Zobrazeno 1 - 10
of 146
pro vyhledávání: '"Lodewijk Ijlst"'
Autor:
Selva Turkolmez, Serhii Chornyi, Sondos Alhajouj, Lodewijk IJlst, Hans R. Waterham, Phil J. Mitchell, Ewald H. Hettema, Carlo W. T. van Roermund
Publikováno v:
Biomolecules, Vol 13, Iss 9, p 1294 (2023)
Debaryomyces hansenii is considered an unconventional yeast with a strong biotechnological potential, which can produce and store high amounts of lipids. However, relatively little is known about its lipid metabolism, and genetic tools for this yeast
Externí odkaz:
https://doaj.org/article/c074d709e88641dba553515120aebcff
Autor:
Carlo W. T. van Roermund, Lodewijk IJlst, Nicole Linka, Ronald J. A. Wanders, Hans R. Waterham
Publikováno v:
Frontiers in Cell and Developmental Biology, Vol 9 (2022)
Peroxisomes are essential organelles involved in various metabolic processes, including fatty acid β-oxidation. Their metabolic functions require a controlled exchange of metabolites and co-factors, including ATP, across the peroxisomal membrane. We
Externí odkaz:
https://doaj.org/article/8c7b751b90d943c080cc6dea25315715
Autor:
Serhii Chornyi, Lodewijk IJlst, Carlo W. T. van Roermund, Ronald J. A. Wanders, Hans R. Waterham
Publikováno v:
Frontiers in Cell and Developmental Biology, Vol 8 (2021)
Peroxisomes are membrane-bound organelles involved in many metabolic pathways and essential for human health. They harbor a large number of enzymes involved in the different pathways, thus requiring transport of substrates, products and cofactors inv
Externí odkaz:
https://doaj.org/article/b543db2b3f514cefb64adb53d4b66f98
Autor:
Arie O. Verkerk, Suzan J. G. Knottnerus, Vincent Portero, Jeannette C. Bleeker, Sacha Ferdinandusse, Kaomei Guan, Lodewijk IJlst, Gepke Visser, Ronald J. A. Wanders, Frits A. Wijburg, Connie R. Bezzina, Isabella Mengarelli, Riekelt H. Houtkooper
Publikováno v:
Frontiers in Pharmacology, Vol 11 (2021)
Patients with a deficiency in very long-chain acyl-CoA dehydrogenase (VLCAD), an enzyme that is involved in the mitochondrial beta-oxidation of long-chain fatty acids, are at risk for developing cardiac arrhythmias. In human induced pluripotent stem
Externí odkaz:
https://doaj.org/article/9bf15979cc1e485ca8dbed0f9ba01156
Autor:
Nadav Shai, Eden Yifrach, Carlo W. T. van Roermund, Nir Cohen, Chen Bibi, Lodewijk IJlst, Laetitia Cavellini, Julie Meurisse, Ramona Schuster, Lior Zada, Muriel C. Mari, Fulvio M. Reggiori, Adam L. Hughes, Mafalda Escobar-Henriques, Mickael M. Cohen, Hans R. Waterham, Ronald J. A. Wanders, Maya Schuldiner, Einat Zalckvar
Publikováno v:
Nature Communications, Vol 9, Iss 1, Pp 1-13 (2018)
The internal organization of the cell has been enriched by the discovery that organelles establish membrane contact sites, however the entire repertoire of these contacts is still being explored. Here the authors systematically identify the landscape
Externí odkaz:
https://doaj.org/article/2dc37c819f6140ea903c97bd975857d0
Autor:
Suzan J. G. Knottnerus, Isabella Mengarelli, Rob C. I. Wüst, Antonius Baartscheer, Jeannette C. Bleeker, Ruben Coronel, Sacha Ferdinandusse, Kaomei Guan, Lodewijk IJlst, Wener Li, Xiaojing Luo, Vincent M. Portero, Ying Ulbricht, Gepke Visser, Ronald J. A. Wanders, Frits A. Wijburg, Arie O. Verkerk, Riekelt H. Houtkooper, Connie R. Bezzina
Publikováno v:
International Journal of Molecular Sciences, Vol 21, Iss 7, p 2589 (2020)
Patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) can present with life-threatening cardiac arrhythmias. The pathophysiological mechanism is unknown. We reprogrammed fibroblasts from one mildly and one severely affected VLCADD
Externí odkaz:
https://doaj.org/article/9291905b068a4faa9e19d8a94197632a
Autor:
Katharina Herzog, Lodewijk IJlst, Arno G. van Cruchten, Carlo W.T. van Roermund, Wim Kulik, Ronald J. A. Wanders, Hans R. Waterham
Publikováno v:
Metabolites, Vol 9, Iss 3, p 45 (2019)
Oxidative stress plays a role in the onset and progression of a number of diseases, such as Alzheimer’s disease, diabetes and cancer, as well as ageing. Oxidative stress is caused by an increased production of reactive oxygen species and reduced an
Externí odkaz:
https://doaj.org/article/471176eed8914716b9a07e6f1582ce7f
Autor:
Sacha Ferdinandusse, Simone Denis, Lodewijk IJlst, Georges Dacremont, Hans R. Waterham, Ronald J.A. Wanders
Publikováno v:
Journal of Lipid Research, Vol 41, Iss 11, Pp 1890-1896 (2000)
α-Methylacyl-CoA racemase plays an important role in the β-oxidation of branched-chain fatty acids and fatty acid derivatives because it catalyzes the conversion of several (2R)-methyl-branched-chain fatty acyl-CoAs to their (S)-stereoisomers. Only
Externí odkaz:
https://doaj.org/article/7f873e2ce35b44028f1eff8a23853ca2
Autor:
Marit Schwantje, Sabine A. Fuchs, Lonneke de Boer, Annet M. Bosch, Inge Cuppen, Eugenie Dekkers, Terry G. J. Derks, Sacha Ferdinandusse, Lodewijk Ijlst, Riekelt H. Houtkooper, Rose Maase, W. Ludo van der Pol, Maaike C. de Vries, Rendelien K. Verschoof‐Puite, Ronald J. A. Wanders, Monique Williams, Frits Wijburg, Gepke Visser
Publikováno v:
Schwantje, M, Fuchs, S A, de Boer, L, Bosch, A M, Cuppen, I, Dekkers, E, Derks, T G J, Ferdinandusse, S, Ijlst, L, Houtkooper, R H, Maase, R, van der Pol, W L, de Vries, M C, Verschoof-Puite, R K, Wanders, R J A, Williams, M, Wijburg, F & Visser, G 2022, ' Genetic, biochemical, and clinical spectrum of patients with mitochondrial trifunctional protein deficiency identified after the introduction of newborn screening in the Netherlands ', Journal of Inherited Metabolic Disease, vol. 45, no. 4, pp. 804-818 . https://doi.org/10.1002/jimd.12502
Journal of Inherited Metabolic Disease, 45(4), 804-818. Springer Netherlands
Journal of inherited metabolic disease, 45(4), 804-818. Springer Netherlands
Journal of Inherited Metabolic Disease, 45(4), 804-818. SPRINGER
Journal of Inherited Metabolic Disease, 45, 804-818
Journal of Inherited Metabolic Disease, 45, 4, pp. 804-818
Journal of Inherited Metabolic Disease, 45(4), 804-818. Springer Netherlands
Journal of inherited metabolic disease, 45(4), 804-818. Springer Netherlands
Journal of Inherited Metabolic Disease, 45(4), 804-818. SPRINGER
Journal of Inherited Metabolic Disease, 45, 804-818
Journal of Inherited Metabolic Disease, 45, 4, pp. 804-818
Contains fulltext : 283151.pdf (Publisher’s version ) (Open Access) Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is included in many newborn screening (NBS) programs. Acylcarnitine-based NBS for LCHADD not only identifies LCHADD,
Autor:
Lodewijk IJlst, Loek L. Crefcoeur, Riekelt H. Houtkooper, Jeannette C. Bleeker, Frédéric M. Vaz, Sacha Ferdinandusse, Johannes B. van Goudoever, Dewi van Harskamp, Henk Schierbeek, Suzan J. G. Knottnerus, Frits A. Wijburg, Ronald J.A. Wanders, Gepke Visser, Mirjam Langeveld
Publikováno v:
Clinical nutrition (Edinburgh, Scotland), 40(3), 1396-1404. Churchill Livingstone
Background & aims: Medium chain triglyceride (MCT) supplementation is often recommended as treatment for patients with long-chain fatty acid β-oxidation (lcFAO) disorders, since they can be utilized as an energy source without the use of the defecti