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Items: 1 to 50 of 130

1.

Liver disease predominates in a mouse model for mild human Zellweger spectrum disorder.

Berendse K, Boek M, Gijbels M, Van der Wel NN, Klouwer FC, van den Bergh-Weerman MA, Shinde AB, Ofman R, Poll-The BT, Houten SM, Baes M, Wanders RJA, Waterham HR.

Biochim Biophys Acta Mol Basis Dis. 2019 Oct 1;1865(10):2774-2787. doi: 10.1016/j.bbadis.2019.06.013. Epub 2019 Jun 15.

2.

Mild inborn errors of metabolism in commonly used inbred mouse strains.

Leandro J, Violante S, Argmann CA, Hagen J, Dodatko T, Bender A, Zhang W, Williams EG, Bachmann AM, Auwerx J, Yu C, Houten SM.

Mol Genet Metab. 2019 Apr;126(4):388-396. doi: 10.1016/j.ymgme.2019.01.021. Epub 2019 Jan 24.

PMID:
30709776
3.

PLPHP deficiency: clinical, genetic, biochemical, and mechanistic insights.

Johnstone DL, Al-Shekaili HH, Tarailo-Graovac M, Wolf NI, Ivy AS, Demarest S, Roussel Y, Ciapaite J, van Roermund CWT, Kernohan KD, Kosuta C, Ban K, Ito Y, McBride S, Al-Thihli K, Abdelrahim RA, Koul R, Al Futaisi A, Haaxma CA, Olson H, Sigurdardottir LY, Arnold GL, Gerkes EH, Boon M, Heiner-Fokkema MR, Noble S, Bosma M, Jans J, Koolen DA, Kamsteeg EJ, Drögemöller B, Ross CJ, Majewski J, Cho MT, Begtrup A, Wasserman WW, Bui T, Brimble E, Violante S, Houten SM, Wevers RA, van Faassen M, Kema IP, Lepage N; Care4Rare Canada Consortium, Lines MA, Dyment DA, Wanders RJA, Verhoeven-Duif N, Ekker M, Boycott KM, Friedman JM, Pena IA, van Karnebeek CDM.

Brain. 2019 Mar 1;142(3):542-559. doi: 10.1093/brain/awy346.

PMID:
30668673
4.

Saccharopine, a lysine degradation intermediate, is a mitochondrial toxin.

Leandro J, Houten SM.

J Cell Biol. 2019 Feb 4;218(2):391-392. doi: 10.1083/jcb.201901033. Epub 2019 Jan 16.

5.

Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4.

Violante S, Achetib N, van Roermund CWT, Hagen J, Dodatko T, Vaz FM, Waterham HR, Chen H, Baes M, Yu C, Argmann CA, Houten SM.

FASEB J. 2019 Mar;33(3):4355-4364. doi: 10.1096/fj.201801498R. Epub 2018 Dec 12.

PMID:
30540494
6.

Increased cardiac fatty acid oxidation in a mouse model with decreased malonyl-CoA sensitivity of CPT1B.

van Weeghel M, Abdurrachim D, Nederlof R, Argmann CA, Houtkooper RH, Hagen J, Nabben M, Denis S, Ciapaite J, Kolwicz SC Jr, Lopaschuk GD, Auwerx J, Nicolay K, Des Rosiers C, Wanders RJ, Zuurbier CJ, Prompers JJ, Houten SM.

Cardiovasc Res. 2018 Aug 1;114(10):1324-1334. doi: 10.1093/cvr/cvy089.

PMID:
29635338
7.

Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome.

Lake NJ, Webb BD, Stroud DA, Richman TR, Ruzzenente B, Compton AG, Mountford HS, Pulman J, Zangarelli C, Rio M, Boddaert N, Assouline Z, Sherpa MD, Schadt EE, Houten SM, Byrnes J, McCormick EM, Zolkipli-Cunningham Z, Haude K, Zhang Z, Retterer K, Bai R, Calvo SE, Mootha VK, Christodoulou J, Rötig A, Filipovska A, Cristian I, Falk MJ, Metodiev MD, Thorburn DR.

Am J Hum Genet. 2018 Apr 5;102(4):713. doi: 10.1016/j.ajhg.2018.03.015. No abstract available.

8.

Protein moonlighting in inborn errors of metabolism: the case of the mitochondrial acylglycerol kinase.

Houten SM.

J Inherit Metab Dis. 2017 Nov;40(6):755-756. doi: 10.1007/s10545-017-0090-y. Epub 2017 Sep 21. No abstract available.

9.

Germline deletion of Krüppel-like factor 14 does not increase risk of diet induced metabolic syndrome in male C57BL/6 mice.

Argmann CA, Violante S, Dodatko T, Amaro MP, Hagen J, Gillespie VL, Buettner C, Schadt EE, Houten SM.

Biochim Biophys Acta Mol Basis Dis. 2017 Dec;1863(12):3277-3285. doi: 10.1016/j.bbadis.2017.09.021. Epub 2017 Sep 28.

10.

Deficiency of the Mitochondrial NAD Kinase Causes Stress-Induced Hepatic Steatosis in Mice.

Zhang K, Kim H, Fu Z, Qiu Y, Yang Z, Wang J, Zhang D, Tong X, Yin L, Li J, Wu J, Qi NR, Houten SM, Zhang R.

Gastroenterology. 2018 Jan;154(1):224-237. doi: 10.1053/j.gastro.2017.09.010. Epub 2017 Sep 18.

11.

Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome.

Lake NJ, Webb BD, Stroud DA, Richman TR, Ruzzenente B, Compton AG, Mountford HS, Pulman J, Zangarelli C, Rio M, Boddaert N, Assouline Z, Sherpa MD, Schadt EE, Houten SM, Byrnes J, McCormick EM, Zolkipli-Cunningham Z, Haude K, Zhang Z, Retterer K, Bai R, Calvo SE, Mootha VK, Christodoulou J, Rötig A, Filipovska A, Cristian I, Falk MJ, Metodiev MD, Thorburn DR.

Am J Hum Genet. 2017 Aug 3;101(2):239-254. doi: 10.1016/j.ajhg.2017.07.005. Erratum in: Am J Hum Genet. 2018 Apr 5;102(4):713.

12.

Acute detachment of hexokinase II from mitochondria modestly increases oxygen consumption of the intact mouse heart.

Nederlof R, Denis S, Lauzier B, Rosiers CD, Laakso M, Hagen J, Argmann C, Wanders R, Houtkooper RH, Hollmann MW, Houten SM, Zuurbier CJ.

Metabolism. 2017 Jul;72:66-74. doi: 10.1016/j.metabol.2017.04.008. Epub 2017 Apr 21.

13.

Heterozygous Pathogenic Variant in DACT1 Causes an Autosomal-Dominant Syndrome with Features Overlapping Townes-Brocks Syndrome.

Webb BD, Metikala S, Wheeler PG, Sherpa MD, Houten SM, Horb ME, Schadt EE.

Hum Mutat. 2017 Apr;38(4):373-377. doi: 10.1002/humu.23171. Epub 2017 Feb 2.

14.

Changes in the Metabolome in Response to Low-Dose Exposure to Environmental Chemicals Used in Personal Care Products during Different Windows of Susceptibility.

Houten SM, Chen J, Belpoggi F, Manservisi F, Sánchez-Guijo A, Wudy SA, Teitelbaum SL.

PLoS One. 2016 Jul 28;11(7):e0159919. doi: 10.1371/journal.pone.0159919. eCollection 2016.

15.

Assessment of plasma acylcarnitines before and after weight loss in obese subjects.

Schooneman MG, Napolitano A, Houten SM, Ambler GK, Murgatroyd PR, Miller SR, Hollak CE, Tan CY, Virtue S, Vidal-Puig A, Nunez DJ, Soeters MR.

Arch Biochem Biophys. 2016 Sep 15;606:73-80. doi: 10.1016/j.abb.2016.07.013. Epub 2016 Jul 19.

PMID:
27444119
16.

A PPARγ-Bnip3 Axis Couples Adipose Mitochondrial Fusion-Fission Balance to Systemic Insulin Sensitivity.

Tol MJ, Ottenhoff R, van Eijk M, Zelcer N, Aten J, Houten SM, Geerts D, van Roomen C, Bierlaagh MC, Scheij S, Hoeksema MA, Aerts JM, Bogan JS, Dorn GW 2nd, Argmann CA, Verhoeven AJ.

Diabetes. 2016 Sep;65(9):2591-605. doi: 10.2337/db16-0243. Epub 2016 Jun 20.

17.

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.

18.

Systems proteomics of liver mitochondria function.

Williams EG, Wu Y, Jha P, Dubuis S, Blattmann P, Argmann CA, Houten SM, Amariuta T, Wolski W, Zamboni N, Aebersold R, Auwerx J.

Science. 2016 Jun 10;352(6291):aad0189. doi: 10.1126/science.aad0189.

19.

The impact of altered carnitine availability on acylcarnitine metabolism, energy expenditure and glucose tolerance in diet-induced obese mice.

Schooneman MG, Houtkooper RH, Hollak CE, Wanders RJ, Vaz FM, Soeters MR, Houten SM.

Biochim Biophys Acta. 2016 Aug;1862(8):1375-82. doi: 10.1016/j.bbadis.2016.04.012. Epub 2016 Apr 22.

20.

Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.

Diekman EF, Visser G, Schmitz JP, Nievelstein RA, de Sain-van der Velden M, Wardrop M, Van der Pol WL, Houten SM, van Riel NA, Takken T, Jeneson JA.

PLoS One. 2016 Feb 16;11(2):e0147818. doi: 10.1371/journal.pone.0147818. eCollection 2016.

21.

Inter-tissue coexpression network analysis reveals DPP4 as an important gene in heart to blood communication.

Long Q, Argmann C, Houten SM, Huang T, Peng S, Zhao Y, Tu Z; GTEx Consortium, Zhu J.

Genome Med. 2016 Feb 9;8(1):15. doi: 10.1186/s13073-016-0268-1.

22.

A Next Generation Multiscale View of Inborn Errors of Metabolism.

Argmann CA, Houten SM, Zhu J, Schadt EE.

Cell Metab. 2016 Jan 12;23(1):13-26. doi: 10.1016/j.cmet.2015.11.012. Epub 2015 Dec 17. Review.

23.

The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders.

Houten SM, Violante S, Ventura FV, Wanders RJ.

Annu Rev Physiol. 2016;78:23-44. doi: 10.1146/annurev-physiol-021115-105045. Epub 2015 Oct 14. Review.

PMID:
26474213
24.

Carnitine supplementation in high-fat diet-fed rats does not ameliorate lipid-induced skeletal muscle mitochondrial dysfunction in vivo.

Wessels B, van den Broek NM, Ciapaite J, Houten SM, Wanders RJ, Nicolay K, Prompers JJ.

Am J Physiol Endocrinol Metab. 2015 Oct 1;309(7):E670-8. doi: 10.1152/ajpendo.00144.2015. Epub 2015 Aug 18.

25.

In vivo mouse myocardial (31)P MRS using three-dimensional image-selected in vivo spectroscopy (3D ISIS): technical considerations and biochemical validations.

Bakermans AJ, Abdurrachim D, van Nierop BJ, Koeman A, van der Kroon I, Baartscheer A, Schumacher CA, Strijkers GJ, Houten SM, Zuurbier CJ, Nicolay K, Prompers JJ.

NMR Biomed. 2015 Oct;28(10):1218-27. doi: 10.1002/nbm.3371. Epub 2015 Aug 13.

26.

Transorgan fluxes in a porcine model reveal a central role for liver in acylcarnitine metabolism.

Schooneman MG, Ten Have GA, van Vlies N, Houten SM, Deutz NE, Soeters MR.

Am J Physiol Endocrinol Metab. 2015 Aug 1;309(3):E256-64. doi: 10.1152/ajpendo.00503.2014. Epub 2015 Jun 2.

27.

Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.

Hagen J, te Brinke H, Wanders RJ, Knegt AC, Oussoren E, Hoogeboom AJ, Ruijter GJ, Becker D, Schwab KO, Franke I, Duran M, Waterham HR, Sass JO, Houten SM.

J Inherit Metab Dis. 2015 Sep;38(5):873-9. doi: 10.1007/s10545-015-9841-9. Epub 2015 Apr 10.

PMID:
25860818
28.

Fatty acid oxidation flux predicts the clinical severity of VLCAD deficiency.

Diekman EF, Ferdinandusse S, van der Pol L, Waterham HR, Ruiter JP, Ijlst L, Wanders RJ, Houten SM, Wijburg FA, Blank AC, Asselbergs FW, Houtkooper RH, Visser G.

Genet Med. 2015 Dec;17(12):989-94. doi: 10.1038/gim.2015.22. Epub 2015 Apr 2.

PMID:
25834949
29.

Cholesterol-induced hepatic inflammation does not underlie the predisposition to insulin resistance in dyslipidemic female LDL receptor knockout mice.

Gruben N, Funke A, Kloosterhuis NJ, Schreurs M, Sheedfar F, Havinga R, Houten SM, Shiri-Sverdlov R, van de Sluis B, Kuivenhoven JA, Koonen DP, Hofker MH.

J Diabetes Res. 2015;2015:956854. doi: 10.1155/2015/956854. Epub 2015 Feb 28.

30.

Novel, compound heterozygous, single-nucleotide variants in MARS2 associated with developmental delay, poor growth, and sensorineural hearing loss.

Webb BD, Wheeler PG, Hagen JJ, Cohen N, Linderman MD, Diaz GA, Naidich TP, Rodenburg RJ, Houten SM, Schadt EE.

Hum Mutat. 2015 Jun;36(6):587-92. doi: 10.1002/humu.22781. Epub 2015 Apr 8.

31.

Fiber-type-specific sensitivities and phenotypic adaptations to dietary fat overload differentially impact fast- versus slow-twitch muscle contractile function in C57BL/6J mice.

Ciapaite J, van den Berg SA, Houten SM, Nicolay K, van Dijk KW, Jeneson JA.

J Nutr Biochem. 2015 Feb;26(2):155-64. doi: 10.1016/j.jnutbio.2014.09.014. Epub 2014 Oct 25.

PMID:
25516489
32.

High-protein diets prevent steatosis and induce hepatic accumulation of monomethyl branched-chain fatty acids.

Garcia Caraballo SC, Comhair TM, Houten SM, Dejong CH, Lamers WH, Koehler SE.

J Nutr Biochem. 2014 Dec;25(12):1263-74. doi: 10.1016/j.jnutbio.2014.07.005. Epub 2014 Sep 16.

PMID:
25287814
33.

Multilayered genetic and omics dissection of mitochondrial activity in a mouse reference population.

Wu Y, Williams EG, Dubuis S, Mottis A, Jovaisaite V, Houten SM, Argmann CA, Faridi P, Wolski W, Kutalik Z, Zamboni N, Auwerx J, Aebersold R.

Cell. 2014 Sep 11;158(6):1415-1430. doi: 10.1016/j.cell.2014.07.039.

34.

Pioglitazone treatment restores in vivo muscle oxidative capacity in a rat model of diabetes.

Wessels B, Ciapaite J, van den Broek NM, Houten SM, Nicolay K, Prompers JJ.

Diabetes Obes Metab. 2015 Jan;17(1):52-60. doi: 10.1111/dom.12388. Epub 2014 Oct 6.

PMID:
25200673
35.

SUMOylation-dependent LRH-1/PROX1 interaction promotes atherosclerosis by decreasing hepatic reverse cholesterol transport.

Stein S, Oosterveer MH, Mataki C, Xu P, Lemos V, Havinga R, Dittner C, Ryu D, Menzies KJ, Wang X, Perino A, Houten SM, Melchior F, Schoonjans K.

Cell Metab. 2014 Oct 7;20(4):603-13. doi: 10.1016/j.cmet.2014.07.023. Epub 2014 Aug 28.

36.

A mitochondrial expatriate: nuclear pyruvate dehydrogenase.

de Boer VC, Houten SM.

Cell. 2014 Jul 3;158(1):9-10. doi: 10.1016/j.cell.2014.06.018.

37.

In vivo proton T1 relaxation times of mouse myocardial metabolites at 9.4 T.

Bakermans AJ, Abdurrachim D, Geraedts TR, Houten SM, Nicolay K, Prompers JJ.

Magn Reson Med. 2015 Jun;73(6):2069-74. doi: 10.1002/mrm.25340. Epub 2014 Jun 24.

PMID:
24962369
38.

Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia.

Houten SM, Denis S, Te Brinke H, Jongejan A, van Kampen AH, Bradley EJ, Baas F, Hennekam RC, Millington DS, Young SP, Frazier DM, Gucsavas-Calikoglu M, Wanders RJ.

Hum Mol Genet. 2014 Sep 15;23(18):5009-16. doi: 10.1093/hmg/ddu218. Epub 2014 May 8.

PMID:
24847004
39.

Bile acids alter male fertility through G-protein-coupled bile acid receptor 1 signaling pathways in mice.

Baptissart M, Vega A, Martinot E, Pommier AJ, Houten SM, Marceau G, de Haze A, Baron S, Schoonjans K, Lobaccaro JM, Volle DH.

Hepatology. 2014 Sep;60(3):1054-65. doi: 10.1002/hep.27204.

PMID:
24798773
40.

Plasma acylcarnitines inadequately reflect tissue acylcarnitine metabolism.

Schooneman MG, Achterkamp N, Argmann CA, Soeters MR, Houten SM.

Biochim Biophys Acta. 2014 Jul;1841(7):987-94. doi: 10.1016/j.bbalip.2014.04.001. Epub 2014 Apr 18.

PMID:
24747043
41.
42.

Food withdrawal lowers energy expenditure and induces inactivity in long-chain fatty acid oxidation-deficient mouse models.

Diekman EF, van Weeghel M, Wanders RJ, Visser G, Houten SM.

FASEB J. 2014 Jul;28(7):2891-900. doi: 10.1096/fj.14-250241. Epub 2014 Mar 19.

PMID:
24648546
43.

Aberrant protein acylation is a common observation in inborn errors of acyl-CoA metabolism.

Pougovkina O, Te Brinke H, Wanders RJ, Houten SM, de Boer VC.

J Inherit Metab Dis. 2014 Sep;37(5):709-14. doi: 10.1007/s10545-014-9684-9. Epub 2014 Feb 15.

PMID:
24531926
44.

Mitochondrial protein acetylation is driven by acetyl-CoA from fatty acid oxidation.

Pougovkina O, te Brinke H, Ofman R, van Cruchten AG, Kulik W, Wanders RJ, Houten SM, de Boer VC.

Hum Mol Genet. 2014 Jul 1;23(13):3513-22. doi: 10.1093/hmg/ddu059. Epub 2014 Feb 10.

PMID:
24516071
45.

Fasting serum taurine-conjugated bile acids are elevated in type 2 diabetes and do not change with intensification of insulin.

Wewalka M, Patti ME, Barbato C, Houten SM, Goldfine AB.

J Clin Endocrinol Metab. 2014 Apr;99(4):1442-51. doi: 10.1210/jc.2013-3367. Epub 2014 Jan 16.

46.

Identification and characterization of Eci3, a murine kidney-specific Δ3,Δ2-enoyl-CoA isomerase.

van Weeghel M, Ofman R, Argmann CA, Ruiter JP, Claessen N, Oussoren SV, Wanders RJ, Aten J, Houten SM.

FASEB J. 2014 Mar;28(3):1365-74. doi: 10.1096/fj.13-240416. Epub 2013 Dec 16.

PMID:
24344334
47.

Muscle MRI in patients with long-chain fatty acid oxidation disorders.

Diekman EF, van der Pol WL, Nievelstein RA, Houten SM, Wijburg FA, Visser G.

J Inherit Metab Dis. 2014 May;37(3):405-13. doi: 10.1007/s10545-013-9666-3. Epub 2013 Dec 5.

PMID:
24305961
48.

ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies.

Nouws J, Te Brinke H, Nijtmans LG, Houten SM.

Hum Mol Genet. 2014 Mar 1;23(5):1311-9. doi: 10.1093/hmg/ddt521. Epub 2013 Oct 24.

PMID:
24158852
49.

Myocardial energy shortage and unmet anaplerotic needs in the fasted long-chain acyl-CoA dehydrogenase knockout mouse.

Bakermans AJ, Dodd MS, Nicolay K, Prompers JJ, Tyler DJ, Houten SM.

Cardiovasc Res. 2013 Dec 1;100(3):441-9. doi: 10.1093/cvr/cvt212. Epub 2013 Sep 16.

PMID:
24042017
50.

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.

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