Search Results - "Wanders, A."

Trans fatty acids and cardiovascular health: research completed? by Brouwer, I A, Wanders, A J, Katan, M B

“…This review asks the question if further research on trans fatty acids and cardiovascular health is needed. We therefore review the evidence from human studies…”
Get full text

Effects of dietary fibre on subjective appetite, energy intake and body weight: a systematic review of randomized controlled trials by Wanders, A. J, van den Borne, J. J. G. C, de Graaf, C, Hulshof, T, Jonathan, M. C, Kristensen, M, Mars, M, Schols, H. A, Feskens, E. J. M

“…Dietary fibres are believed to reduce subjective appetite, energy intake and body weight. However, different types of dietary fibre may affect these outcomes…”
Get full text

NAD+ homeostasis in human health and disease by Zapata‐Pérez, Rubén, Wanders, Ronald J A, van Karnebeek, Clara D M, Houtkooper, Riekelt H

“…Depletion of nicotinamide adenine dinucleotide (NAD + ), a central redox cofactor and the substrate of key metabolic enzymes, is the causative factor of a…”
Get full text

Systematic mapping of contact sites reveals tethers and a function for the peroxisome-mitochondria contact by Shai, Nadav, Yifrach, Eden, van Roermund, Carlo W. T., Cohen, Nir, Bibi, Chen, IJlst, Lodewijk, Cavellini, Laetitia, Meurisse, Julie, Schuster, Ramona, Zada, Lior, Mari, Muriel C., Reggiori, Fulvio M., Hughes, Adam L., Escobar-Henriques, Mafalda, Cohen, Mickael M., Waterham, Hans R., Wanders, Ronald J. A., Schuldiner, Maya, Zalckvar, Einat

“…The understanding that organelles are not floating in the cytosol, but rather held in an organized yet dynamic interplay through membrane contact sites, is…”
Get full text

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…”
Get full text

Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle by Knottnerus, Suzan J. G., Bleeker, Jeannette C., Wüst, Rob C. I., Ferdinandusse, Sacha, IJlst, Lodewijk, Wijburg, Frits A., Wanders, Ronald J. A., Visser, Gepke, Houtkooper, Riekelt H.

“…Mitochondrial fatty acid oxidation is an essential pathway for energy production, especially during prolonged fasting and sub-maximal exercise. Long-chain…”
Get full text

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…”
Get full text

Mutations in PCYT2 disrupt etherlipid biosynthesis and cause a complex hereditary spastic paraplegia by Vaz, Frédéric M, McDermott, John H, Alders, Mariëlle, Wortmann, Saskia B, Kölker, Stefan, Pras-Raves, Mia L, Vervaart, Martin A T, van Lenthe, Henk, Luyf, Angela C M, Elfrink, Hyung L, Metcalfe, Kay, Cuvertino, Sara, Clayton, Peter E, Yarwood, Rebecca, Lowe, Martin P, Lovell, Simon, Rogers, Richard C, van Kampen, Antoine H C, Ruiter, Jos P N, Wanders, Ronald J A, Ferdinandusse, Sacha, van Weeghel, Michel, Engelen, Marc, Banka, Siddharth

“…CTP:phosphoethanolamine cytidylyltransferase (ET), encoded by PCYT2, is the rate-limiting enzyme for phosphatidylethanolamine synthesis via the…”
Get full text

C26:0-Carnitine Is a New Biomarker for X-Linked Adrenoleukodystrophy in Mice and Man by van de Beek, Malu-Clair, Dijkstra, Inge M E, van Lenthe, Henk, Ofman, Rob, Goldhaber-Pasillas, Dalia, Schauer, Nicolas, Schackmann, Martin, Engelen-Lee, Joo-Yeon, Vaz, Frédéric M, Kulik, Wim, Wanders, Ronald J A, Engelen, Marc, Kemp, Stephan

“…X-linked adrenoleukodystrophy (ALD), a progressive neurodegenerative disease, is caused by mutations in ABCD1 and characterized by very-long-chain fatty acids…”
Get full text

The Brown-Vialetto-Van Laere and Fazio Londe syndrome revisited: natural history, genetics, treatment and future perspectives by Bosch, Annet M, Stroek, Kevin, Abeling, Nico G, Waterham, Hans R, Ijlst, Lodewijk, Wanders, Ronald J A

“…The Brown-Vialetto-Van Laere syndrome is a rare neurological disorder which may present at all ages with sensorineural deafness, bulbar palsy and respiratory…”
Get full text

Abnormal activation of MAPKs pathways and inhibition of autophagy in a group of patients with Zellweger spectrum disorders and X-linked adrenoleukodystrophy by Gragnaniello, Vincenza, Gueraldi, Daniela, Puma, Andrea, Commone, Anna, Cazzorla, Chiara, Loro, Christian, Porcù, Elena, Stornaiuolo, Maria, Miglioranza, Paolo, Salviati, Leonardo, Wanders, Ronald J. A, Burlina, Alberto

“…Abstract Background Zellweger spectrum disorders (ZSD) and X-linked adrenoleukodystrophy (X-ALD) are inherited metabolic diseases characterized by dysfunction…”
Get full text

Intrinsic acyl-CoA thioesterase activity of a peroxisomal ATP binding cassette transporter is required for transport and metabolism of fatty acids by De Marcos Lousa, Carine, van Roermund, Carlo W. T., Postis, Vincent L. G., Dietrich, Daniela, Kerr, Ian D., Wanders, Ronald J. A., Baldwin, Stephen A., Baker, Alison, Theodoulou, Frederica L.

“…Peroxisomes are organelles that perform diverse metabolic functions in different organisms, but a common function is β-oxidation of a variety of long chain…”
Get full text

enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results by Wanders, Ronald J. A, Ruiter, Jos P. N, IJlst, Lodewijk, Waterham, Hans R, Houten, Sander M

“…Oxidation of fatty acids in mitochondria is a key physiological process in higher eukaryotes including humans. The importance of the mitochondrial…”
Get full text

Satiety and energy intake after single and repeated exposure to gel-forming dietary fiber: post-ingestive effects by Wanders, A J, Mars, M, Borgonjen-van den Berg, K J, de Graaf, C, Feskens, E J M

“…Background: Viscous or gel-forming dietary fibers can increase satiety by a more firm texture and increased eating time. Effects of viscous or gel-forming…”
Get full text

Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance by Kemp, Stephan, Theodoulou, Frederica L, Wanders, Ronald JA

“…Peroxisomes are indispensable organelles in higher eukaryotes. They are essential for a number of important metabolic pathways, including fatty acid α‐ and…”
Get full text

Aberrant protein acylation is a common observation in inborn errors of acyl-CoA metabolism by Pougovkina, Olga, te Brinke, Heleen, Wanders, Ronald J. A., Houten, Sander M., de Boer, Vincent C. J.

“…Inherited disorders of acyl-CoA metabolism, such as defects in amino acid metabolism and fatty acid oxidation can present with severe clinical symptoms either…”
Get full text

The important role of biochemical and functional studies in the diagnostics of peroxisomal disorders by Ferdinandusse, Sacha, Ebberink, Merel S., Vaz, Frédéric M., Waterham, Hans R., Wanders, Ronald J. A.

“…Peroxisomes are dynamic organelles that play an essential role in a variety of metabolic pathways. Peroxisomal dysfunction can lead to various biochemical…”
Get full text

The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl–CoA esters by Roermund, Carlo W. T., Visser, Wouter F., IJlst, Lodewijk, Cruchten, Arno, Boek, Maxim, Kulik, Wim, Waterham, Hans R., Wanders, Ronald J. A.

“…Peroxisomes play a major role in human cellular lipid metabolism, including the β‐oxidation of fatty acids. The most frequent peroxisomal disorder is X‐linked…”
Get full text

Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: A review by Silva, M. F. B., Aires, C. C. P., Luis, P. B. M., Ruiter, J. P. N., IJlst, L., Duran, M., Wanders, R. J. A., Tavares de Almeida, I.

“…Summary Valproic acid (VPA; 2- n -propylpentanoic acid) is widely used as a major drug in the treatment of epilepsy and in the control of several types of…”
Get full text

Mice with a deficiency in Peroxisomal Membrane Protein 4 (PXMP4) display mild changes in hepatic lipid metabolism by Blankestijn, Maaike, Bloks, Vincent W., Struik, Dicky, Huijkman, Nicolette, Kloosterhuis, Niels, Wolters, Justina C., Wanders, Ronald J. A., Vaz, Frédéric M., Islinger, Markus, Kuipers, Folkert, van de Sluis, Bart, Groen, Albert K., Verkade, Henkjan J., Jonker, Johan W.

“…Peroxisomes play an important role in the metabolism of a variety of biomolecules, including lipids and bile acids. Peroxisomal Membrane Protein 4 (PXMP4) is a…”
Get full text