Search Results - "Wanders, Ronald J.A"

Phytanic acid metabolism in health and disease by Wanders, Ronald J.A., Komen, Jasper, Ferdinandusse, Sacha

“…Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid which cannot be beta-oxidized due to the presence of the first methyl…”
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A Lethal Defect of Mitochondrial and Peroxisomal Fission by Waterham, Hans R, Koster, Janet, van Roermund, Carlo W.T, Mooyer, Petra A.W, Wanders, Ronald J.A, Leonard, James V

“…Some types of neuropathy are caused by mutant dynamin proteins, which prevent the fusion of mitochondria. In this article, an infant with severe and lethal…”
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Metabolic rerouting via SCD1 induction impacts X-linked adrenoleukodystrophy by Raas, Quentin, van de Beek, Malu-Clair, Forss-Petter, Sonja, Dijkstra, Inge Me, Deschiffart, Abigail, Freshner, Briana C, Stevenson, Tamara J, Jaspers, Yorrick Rj, Nagtzaam, Liselotte, Wanders, Ronald Ja, van Weeghel, Michel, Engelen-Lee, Joo-Yeon, Engelen, Marc, Eichler, Florian, Berger, Johannes, Bonkowsky, Joshua L, Kemp, Stephan

“…X-linked adrenoleukodystrophy (ALD) is a progressive neurodegenerative disease caused by mutations in ABCD1, the peroxisomal very long-chain fatty acid (VLCFA)…”
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Cardiolipin provides an essential activating platform for caspase-8 on mitochondria by Gonzalvez, Francois, Schug, Zachary T, Houtkooper, Riekelt H, MacKenzie, Elaine D, Brooks, David G, Wanders, Ronald J.A, Petit, Patrice X, Vaz, Frédéric M, Gottlieb, Eyal

“…Cardiolipin is a mitochondria-specific phospholipid known to be intimately involved with apoptosis. However, the lack of appropriate cellular models to date…”
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Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient by Violante, Sara, IJlst, Lodewijk, te Brinke, Heleen, Koster, Janet, Tavares de Almeida, Isabel, Wanders, Ronald J.A., Ventura, Fátima V., Houten, Sander M.

“…Fatty acid β-oxidation may occur in both mitochondria and peroxisomes. While peroxisomes oxidize specific carboxylic acids such as very long-chain fatty acids,…”
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Bile acids: the role of peroxisomes by Ferdinandusse, Sacha, Denis, Simone, Faust, Phyllis L., Wanders, Ronald J.A.

“…It is well established that peroxisomes play a crucial role in de novo bile acid synthesis. Studies in patients with a peroxisomal disorder have been…”
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Functions and biosynthesis of plasmalogens in health and disease by Brites, Pedro, Waterham, Hans R, Wanders, Ronald J.A

“…Plasmalogens (1- O-alk-1′-enyl-2-acyl glycerophospholipids) constitute a special class of phospholipids characterized by the presence of a vinyl–ether bond at…”
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New insights on the mechanisms of valproate-induced hyperammonemia: Inhibition of hepatic N -acetylglutamate synthase activity by valproyl-CoA by Aires, Cátia C.P, van Cruchten, Arno, IJlst, Lodewijk, de Almeida, Isabel Tavares, Duran, Marinus, Wanders, Ronald J.A, Silva, Margarida F.B

“…Background & Aims Hyperammonemia is a frequent side-effect of valproic acid (VPA) therapy, which points to an imbalance between ammoniagenesis and ammonia…”
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Peroxisomes, peroxisomal diseases, and the hepatotoxicity induced by peroxisomal metabolites by Wanders, Ronald J A, Ferdinandusse, Sacha

“…The group of peroxisomal disorders represents a growing number of genetically determined diseases in humans in which there is an impairment in one or more…”
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Metabolic functions of peroxisomes in health and disease by Wanders, Ronald J.A.

“…Peroxisomes are subcellular organelles which are present in virtually every eukaryotic cell and catalyze a large number of metabolic functions. The importance…”
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The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders by Houten, Sander M, Violante, Sara, Ventura, Fatima V, Wanders, Ronald J.A

“…Mitochondrial fatty acid β-oxidation (FAO) is the major pathway for the degradation of fatty acids and is essential for maintaining energy homeostasis in the…”
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Neonatal carnitine concentrations in relation to gestational age and weight by Crefcoeur, Loek L., Sain‐van der Velden, Monique G. M., Ferdinandusse, Sacha, Langeveld, Mirjam, Maase, Rose, Vaz, Frédéric M., Visser, Gepke, Wanders, Ronald J.A., Wijburg, Frits A., Verschoof‐Puite, Rendelien K., Schielen, Peter C. J. I.

“…Background Free carnitine has been measured in the Dutch newborn screening (NBS) program since 2007 with a referral threshold of ≤5 μmol/L, regardless of…”
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Identification of PEX7 as the Second Gene Involved in Refsum Disease by van den Brink, Daan M., Brites, Pedro, Haasjes, Janet, Wierzbicki, Anthony S., Mitchell, John, Lambert-Hamill, Michelle, de Belleroche, Jacqueline, Jansen, Gerbert A., Waterham, Hans R., Ronald Wanders, J.A.

“…Patients affected with Refsum disease (RD) have elevated levels of phytanic acid due to a deficiency of the peroxisomal enzyme phytanoyl-CoA hydroxylase…”
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Analysis of very long-chain fatty acids using electrospray ionization mass spectrometry by Valianpour, Fredoen, Selhorst, Jacqueline J.M, van Lint, Lia E.M, van Gennip, Albert H, Wanders, Ronald J.A, Kemp, Stephan

“…Elevated levels of very long-chain fatty acids (VLCFA) in plasma and tissues are the biochemical hallmark for patients with X-linked adrenoleukodystrophy…”
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Biochemistry of mammalian peroxisomes revisited by Wanders, Ronald J A, Waterham, Hans R

“…In this review, we describe the current state of knowledge about the biochemistry of mammalian peroxisomes, especially human peroxisomes. The identification…”
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Monitoring of fatty aldehyde dehydrogenase by formation of pyrenedecanoic acid from pyrenedecanal by Keller, Markus A., Watschinger, Katrin, Golderer, Georg, Maglione, Manuel, Sarg, Bettina, Lindner, Herbert H., Werner-Felmayer, Gabriele, Terrinoni, Alessandro, Wanders, Ronald J.A., Werner, Ernst R.

“…Fatty aldehyde dehydrogenase (EC 1.2.1.48) converts long-chain fatty aldehydes to the corresponding acids. Deficiency in this enzyme causes the Sjogren Larsson…”
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Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies by Wang, Gang, McCain, Megan L, Yang, Luhan, He, Aibin, Pasqualini, Francesco Silvio, Agarwal, Ashutosh, Yuan, Hongyan, Jiang, Dawei, Zhang, Donghui, Zangi, Lior, Geva, Judith, Roberts, Amy E, Ma, Qing, Ding, Jian, Chen, Jinghai, Wang, Da-Zhi, Li, Kai, Wang, Jiwu, Wanders, Ronald J A, Kulik, Wim, Vaz, Frédéric M, Laflamme, Michael A, Murry, Charles E, Chien, Kenneth R, Kelley, Richard I, Church, George M, Parker, Kevin Kit, Pu, William T

“…Cardiomyocytes generated from induced pluripotent cells hold great promise for understanding and treating heart disease. William Pu and his colleagues apply…”
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Metabolic Interplay between Peroxisomes and Other Subcellular Organelles Including Mitochondria and the Endoplasmic Reticulum by Wanders, Ronald J A, Waterham, Hans R, Ferdinandusse, Sacha

“…Peroxisomes are unique subcellular organelles which play an indispensable role in several key metabolic pathways which include: (1.) etherphospholipid…”
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Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans by Wanders, Ronald J.A, Komen, Jasper, Kemp, Stephan

“…Fatty acids (FAs) can be degraded via different mechanisms including α-, β- and ω-oxidation. In humans, a range of different genetic diseases has been…”
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Mitochondrial protein acetylation is driven by acetyl-CoA from fatty acid oxidation by Pougovkina, Olga, te Brinke, Heleen, Ofman, Rob, van Cruchten, Arno G, Kulik, Wim, Wanders, Ronald J A, Houten, Sander M, de Boer, Vincent C J

“…Mitochondria integrate metabolic networks for maintaining bioenergetic requirements. Deregulation of mitochondrial metabolic networks can lead to mitochondrial…”
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