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Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle.
Knottnerus SJG, Bleeker JC, Wüst RCI, Ferdinandusse S, IJlst L, Wijburg FA, Wanders RJA, Visser G, Houtkooper RH. Knottnerus SJG, et al. Among authors: wust rci. Rev Endocr Metab Disord. 2018 Mar;19(1):93-106. doi: 10.1007/s11154-018-9448-1. Rev Endocr Metab Disord. 2018. PMID: 29926323 Free PMC article. Review.
A Defective Pentose Phosphate Pathway Reduces Inflammatory Macrophage Responses during Hypercholesterolemia.
Baardman J, Verberk SGS, Prange KHM, van Weeghel M, van der Velden S, Ryan DG, Wüst RCI, Neele AE, Speijer D, Denis SW, Witte ME, Houtkooper RH, O'neill LA, Knatko EV, Dinkova-Kostova AT, Lutgens E, de Winther MPJ, Van den Bossche J. Baardman J, et al. Among authors: wust rci. Cell Rep. 2018 Nov 20;25(8):2044-2052.e5. doi: 10.1016/j.celrep.2018.10.092. Cell Rep. 2018. PMID: 30463003 Free article.
Nutritional ketosis improves exercise metabolism in patients with very long-chain acyl-CoA dehydrogenase deficiency.
Bleeker JC, Visser G, Clarke K, Ferdinandusse S, de Haan FH, Houtkooper RH, IJlst L, Kok IL, Langeveld M, van der Pol WL, de Sain-van der Velden MGM, Sibeijn-Kuiper A, Takken T, Wanders RJA, van Weeghel M, Wijburg FA, van der Woude LH, Wüst RCI, Cox PJ, Jeneson JAL. Bleeker JC, et al. Among authors: wust rci. J Inherit Metab Dis. 2020 Jul;43(4):787-799. doi: 10.1002/jimd.12217. Epub 2020 Feb 5. J Inherit Metab Dis. 2020. PMID: 31955429 Free PMC article. Clinical Trial.
Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Can Be Improved by Lowering Accumulation of Fatty Acid Oxidation Intermediates.
Knottnerus SJG, Mengarelli I, Wüst RCI, Baartscheer A, Bleeker JC, Coronel R, Ferdinandusse S, Guan K, IJlst L, Li W, Luo X, Portero VM, Ulbricht Y, Visser G, Wanders RJA, Wijburg FA, Verkerk AO, Houtkooper RH, Bezzina CR. Knottnerus SJG, et al. Among authors: wust rci. Int J Mol Sci. 2020 Apr 8;21(7):2589. doi: 10.3390/ijms21072589. Int J Mol Sci. 2020. PMID: 32276429 Free PMC article.
79 results