquine as the top hit. Chloroquine increased expression of stearoyl-CoA desaturase-1 (scd1), the enzyme mediating fatty acid saturation status, suggesting that a shift toward monounsaturated fatty acids relieved toxicity. In human ALD fibroblasts, chloroquine also increased SCD1 levels and reduced saturated VLCFAs. Conversely, pharmacological inhibition of SCD1 expression led to an increase in saturated VLCFAs, and CRISPR knockout of scd1 in zebrafish mimicked the motor phenotype of ALD zebrafish. Importantly, saturated VLCFAs caused ER stress in ALD fibroblasts, whereas monounsaturated VLCFA did not. In parallel, we used liver X receptor (LXR) agonists to increase SCD1 expression, causing a shift from saturated toward monounsaturated VLCFA and normalizing phospholipid profiles. Finally, [Abcd.sup.1-/y] mice receiving LXR agonist in their diet had VLCFA reductions in ALD-relevant tissues. These results suggest that metabolic rerouting of saturated to monounsaturated VLCFAs may alleviate lipid toxicity, a strategy that may be beneficial in ALD and other peroxisomal diseases in which VLCFAs play a key role., Introduction X-linked adrenoleukodystrophy (ALD) is a neurometabolic disease caused by mutations in the ABCD1 gene encoding the peroxisomal CoA-activated very long-chain fatty acid (VLCFA) ([greater than or equal to] C22:0; [...]

Published
2021
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6. Microglial response in triggering receptor expressed on myeloid cells 2 (TREM2) knock-out mice after systemic stimulation with Escherichia coli

Author

Hoogland, Inge C.M., primary, Yik, Jutka, additional, Westhoff, Dunja, additional, Engelen-Lee, Joo-Yeon, additional, Valls Seron, Merche, additional, Man, Wing-Kit, additional, Houben-Weerts, Judith H.P.M., additional, Tanck, Michael W., additional, van Westerloo, David J., additional, van der Poll, Tom, additional, van Gool, Willem A., additional, and van de Beek, Diederik, additional

Published
2022
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11. Microglial Activation After Systemic Stimulation With Lipopolysaccharide and Escherichia coli

Author

Hoogland, Inge C. M., primary, Westhoff, Dunja, additional, Engelen-Lee, Joo-Yeon, additional, Melief, Jeroen, additional, Valls Serón, Mercedes, additional, Houben-Weerts, Judith H. M. P., additional, Huitinga, Inge, additional, van Westerloo, David J., additional, van der Poll, Tom, additional, van Gool, Willem A., additional, and van de Beek, Diederik, additional

Published
2018
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13. pIgR and PECAM-1 bind to pneumococcal adhesins RrgA and PspC mediating bacterial brain invasion

Author

Iovino, Federico, primary, Engelen-Lee, Joo-Yeon, additional, Brouwer, Matthijs, additional, van de Beek, Diederik, additional, van der Ende, Arie, additional, Valls Seron, Merche, additional, Mellroth, Peter, additional, Muschiol, Sandra, additional, Bergstrand, Jan, additional, Widengren, Jerker, additional, and Henriques-Normark, Birgitta, additional

Published
2017
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14. C26:0-Carnitine Is a New Biomarker for X-Linked Adrenoleukodystrophy in Mice and Man

Author

van de Beek, Malu-Clair, primary, Dijkstra, Inge M. E., additional, van Lenthe, Henk, additional, Ofman, Rob, additional, Goldhaber-Pasillas, Dalia, additional, Schauer, Nicolas, additional, Schackmann, Martin, additional, Engelen-Lee, Joo-Yeon, additional, Vaz, Frédéric M., additional, Kulik, Wim, additional, Wanders, Ronald J. A., additional, Engelen, Marc, additional, and Kemp, Stephan, additional

Published
2016
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