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Transcriptome analysis suggests a compensatory role of the cofactors coenzyme A and NAD+ in medium-chain acyl-CoA dehydrogenase knockout mice.

Martines AMF, Gerding A, Stolle S, Vieira-Lara MA, Wolters JC, Jurdzinski A, Bongiovanni L, de Bruin A, van der Vlies P, van der Vries G, Bloks VW, Derks TGJ, Reijngoud DJ, Bakker BM.

Sci Rep. 2019 Oct 10;9(1):14539. doi: 10.1038/s41598-019-50758-0.


Running wheel access fails to resolve impaired sustainable health in mice feeding a high fat sucrose diet.

Reijne AC, Talarovicova A, Ciapaite J, Bruggink JE, Bleeker A, Groen AK, Reijngoud DJ, Bakker BM, van Dijk G.

Aging (Albany NY). 2019 Mar 11;11(5):1564-1579. doi: 10.18632/aging.101857.


A large pooled analysis refines gene expression-based molecular subclasses in cutaneous melanoma.

Wind TT, Jalving M, de Haan JJ, de Vries EGE, van Vugt MATM, Reijngoud DJ, van Rijn RS, Haanen JBAG, Blank CU, Hospers GAP, Fehrmann RSN.

Oncoimmunology. 2019 Jan 3;8(3):1558664. doi: 10.1080/2162402X.2018.1558664. eCollection 2019.


One- vs two-phase extraction: re-evaluation of sample preparation procedures for untargeted lipidomics in plasma samples.

Gil A, Zhang W, Wolters JC, Permentier H, Boer T, Horvatovich P, Heiner-Fokkema MR, Reijngoud DJ, Bischoff R.

Anal Bioanal Chem. 2018 Sep;410(23):5859-5870. doi: 10.1007/s00216-018-1200-x. Epub 2018 Jul 2.


Flux analysis of inborn errors of metabolism.

Reijngoud DJ.

J Inherit Metab Dis. 2018 May;41(3):309-328. doi: 10.1007/s10545-017-0124-5. Epub 2018 Jan 9. Review.


Running-wheel activity delays mitochondrial respiratory flux decline in aging mouse muscle via a post-transcriptional mechanism.

Stolle S, Ciapaite J, Reijne AC, Talarovicova A, Wolters JC, Aguirre-Gamboa R, van der Vlies P, de Lange K, Neerincx PB, van der Vries G, Deelen P, Swertz MA, Li Y, Bischoff R, Permentier HP, Horvatovitch PL, Groen AK, van Dijk G, Reijngoud DJ, Bakker BM.

Aging Cell. 2018 Feb;17(1). doi: 10.1111/acel.12700. Epub 2017 Nov 9.


The art of quantifying glucose metabolism.

van Dijk TH, Reijngoud D, Kuipers F.

Am J Physiol Endocrinol Metab. 2017 Aug 1;313(2):E257-E258. doi: 10.1152/ajpendo.00066.2017. No abstract available.


Hepatocytes contribute to residual glucose production in a mouse model for glycogen storage disease type Ia.

Hijmans BS, Boss A, van Dijk TH, Soty M, Wolters H, Mutel E, Groen AK, Derks TGJ, Mithieux G, Heerschap A, Reijngoud DJ, Rajas F, Oosterveer MH.

Hepatology. 2017 Dec;66(6):2042-2054. doi: 10.1002/hep.29389. Epub 2017 Oct 30.


The promiscuous enzyme medium-chain 3-keto-acyl-CoA thiolase triggers a vicious cycle in fatty-acid beta-oxidation.

Martines AMF, van Eunen K, Reijngoud DJ, Bakker BM.

PLoS Comput Biol. 2017 Apr 3;13(4):e1005461. doi: 10.1371/journal.pcbi.1005461. eCollection 2017 Apr.


The degradation of nucleotide triphosphates extracted under boiling ethanol conditions is prevented by the yeast cellular matrix.

Gil A, Siegel D, Bonsing-Vedelaar S, Permentier H, Reijngoud DJ, Dekker F, Bischoff R.

Metabolomics. 2017;13(1):1. Epub 2016 Nov 28.


Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders.

van Eunen K, Volker-Touw CM, Gerding A, Bleeker A, Wolters JC, van Rijt WJ, Martines AM, Niezen-Koning KE, Heiner RM, Permentier H, Groen AK, Reijngoud DJ, Derks TG, Bakker BM.

BMC Biol. 2016 Dec 7;14(1):107.


Malnutrition-associated liver steatosis and ATP depletion is caused by peroxisomal and mitochondrial dysfunction.

van Zutphen T, Ciapaite J, Bloks VW, Ackereley C, Gerding A, Jurdzinski A, de Moraes RA, Zhang L, Wolters JC, Bischoff R, Wanders RJ, Houten SM, Bronte-Tinkew D, Shatseva T, Lewis GF, Groen AK, Reijngoud DJ, Bakker BM, Jonker JW, Kim PK, Bandsma RH.

J Hepatol. 2016 Dec;65(6):1198-1208. doi: 10.1016/j.jhep.2016.05.046. Epub 2016 Jun 14.


Reliable Diagnosis of Carnitine Palmitoyltransferase Type IA Deficiency by Analysis of Plasma Acylcarnitine Profiles.

Heiner-Fokkema MR, Vaz FM, Maatman R, Kluijtmans LA, van Spronsen FJ, Reijngoud DJ.

JIMD Rep. 2017;32:33-39. doi: 10.1007/8904_2016_564. Epub 2016 Jun 14.


Whole-Body Vibration Partially Reverses Aging-Induced Increases in Visceral Adiposity and Hepatic Lipid Storage in Mice.

Reijne AC, Ciapaite J, van Dijk TH, Havinga R, van der Zee EA, Groen AK, Reijngoud DJ, Bakker BM, van Dijk G.

PLoS One. 2016 Feb 17;11(2):e0149419. doi: 10.1371/journal.pone.0149419. eCollection 2016.


Extracellular 4'-phosphopantetheine is a source for intracellular coenzyme A synthesis.

Srinivasan B, Baratashvili M, van der Zwaag M, Kanon B, Colombelli C, Lambrechts RA, Schaap O, Nollen EA, Podgoršek A, Kosec G, Petković H, Hayflick S, Tiranti V, Reijngoud DJ, Grzeschik NA, Sibon OC.

Nat Chem Biol. 2015 Oct;11(10):784-92. doi: 10.1038/nchembio.1906. Epub 2015 Aug 31.


Protection against the Metabolic Syndrome by Guar Gum-Derived Short-Chain Fatty Acids Depends on Peroxisome Proliferator-Activated Receptor γ and Glucagon-Like Peptide-1.

den Besten G, Gerding A, van Dijk TH, Ciapaite J, Bleeker A, van Eunen K, Havinga R, Groen AK, Reijngoud DJ, Bakker BM.

PLoS One. 2015 Aug 20;10(8):e0136364. doi: 10.1371/journal.pone.0136364. eCollection 2015.


Stability of energy metabolites-An often overlooked issue in metabolomics studies: A review.

Gil A, Siegel D, Permentier H, Reijngoud DJ, Dekker F, Bischoff R.

Electrophoresis. 2015 Sep;36(18):2156-2169. doi: 10.1002/elps.201500031. Epub 2015 Jul 6. Review.


Short-Chain Fatty Acids Protect Against High-Fat Diet-Induced Obesity via a PPARγ-Dependent Switch From Lipogenesis to Fat Oxidation.

den Besten G, Bleeker A, Gerding A, van Eunen K, Havinga R, van Dijk TH, Oosterveer MH, Jonker JW, Groen AK, Reijngoud DJ, Bakker BM.

Diabetes. 2015 Jul;64(7):2398-408. doi: 10.2337/db14-1213. Epub 2015 Feb 18.


Phenylketonuria: brain phenylalanine concentrations relate inversely to cerebral protein synthesis.

de Groot MJ, Sijens PE, Reijngoud DJ, Paans AM, van Spronsen FJ.

J Cereb Blood Flow Metab. 2015 Feb;35(2):200-5. doi: 10.1038/jcbfm.2014.183. Epub 2014 Oct 29.


The short-chain fatty acid uptake fluxes by mice on a guar gum supplemented diet associate with amelioration of major biomarkers of the metabolic syndrome.

den Besten G, Havinga R, Bleeker A, Rao S, Gerding A, van Eunen K, Groen AK, Reijngoud DJ, Bakker BM.

PLoS One. 2014 Sep 9;9(9):e107392. doi: 10.1371/journal.pone.0107392. eCollection 2014.


A patient with acute liver failure and extreme hypoglycaemia with lactic acidosis who was not in a coma: causes and consequences of lactate-protected hypoglycaemia.

Oldenbeuving G, McDonald JR, Goodwin ML, Sayilir R, Reijngoud DJ, Gladden LB, Nijsten MW.

Anaesth Intensive Care. 2014 Jul;42(4):507-11.


From genome to phenome-Simple inborn errors of metabolism as complex traits.

Touw CM, Derks TG, Bakker BM, Groen AK, Smit GP, Reijngoud DJ.

Biochim Biophys Acta. 2014 Oct;1842(10):2021-2029. doi: 10.1016/j.bbadis.2014.05.032. Epub 2014 Jun 3. Review.


Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency.

Derks TG, Touw CM, Ribas GS, Biancini GB, Vanzin CS, Negretto G, Mescka CP, Reijngoud DJ, Smit GP, Wajner M, Vargas CR.

J Inherit Metab Dis. 2014 Sep;37(5):783-9. doi: 10.1007/s10545-014-9700-0. Epub 2014 Mar 13.


Chemical and technical challenges in the analysis of central carbon metabolites by liquid-chromatography mass spectrometry.

Siegel D, Permentier H, Reijngoud DJ, Bischoff R.

J Chromatogr B Analyt Technol Biomed Life Sci. 2014 Sep 1;966:21-33. doi: 10.1016/j.jchromb.2013.11.022. Epub 2013 Nov 26. Review.


Gut-derived short-chain fatty acids are vividly assimilated into host carbohydrates and lipids.

den Besten G, Lange K, Havinga R, van Dijk TH, Gerding A, van Eunen K, Müller M, Groen AK, Hooiveld GJ, Bakker BM, Reijngoud DJ.

Am J Physiol Gastrointest Liver Physiol. 2013 Dec;305(12):G900-10. doi: 10.1152/ajpgi.00265.2013. Epub 2013 Oct 17.


Phenylketonuria: reduced tyrosine brain influx relates to reduced cerebral protein synthesis.

de Groot MJ, Hoeksma M, Reijngoud DJ, de Valk HW, Paans AM, Sauer PJ, van Spronsen FJ.

Orphanet J Rare Dis. 2013 Sep 4;8:133. doi: 10.1186/1750-1172-8-133.


Biochemical competition makes fatty-acid β-oxidation vulnerable to substrate overload.

van Eunen K, Simons SM, Gerding A, Bleeker A, den Besten G, Touw CM, Houten SM, Groen BK, Krab K, Reijngoud DJ, Bakker BM.

PLoS Comput Biol. 2013;9(8):e1003186. doi: 10.1371/journal.pcbi.1003186. Epub 2013 Aug 15.


Impaired amino acid metabolism contributes to fasting-induced hypoglycemia in fatty acid oxidation defects.

Houten SM, Herrema H, Te Brinke H, Denis S, Ruiter JP, van Dijk TH, Argmann CA, Ottenhoff R, Müller M, Groen AK, Kuipers F, Reijngoud DJ, Wanders RJ.

Hum Mol Genet. 2013 Dec 20;22(25):5249-61. doi: 10.1093/hmg/ddt382. Epub 2013 Aug 9.


The role of short-chain fatty acids in the interplay between diet, gut microbiota, and host energy metabolism.

den Besten G, van Eunen K, Groen AK, Venema K, Reijngoud DJ, Bakker BM.

J Lipid Res. 2013 Sep;54(9):2325-40. doi: 10.1194/jlr.R036012. Epub 2013 Jul 2. Review.


In vitro and in vivo consequences of variant medium-chain acyl-CoA dehydrogenase genotypes.

Touw CM, Smit GP, Niezen-Koning KE, Bosgraaf-de Boer C, Gerding A, Reijngoud DJ, Derks TG.

Orphanet J Rare Dis. 2013 Mar 20;8:43. doi: 10.1186/1750-1172-8-43.


A novel approach to monitor glucose metabolism using stable isotopically labelled glucose in longitudinal studies in mice.

van Dijk TH, Laskewitz AJ, Grefhorst A, Boer TS, Bloks VW, Kuipers F, Groen AK, Reijngoud DJ.

Lab Anim. 2013 Apr;47(2):79-88. doi: 10.1177/0023677212473714. Epub 2013 Mar 14.


The gut microbiota elicits a profound metabolic reorientation in the mouse jejunal mucosa during conventionalisation.

El Aidy S, Merrifield CA, Derrien M, van Baarlen P, Hooiveld G, Levenez F, Doré J, Dekker J, Holmes E, Claus SP, Reijngoud DJ, Kleerebezem M.

Gut. 2013 Sep;62(9):1306-14. doi: 10.1136/gutjnl-2011-301955. Epub 2012 Jun 21.


Chronic prednisolone treatment aggravates hyperglycemia in mice fed a high-fat diet but does not worsen dietary fat-induced insulin resistance.

Laskewitz AJ, van Dijk TH, Grefhorst A, van Lierop MJ, Schreurs M, Bloks VW, Reijngoud DJ, Dokter WH, Kuipers F, Groen AK.

Endocrinology. 2012 Aug;153(8):3713-23. doi: 10.1210/en.2011-1891. Epub 2012 May 31.


Risk stratification by residual enzyme activity after newborn screening for medium-chain acyl-CoA dehyrogenase deficiency: data from a cohort study.

Touw CM, Smit GP, de Vries M, de Klerk JB, Bosch AM, Visser G, Mulder MF, Rubio-Gozalbo ME, Elvers B, Niezen-Koning KE, Wanders RJ, Waterham HR, Reijngoud DJ, Derks TG.

Orphanet J Rare Dis. 2012 May 25;7:30. doi: 10.1186/1750-1172-7-30.


Kwashiorkor and marasmus are both associated with impaired glucose clearance related to pancreatic β-cell dysfunction.

Spoelstra MN, Mari A, Mendel M, Senga E, van Rheenen P, van Dijk TH, Reijngoud DJ, Zegers RG, Heikens GT, Bandsma RH.

Metabolism. 2012 Sep;61(9):1224-30. doi: 10.1016/j.metabol.2012.01.019. Epub 2012 Mar 3.


Bile acid sequestration reduces plasma glucose levels in db/db mice by increasing its metabolic clearance rate.

Meissner M, Herrema H, van Dijk TH, Gerding A, Havinga R, Boer T, Müller M, Reijngoud DJ, Groen AK, Kuipers F.

PLoS One. 2011;6(11):e24564. doi: 10.1371/journal.pone.0024564. Epub 2011 Nov 7.


Diurnal variations in blood phenylalanine of PKU infants under different feeding regimes.

van Rijn M, Hoeksma M, Sauer PJ, Modderman P, Reijngoud DJ, van Spronsen FJ.

Mol Genet Metab. 2011;104 Suppl:S68-72. doi: 10.1016/j.ymgme.2011.08.010. Epub 2011 Aug 16.


De novo lipogenesis in the differentiating human adipocyte can provide all fatty acids necessary for maturation.

Collins JM, Neville MJ, Pinnick KE, Hodson L, Ruyter B, van Dijk TH, Reijngoud DJ, Fielding MD, Frayn KN.

J Lipid Res. 2011 Sep;52(9):1683-92. doi: 10.1194/jlr.M012195. Epub 2011 Jun 15.


Adult patients with well-controlled phenylketonuria tolerate incidental additional intake of phenylalanine.

van Rijn M, Hoeksma M, Sauer PJ, Modderman P, Reijngoud DJ, van Spronsen FJ.

Ann Nutr Metab. 2011;58(2):94-100. doi: 10.1159/000324924. Epub 2011 Apr 8.


Serum vitamin B12 concentrations within reference values do not exclude functional vitamin B12 deficiency in PKU patients of various ages.

Vugteveen I, Hoeksma M, Monsen AL, Fokkema MR, Reijngoud DJ, van Rijn M, van Spronsen FJ.

Mol Genet Metab. 2011 Jan;102(1):13-7. doi: 10.1016/j.ymgme.2010.07.004. Epub 2010 Oct 27.


Large neutral amino acids in the treatment of PKU: from theory to practice.

van Spronsen FJ, de Groot MJ, Hoeksma M, Reijngoud DJ, van Rijn M.

J Inherit Metab Dis. 2010 Dec;33(6):671-6. doi: 10.1007/s10545-010-9216-1. Epub 2010 Oct 26. Review.


Carbohydrate-response-element-binding protein (ChREBP) and not the liver X receptor α (LXRα) mediates elevated hepatic lipogenic gene expression in a mouse model of glycogen storage disease type 1.

Grefhorst A, Schreurs M, Oosterveer MH, Cortés VA, Havinga R, Herling AW, Reijngoud DJ, Groen AK, Kuipers F.

Biochem J. 2010 Dec 1;432(2):249-54. doi: 10.1042/BJ20101225.


Metabolic investigations prevent liver transplantation in two young children with citrullinemia type I.

de Groot MJ, Cuppen M, Eling M, Verheijen FW, Rings EH, Reijngoud DJ, de Vries MM, van Spronsen FJ.

J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S413-6. doi: 10.1007/s10545-010-9207-2. Epub 2010 Sep 18.


Mechanisms behind decreased endogenous glucose production in malnourished children.

Bandsma RH, Mendel M, Spoelstra MN, Reijngoud DJ, Boer T, Stellaard F, Brabin B, Schellekens R, Senga E, Heikens GT.

Pediatr Res. 2010 Nov;68(5):423-8. doi: 10.1203/PDR.0b013e3181f2b959.


Pantethine rescues a Drosophila model for pantothenate kinase-associated neurodegeneration.

Rana A, Seinen E, Siudeja K, Muntendam R, Srinivasan B, van der Want JJ, Hayflick S, Reijngoud DJ, Kayser O, Sibon OC.

Proc Natl Acad Sci U S A. 2010 Apr 13;107(15):6988-93. doi: 10.1073/pnas.0912105107. Epub 2010 Mar 29.


Pathogenesis of cognitive dysfunction in phenylketonuria: review of hypotheses.

de Groot MJ, Hoeksma M, Blau N, Reijngoud DJ, van Spronsen FJ.

Mol Genet Metab. 2010;99 Suppl 1:S86-9. doi: 10.1016/j.ymgme.2009.10.016. Review.


Chronic prednisolone treatment reduces hepatic insulin sensitivity while perturbing the fed-to-fasting transition in mice.

Laskewitz AJ, van Dijk TH, Bloks VW, Reijngoud DJ, van Lierop MJ, Dokter WH, Kuipers F, Groen AK, Grefhorst A.

Endocrinology. 2010 May;151(5):2171-8. doi: 10.1210/en.2009-1374. Epub 2010 Feb 25.


Bile salt sequestration induces hepatic de novo lipogenesis through farnesoid X receptor- and liver X receptor alpha-controlled metabolic pathways in mice.

Herrema H, Meissner M, van Dijk TH, Brufau G, Boverhof R, Oosterveer MH, Reijngoud DJ, Müller M, Stellaard F, Groen AK, Kuipers F.

Hepatology. 2010 Mar;51(3):806-16. doi: 10.1002/hep.23408.


Fenofibrate simultaneously induces hepatic fatty acid oxidation, synthesis, and elongation in mice.

Oosterveer MH, Grefhorst A, van Dijk TH, Havinga R, Staels B, Kuipers F, Groen AK, Reijngoud DJ.

J Biol Chem. 2009 Dec 4;284(49):34036-44. doi: 10.1074/jbc.M109.051052. Epub 2009 Oct 2.


High fat feeding induces hepatic fatty acid elongation in mice.

Oosterveer MH, van Dijk TH, Tietge UJ, Boer T, Havinga R, Stellaard F, Groen AK, Kuipers F, Reijngoud DJ.

PLoS One. 2009 Jun 26;4(6):e6066. doi: 10.1371/journal.pone.0006066.

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