C.W.T. van Roermund PhD publications

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Publications C.W.T. van Roermund PhD

Position
Research Associate
Main activities
Education, Research
Specialisation
Scientist: In vivo and in vitro transport of metabolites across the peroxisomal membrane
Focus of research

In recent years, much progress has been made with respect to the unravelling of the functions of peroxisomes in metabolism, and it is now well established that peroxisomes are indispensable organelles, especially in higher eukaryotes. Peroxisomes catalyse a number of essential metabolic functions including fatty acid beta-oxidation, ether phospholipid biosynthesis, fatty acid a-oxidation and glyoxylate detoxification. The involvement of peroxisomes in these metabolic pathways necessitates the transport of metabolites in and out of peroxisomes. Recently, considerable progress has been made in the characterization of metabolite transport across the peroxisomal membrane. Peroxisomes posses several specialized transport systems to transport metabolites. This is exemplified by the identification of a specific transporter for adenine nucleotides and several half-ABC (ATP-binding cassette) transporters (ABCD1-3) which may be present as hetero- and homo-dimers. The importance to investigate permeability properties of the peroxisomal membrane is underlined by the existence of a number of different genetic diseases. One of these peroxisomal disorders is X-linked adrenoleukodystrophy (X-ALD). The gene mutated in X-linked adrenoleukodystrophy (X-ALD) codes for the HsABCD1 protein, a member of the superfamily of ATP-binding cassette (ABC) transporters and required for fatty acid transport across the peroxisomal membrane. Although defective HsABCD1 results in the accumulation of very long-chain fatty acids in plasma of X-ALD patients, there is still no direct biochemical evidence that HsABCD1 actually transports very long-chain fatty acids.

To study the transport of fatty acids across the peroxisomal membrane and their metabolism in vivo we have chosen Saccharomyces cerevisiae as a model system. An important advantage of studies in the yeast S. cerevisiae is that peroxisomes are the only organelles in which beta-oxidation of fatty acids takes place, in contrast to the situation in mammalian cells. In the latter case peroxisomes as well as mitochondria participate in fatty acid oxidation. Secondly, we study the mechanism of metabolic transport in vitro by  functional reconstitution of peroxisomal transporters into proteoliposomes. The resolution of the mechanism of these transporters is utmost significance for our thinking about peroxisomal disorders e.g. X-linked adrenoleukodystrophy and its pathophysiological mechanisms and future therapies.

2020

  • Devarajan S., Meurer M., van Roermund C. W. T., Chen X., Hettema E. H., Kemp S., Knop M., Williams C. Proteasome-dependent protein quality control of the peroxisomal membrane protein Pxa1p BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES 2020;1862 (9) [PubMed]

2019

  • Herzog Katharina, Ijlst Lodewijk, van Cruchten Arno G., van Roermund Carlo W. T., Kulik Wim, Wanders Ronald J. A., Waterham Hans R. An UPLC-MS/MS assay to measure glutathione as marker for oxidative stress in cultured cells Metabolites 2019;9 (3) [PubMed]
  • Raja Vaishnavi, Salsaa Michael, Joshi Amit S., Li Yiran, van Roermund Carlo W. T., Saadat Nadia, Lazcano Pablo, Schmidtke Michael, Hüttemann Maik, Gupta Smiti V., Wanders Ronald J. A., Greenberg Miriam L. Cardiolipin-deficient cells depend on anaplerotic pathways to ameliorate defective TCA cycle function BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS 2019;1864 (5):654-661 [PubMed]
  • Raja Vaishnavi, Salsaa Michael, Joshi Amit S., Li Yiran, van Roermund Carlo W. T., Saadat Nadia, Lazcano Pablo, Schmidtke Michael, Hüttemann Maik, Gupta Smiti V., Wanders Ronald J. A., Greenberg Miriam L. Corrigendum to "Cardiolipin-deficient cells depend on anaplerotic pathways to ameliorate defective TCA cycle function" [Biochim. Biophys. Acta, Mol. Cell Biol. Lipids 1864/5(2019) 654-661] BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS 2019;1864 (8):1183 [PubMed]
  • Carrier David J., van Roermund Carlo W. T., Schaedler Theresia A., Rong Hong Lin, IJlst Lodewijk, Wanders Ronald J. A., Baldwin Stephen A., Waterham Hans R., Theodoulou Frederica L., Baker Alison Mutagenesis separates ATPase and thioesterase activities of the peroxisomal ABC transporter, Comatose Scientific reports 2019;9 (1) [PubMed]

2018

  • Ferdinandusse Sacha, Denis Simone, van Roermund Carlo W. T., Preece Mary Anne, Koster Janet, Ebberink Merel S., Waterham Hans R., Wanders Ronald J. A. A novel case of ACOX2 deficiency leads to recognition of a third human peroxisomal acyl-CoA oxidase BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE 2018;1864 (3):952-958 [PubMed]
  • 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 Systematic mapping of contact sites reveals tethers and a function for the peroxisome-mitochondria contact Nature communications 2018;9 (1) [PubMed]

2017

  • Ferdinandusse Sacha, Falkenberg Kim D., Koster Janet, Mooyer Petra A., Jones Richard, van Roermund Carlo W. T., Pizzino Amy, Schrader Michael, Wanders Ronald J. A., Vanderver Adeline, Waterham Hans R. ACBD5 deficiency causes a defect in peroxisomal very long-chain fatty acid metabolism Journal of medical genetics 2017;54 (5):330-337 [PubMed]
  • Al-Saryi Nadal A., Al-Hejjaj Murtakab Y., van Roermund Carlo W. T., Hulmes Georgia E., Ekal Lakhan, Payton Chantell, Wanders Ronald J. A., Hettema Ewald H. Two NAD-linked redox shuttles maintain the peroxisomal redox balance in Saccharomyces cerevisiae Scientific reports 2017;7 (1):11868 [PubMed]

2016

  • van Roermund Carlo W. T., Schroers Martin G., Wiese Jan, Facchinelli Fabio, Kurz Samantha, Wilkinson Sabrina, Charton Lennart, Wanders Ronald J. A., Waterham Hans R., Weber Andreas P. M., Link Nicole The Peroxisomal NAD Carrier from Arabidopsis Imports NAD in Exchange with AMP Plant physiology 2016;171 (3):2127-2139 [PubMed]

2015

  • Ferdinandusse Sacha, Jimenez-Sanchez Gerardo, Koster Janet, Denis Simone, van Roermund Carlo W., Silva-Zolezzi Irma, Moser Ann B., Visser Wouter F., Gulluoglu Mine, Durmaz Ozlem, Demirkol Mubeccel, Waterham Hans R., Gökcay Gülden, Wanders Ronald J. A., Valle David A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3 Human molecular genetics 2015;24 (2):361-370 [PubMed]
  • Baker Alison, Carrier David J., Schaedler Theresia, Waterham Hans R., van Roermund Carlo W., Theodoulou Frederica L. Peroxisomal ABC transporters: functions and mechanism Biochemical Society transactions 2015;43 (5):959-965 [PubMed]

2014

  • van Roermund Carlo W. T., Ijlst Lodewijk, Wagemans Tom, Wanders Ronald J. A., Waterham Hans R. A role for the human peroxisomal half-transporter ABCD3 in the oxidation of dicarboxylic acids BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS 2014;1841 (4):563-568 [PubMed]
  • Mendiondo Guillermina M., Medhurst Anne, van Roermund Carlo W., Zhang Xuebin, Devonshire Jean, Scholefield Duncan, Fernández José, Axcell Barry, Ramsay Luke, Waterham Hans R., Waugh Robbie, Theodoulou Frederica L., Holdsworth Michael J. Barley has two peroxisomal ABC transporters with multiple functions in β-oxidation Journal of experimental botany 2014;65 (17):4833-4847 [PubMed]
  • Geillon Flore, Gondcaille Catherine, Charbonnier Soëli, van Roermund Carlo W., Lopez Tatiana E., Dias Alexandre M. M., Pais de Barros Jean-Paul, Arnould Christine, Wanders Ronald J., Trompier Doriane, Savary Stéphane Structure-function analysis of peroxisomal ATP-binding cassette transporters using chimeric dimers Journal of biological chemistry 2014;289 (35):24511-24520 [PubMed]

2013

  • 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. Intrinsic acyl-CoA thioesterase activity of a peroxisomal ATP binding cassette transporter is required for transport and metabolism of fatty acids Proceedings of the National Academy of Sciences of the United States of America 2013;110 (4):1279-1284 [PubMed]
  • Lefevre Sophie D., van Roermund Carlo W., Wanders Ronald J. A., Veenhuis Marten, van der Klei Ida J. The significance of peroxisome function in chronological aging of Saccharomyces cerevisiae Aging cell 2013;12 (5):784-793 [PubMed]

2012

  • van Roermund Carlo W. T., Ijlst Lodewijk, Majczak Wiktor, Waterham Hans R., Folkerts Hendrik, Wanders Ronald J. A., Hellingwerf Klaas J. Peroxisomal Fatty Acid Uptake Mechanism in Saccharomyces cerevisiae Journal of biological chemistry 2012;287 (24):20144-20153 [PubMed]
  • Bartoszewska Magdalena, Williams Chris, Kikhney Alexey, Opaliński Łukasz, van Roermund Carlo W. T., de Boer Rinse, Veenhuis Marten, van der Klei Ida J. Peroxisomal Proteostasis Involves a Lon Family Protein That Functions as Protease and Chaperone Journal of biological chemistry 2012;287 (33):27380-27395 [PubMed]

2011

  • van Roermund Carlo W. T., Visser Wouter F., Ijlst Lodewijk, Waterham Hans R., Wanders Ronald J. A. Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS 2011;1811 (3):148-152 [PubMed]

2010

  • Strijbis Karin, van Roermund Carlo W., van den Burg Janny, van den Berg Marlene, Hardy Guy P. M., Wanders Ronald J., Distel Ben Contributions of Carnitine Acetyltransferases to Intracellular Acetyl Unit Transport in Candida albicans Journal of biological chemistry 2010;285 (32):24335-24346 [PubMed]
  • Nyathi Yvonne, de Marcos Lousa Carine, van Roermund Carlo W., Wanders Ronald J. A., Johnson Barbara, Baldwin Stephen A., Theodoulou Frederica L., Baker Alison The Arabidopsis peroxisomal ABC transporter, comatose, complements the Saccharomyces cerevisiae pxa1 pxa2& [delta] mutant for metabolism of long chain fatty acids and exhibits fatty acyl-coa stimulated atpase activity Journal of biological chemistry 2010;285 (39):29892-29902 [PubMed]
  • Ofman Rob, Dijkstra Inge M. E., van Roermund Carlo W. T., Burger Nena, Turkenburg Marjolein, van Cruchten Arno, van Engen Catherine E., Wanders Ronald J. A., Kemp Stephan The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy EMBO molecular medicine 2010;2 (3):90-97 [PubMed]

2009

  • Strijbis Karin, van Roermund Carlo W. T., Hardy Guy P., van den Burg Janny, Bloem Karien, de Haan Jolanda, van Vlies Naomi, Wanders Ronald J. A., Vaz Frédéric M., Distel Ben Identification and characterization of a complete carnitine biosynthesis pathway in Candida albicans FASEB journal 2009;23 (8):2349-2359 [PubMed]

2008

  • Strijbis Karin, van Roermund Carlo W. T., Visser Wouter F., Mol Els C., van den Burg Janny, Maccallum Donna M., Odds Frank C., Paramonova Ekaterina, Krom Bastiaan P., Distel Ben Carnitine-dependent transport of acetyl coenzyme A in Candida albicans is essential for growth on nonfermentable carbon sources and contributes to biofilm formation Eukaryotic cell 2008;7 (4):610-618 [PubMed]
  • Piekarska Katarzyna, Hardy Guy, Mol Els, van den Burg Janny, Strijbis Karin, van Roermund Carlo, van den Berg Marlene, Distel Ben The activity of the glyoxylate cycle in peroxisomes of Candida albicans depends on a functional beta-oxidation pathway: evidence for reduced metabolite transport across the peroxisomal membrane Microbiology (Reading, England) 2008;154 (Part 10):3061-3072 [PubMed]
  • van Roermund Carlo W. T., Visser Wouter F., Ijlst Lodewijk, van Cruchten Arno, Boek Maxim, Kulik Wim, Waterham Hans R., Wanders Ronald J. A. The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters FASEB journal 2008;22 (12):4201-4208 [PubMed]

2007

  • Waterham Hans R., Koster Janet, van Roermund Carlo W. T., Mooyer Petra A. W., Wanders Ronald J. A., Leonard James V. A lethal defect of mitochondrial and peroxisomal fission New England journal of medicine 2007;356 (17):1736-1741 [PubMed]
  • Ferdinandusse Sacha, Denis Simone, Hogenhout Eveline M., Koster Janet, van Roermund Carlo W. T., Ijlst Lodewijk, Moser Ann B., Wanders Ronald J. A., Waterham Hans R. Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency Human mutation 2007;28 (9):904-912 [PubMed]
  • Visser Wouter F., van Roermund Carlo W. T., Ijlst Lodewijk, Waterham Hans R., Wanders Ronald J. A. Demonstration of bile acid transport across the mammalian peroxisomal membrane Biochemical and biophysical research communications 2007;357 (2):335-340 [PubMed]
  • Visser Wouter F., van Roermund Carlo W. T., Ijlst Lodewijk, Waterham Hans R., Wanders Ronald J. A. Metabolite transport across the peroxisomal membrane Biochemical journal 2007;401 (2):365-375 [PubMed]
  • Wanders Ronald J. A., Visser Wouter F., van Roermund Carlo W. T., Kemp Stephan, Waterham Hans R. The peroxisomal ABC transporter family Pflugers Archiv 2007;453 (5):719-734 [PubMed]

2006

  • Visser Wouter F., van Roermund Carlo W. T., Ijlst Lodewijk, Hellingwerf Klaas J., Waterham Hans R., Wanders Ronald J. A. First identification of a 2-ketoglutarate/isocitrate transport system in mammalian peroxisomes and its characterization Biochemical and biophysical research communications 2006;348 (4):1224-1231 [PubMed]
  • Piekarska Katarzyna, Mol Els, van den Berg Marlene, Hardy Guy, van den Burg Janny, van Roermund Carlo, MacCallum Donna, Odds Frank, Distel Ben Peroxisomal fatty acid beta-oxidation is not essential for virulence of Candida albicans Eukaryotic cell 2006;5 (11):1847-1856 [PubMed]

2005

  • Visser Wouter F., van Roermund Carlo W., Ijlst Lodewijk, Hellingwerf Klaas J., Wanders Ronald J. A., Waterham Hans R. Demonstration and characterization of phosphate transport in mammalian peroxisomes Biochemical journal 2005;389 (Part 3):717-722 [PubMed]
  • Oey Nadia A., Ijlst Lodewijk, van Roermund Carlo W. T., Wijburg Frits A., Wanders Ronald J. A. dif-1 and colt, both implicated in early embryonic development, encode carnitine acylcarnitine translocase Molecular genetics and metabolism 2005;85 (2):121-124 [PubMed]
  • Hunt M. C., Ruiter J., Mooyer P., van Roermond C. W. T., Ofman R., IJlst L., Wanders R. J. A. Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblasts European journal of clinical investigation 2005;35 (1):38-46 [PubMed]
  • Spaan András N., Ijlst Lodewijk, van Roermund Carlo W. T., Wijburg Frits A., Wanders Ronald J. A., Waterham Hans R. Identification of the human mitochondrial FAD transporter and its potential role in multiple acyl-CoA dehydrogenase deficiency Molecular genetics and metabolism 2005;86 (4):441-447 [PubMed]
  • Visser W. F. Metabolite transport across the peroxisomal membrane 2005. 101p. [UvA Dissertations Online] University of Amsterdam. (Supervisor: Wanders R. J. A.; Co-supervisors: Waterham H. R., van Roermund C. W. T.)

2004

  • Ferdinandusse Sacha, Denis Simone, van Roermund Carlo W. T., Wanders Ronald J. A., Dacremont Georges Identification of the peroxisomal beta-oxidation enzymes involved in the degradation of long-chain dicarboxylic acids Journal of lipid research 2004;45 (6):1104-1111 [PubMed]
  • van Roermund Carlo W. T., de Jong Mark, Ijlst Lodewijk, van Marle Jan, Dansen Tobias B., Wanders Ronald J. A., Waterham Hans R. The peroxisomal lumen in Saccharomyces cerevisiae is alkaline Journal of cell science 2004;117 (Part 18):4231-4237 [PubMed]

2003

  • van Roermund C. W. T., Waterham H. R., IJlst L., Wanders R. J. A. Fatty acid metabolism in Saccharomyces cerevisiae Cellular and molecular life sciences 2003;60 (9):1838-1851 [PubMed]
  • Wanders Ronald J. A., van Roermund Carlo W. T., Visser Wouter F., Ferdinandusse Sacha, Jansen Gerbert A., van den Brink Daan M., Gloerich Jolein, Waterham Hans R. Peroxisomal fatty acid alpha- and beta-oxidation in health and disease: new insights Advances in experimental medicine and biology 2003;544:293-302 [PubMed]

2002

  • van Roermund C. W. T. Fatty acid beta-oxidation in Saccharomices cerevisae: new insights with implications for human diseases 2002. 120p. [UvA Dissertations Online] University of Amsterdam. (Supervisors: Wanders R. J. A., Tabak H. F.)
  • Visser W. F., van Roermund C. W. T., Waterham H. R., Wanders R. J. A. Identification of human PMP34 as a peroxisomal ATP transporter Biochemical and biophysical research communications 2002;299 (3):494-497 [PubMed]
  • Ferdinandusse S., van Grunsven E. G., Oostheim W., Denis S., Hogenhout E. M., IJlst L., van Roermund C. W. T., Waterham H. R., Goldfischer S., Wanders R. J. A. Reinvestigation of peroxisomal 3-ketoacyl-CoA thiolase deficiency: identification of the true defect at the level of d-bifunctional protein American journal of human genetics 2002;70 (6):1589-1593 [PubMed]

2001

  • IJlst L., van Roermund C. W., Iacobazzi V., Oostheim W., Ruiter J. P., Williams J. C., Palmieri F., Wanders R. J. Functional analysis of mutant human carnitine acylcarnitine translocases in yeast Biochemical and biophysical research communications 2001;280 (3):700-706 [PubMed]
  • van Roermund C. W., Drissen R., van den Berg M., IJlst L., Hettema E. H., Tabak H. F., Waterham H. R., Wanders R. J. Identification of a peroxisomal ATP carrier required for medium-chain fatty acid beta-oxidation and normal peroxisome proliferation in Saccharomyces cerevisiae Molecular and cellular biology 2001;21 (13):4321-4329 [PubMed]
  • Wanders R. J., Vreken P., Ferdinandusse S., Jansen G. A., Waterham H. R., van Roermund C. W., van Grunsven E. G. Peroxisomal fatty acid alpha- and beta-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases Biochemical Society transactions 2001;29 (Part 2):250-267 [PubMed]

2000

  • Pex11p plays a primary role in medium-chain fatty acid oxidation, a process that affects peroxisome number and size in Saccharomyces cerevisiae Journal of cell biology 2000;150 (3):489-498 [PubMed]

1999

  • van Roermund C. W., Hettema E. H., van den Berg M., Tabak H. F., Wanders R. J. Molecular characterization of carnitine-dependent transport of acetyl-CoA from peroxisomes to mitochondria in Saccharomyces cerevisiae and identification of a plasma membrane carnitine transporter, Agp2p EMBO journal 1999;18 (21):5843-5852 [PubMed]

1998

  • van Roermund C. W., Hettema E. H., Kal A. J., van den Berg M., Tabak H. F., Wanders R. J. Peroxisomal beta-oxidation of polyunsaturated fatty acids in Saccharomyces cerevisiae: isocitrate dehydrogenase provides NADPH for reduction of double bonds at even positions EMBO journal 1998;17 (3):677-687 [PubMed]

1997

  • Complementation analysis of fibroblasts from peroxisomal fatty acid oxidation deficient patients shows high frequency of bifunctional enzyme deficiency plus intragenic complementation: unequivocal evidence for differential defects in the same enzyme protein Biochemical and biophysical research communications 1997;235 (1):176-179 [PubMed]
  • Verleur N., Elgersma Y., van Roermund C. W., Tabak H. F., Wanders R. J. Cytosolic aspartate aminotransferase encoded by the AAT2 gene is targeted to the peroxisomes in oleate-grown Saccharomyces cerevisiae European Journal of Biochemistry / FEBS 1997;247 (3):972-980 [PubMed]
  • Verleur N., Hettema E. H., van Roermund C. W., Tabak H. F., Wanders R. J. Transport of activated fatty acids by the peroxisomal ATP-binding-cassette transporter Pxa2 in a semi-intact yeast cell system European Journal of Biochemistry / FEBS 1997;249 (3):657-661 [PubMed]

1996

  • Elgersma Y., Vos A., van den Berg M., van Roermund C. W., van der Sluijs P., Distel B., Tabak H. F. Analysis of the carboxyl-terminal peroxisomal targeting signal 1 in a homologous context in Saccharomyces cerevisiae Journal of biological chemistry 1996;271 (42):26375-26382 [PubMed]
  • Metabolic aspects of peroxisomal disorders Annals of the New York Academy of Sciences 1996;804:450-460 [PubMed]
  • Hettema E. H., van Roermund C. W., Distel B., van den Berg M., Vilela C., Rodrigues-Pousada C., Wanders R. J., Tabak H. F. The ABC transporter proteins Pat1 and Pat2 are required for import of long-chain fatty acids into peroxisomes of Saccharomyces cerevisiae EMBO journal 1996;15 (15):3813-3822 [PubMed]

1995

  • Localization of peroxisomal 3-oxoacyl-CoA thiolase in particles of varied density in rat liver: implications for peroxisome biogenesis BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS 1995;1245 (3):348-358 [PubMed]
  • Wanders R. J., Denis S., Ruiter J. P., Schutgens R. B., van Roermund C. W., Jacobs B. S. Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts Journal of inherited metabolic disease 1995;18 (Suppl. 1):113-124 [PubMed]
  • Elgersma Y., van Roermund C. W., Wanders R. J., Tabak H. F. Peroxisomal and mitochondrial carnitine acetyltransferases of Saccharomyces cerevisiae are encoded by a single gene EMBO journal 1995;14 (14):3472-3479 [PubMed]
  • Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome Journal of inherited metabolic disease 1995;18 (2):201-203 [PubMed]
  • van Roermund C. W., Elgersma Y., Singh N., Wanders R. J., Tabak H. F. The membrane of peroxisomes in Saccharomyces cerevisiae is impermeable to NAD(H) and acetyl-CoA under in vivo conditions EMBO journal 1995;14 (14):3480-3486 [PubMed]

1994

  • 2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE 1994;1227 (3):177-182 [PubMed]

1993

  • Subcellular fractionation of cultured normal human melanocytes: new insights into the relationship of melanosomes with lysosomes and peroxisomes BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE 1993;1181 (1):1-6 [PubMed]

1992

  • Bifunctional enzyme deficiency: identification of a new type of peroxisomal disorder in a patient with an impairment in peroxisomal beta-oxidation of unknown aetiology by means of complementation analysis Journal of inherited metabolic disease 1992;15 (3):385-388 [PubMed]
  • Characteristics and subcellular localization of pristanoyl-CoA synthetase in rat liver BIOCHIMICA ET BIOPHYSICA ACTA-LIPIDS AND LIPID METABOLISM 1992;1125 (3):274-279 [PubMed]
  • Molecular analysis of disorders of peroxisomal beta-oxidation Progress in clinical and biological research 1992;375:507-519 [PubMed]
  • X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect Journal of inherited metabolic disease 1992;15 (4):634-644 [PubMed]

1991

  • Accumulation and impaired in vivo metabolism of di- and trihydroxycholestanoic acid in two patients Clinica chimica acta; international journal of clinical chemistry 1991;202 (3):123-132 [PubMed]
  • Acyl-CoA oxidase, peroxisomal thiolase and dihydroxyacetone phosphate acyltransferase: aberrant subcellular localization in Zellweger syndrome Journal of inherited metabolic disease 1991;14 (2):152-164 [PubMed]
  • Di- and trihydroxycholestanaemia in twin sisters Journal of inherited metabolic disease 1991;14 (3):357-360 [PubMed]
  • Identification of pristanoyl-CoA oxidase and phytanic acid decarboxylation in peroxisomes and mitochondria from human liver: implications for Zellweger syndrome Journal of inherited metabolic disease 1991;14 (3):349-352 [PubMed]
  • Peroxisomal enzyme activities in the human hepatoblastoma cell line HepG2 as compared to human liver BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS 1991;1115 (1):54-59 [PubMed]

1990

  • A bifunctional protein with deficient enzymic activity: identification of a new peroxisomal disorder using novel methods to measure the peroxisomal beta-oxidation enzyme activities Journal of inherited metabolic disease 1990;13 (3):375-379 [PubMed]
  • Di- and trihydroxycholestanoic acidaemia with hepatic failure Journal of inherited metabolic disease 1990;13 (3):367-370 [PubMed]
  • Human liver L-alanine-glyoxylate aminotransferase: characteristics and activity in controls and hyperoxaluria type I patients using a simple spectrophotometric method Clinica chimica acta; international journal of clinical chemistry 1990;189 (2):139-144 [PubMed]
  • Identification of pristanoyl-CoA oxidase activity in human liver and its deficiency in the Zellweger syndrome Biochemical and biophysical research communications 1990;172 (2):490-495 [PubMed]
  • Inborn errors of peroxisomal beta-oxidation: biochemical identification using enzymic and immunological methods Progress in clinical and biological research 1990;321:559-567 [PubMed]
  • Heikoop J. C., van Roermund C. W., Just W. W., Ofman R., Schutgens R. B., Heymans H. S., Wanders R. J., Tager J. M. Rhizomelic chondrodysplasia punctata. Deficiency of 3-oxoacyl-coenzyme A thiolase in peroxisomes and impaired processing of the enzyme Journal of clinical investigation 1990;86 (1):126-130 [PubMed]
  • The inborn errors of peroxisomal beta-oxidation: a review Journal of inherited metabolic disease 1990;13 (1):4-36 [PubMed]

1989

  • Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases Journal of inherited metabolic disease 1989;12 (4):415-422 [PubMed]

1988

  • A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy) American journal of human genetics 1988;42 (3):422-434 [PubMed]
  • Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids Biochemical and biophysical research communications 1988;153 (2):618-624 [PubMed]
  • Diversity in residual alanine glyoxylate aminotransferase activity in hyperoxaluria type I: correlation with pyridoxine responsiveness Journal of inherited metabolic disease 1988;11 (Suppl. 2):208-211 [PubMed]
  • Wanders R. J., Romeyn G. J., van Roermund C. W., Schutgens R. B., van den Bosch H., Tager J. M. Identification of L-pipecolate oxidase in human liver and its deficiency in the Zellweger syndrome Biochemical and biophysical research communications 1988;154 (1):33-38 [PubMed]
  • Impaired ability of peroxisomes to activate very-long-chain fatty acids in X-linked adrenoleukodystrophy Lancet 1988;2 (8603):170 [PubMed]
  • Peroxisomes and peroxisomal functions in hyperpipecolic acidaemia Journal of inherited metabolic disease 1988;11 (Suppl. 2):161-164 [PubMed]
  • X-linked adrenoleukodystrophy: identification of the primary defect at the level of a deficient peroxisomal very long chain fatty acyl-CoA synthetase using a newly developed method for the isolation of peroxisomes from skin fibroblasts Journal of inherited metabolic disease 1988;11 (Suppl. 2):173-177 [PubMed]

1987

  • Alanine glyoxylate aminotransferase and the urinary excretion of oxalate and glycollate in hyperoxaluria type I and the Zellweger syndrome Clinica chimica acta; international journal of clinical chemistry 1987;165 (2-3):311-319 [PubMed]
  • Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders Journal of clinical investigation 1987;80 (6):1778-1783 [PubMed]
  • Peroxisomal very long-chain fatty acid beta-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders Clinica chimica acta; international journal of clinical chemistry 1987;166 (2-3):255-263 [PubMed]
  • Peroxisomes and peroxisomal functions in muscle. Studies with muscle cells from controls and a patient with the cerebro-hepato-renal (Zellweger) syndrome Experimental cell research 1987;170 (1):147-152 [PubMed]
  • Prenatal diagnosis of Zellweger syndrome by measurement of very long chain fatty acid (C26:0) beta-oxidation in cultured chorionic villous fibroblasts: implications for early diagnosis of other peroxisomal disorders Clinica chimica acta; international journal of clinical chemistry 1987;165 (2-3):303-310 [PubMed]
  • X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters Clinica chimica acta; international journal of clinical chemistry 1987;165 (2-3):321-329 [PubMed]

1986

  • Groen A. K., van Roermund C. W., Vervoorn R. C., Tager J. M. CONTROL OF GLUCONEOGENESIS IN RAT-LIVER CELLS - FLUX CONTROL COEFFICIENTS OF THE ENZYMES IN THE GLUCONEOGENIC PATHWAY IN THE ABSENCE AND PRESENCE OF GLUCAGON Biochemical journal 1986;237 (2):379-389 [PubMed]
  • Peroxisomal beta-oxidation of palmitoyl-CoA in human liver homogenates and its deficiency in the cerebro-hepato-renal (Zellweger) syndrome Clinica chimica acta; international journal of clinical chemistry 1986;159 (1):1-10 [PubMed]
  • Regulation of mitochondrial respiration in liver Advances in experimental medicine and biology 1986;194:241-254 [PubMed]
  • Regulation of squalene synthetase activity in rat liver: elevation by cholestyramine, but no diurnal variation Biochemical and biophysical research communications 1986;138 (1):335-341 [PubMed]

1985

  • APPLICATION OF CONTROL ANALYSIS IN STUDIES OF REGULATION OF INTERMEDIARY METABOLISM Biomedica biochimica acta 1985;44 (6):943-952 [PubMed]
  • MECHANISM OF THE STIMULATION OF RESPIRATION BY FATTY-ACIDS IN RAT-LIVER FEBS letters 1985;193 (2):243-246 [PubMed]

1984

  • Analysis of the control of citrulline synthesis in isolated rat-liver mitochondria European Journal of Biochemistry / FEBS 1984;142 (2):247-254 [PubMed]
  • Factors determining the relative contribution of the adenine-nucleotide translocator and the ADP-regenerating system to the control of oxidative phosphorylation in isolated rat-liver mitochondria European Journal of Biochemistry / FEBS 1984;142 (2):417-424 [PubMed]

1983

  • Fluorimetric determination of carbamoyl phosphate Analytical biochemistry 1983;129 (1):80-87 [PubMed]
  • Properties of carbamoyl-phosphate synthetase (ammonia) in rat-liver mitochondria made permeable with toluene European Journal of Biochemistry / FEBS 1983;135 (2):251-258 [PubMed]

Corona-maatregelen

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