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Items: 1 to 50 of 188

1.

Mitochondrial acyl carrier protein (ACP) at the interface of metabolic state sensing and mitochondrial function.

Masud AJ, Kastaniotis AJ, Rahman MT, Autio KJ, Hiltunen JK.

Biochim Biophys Acta Mol Cell Res. 2019 Dec;1866(12):118540. doi: 10.1016/j.bbamcr.2019.118540. Epub 2019 Aug 29. Review.

PMID:
31473256
2.

Mitochondrial 2,4-dienoyl-CoA reductase (Decr) deficiency and impairment of thermogenesis in mouse brown adipose tissue.

Mäkelä AM, Hohtola E, Miinalainen IJ, Autio JA, Schmitz W, Niemi KJ, Hiltunen JK, Autio KJ.

Sci Rep. 2019 Aug 19;9(1):12038. doi: 10.1038/s41598-019-48562-x.

3.

17B-hydroxysteroid dehydrogenases as acyl thioester metabolizing enzymes.

Hiltunen JK, Kastaniotis AJ, Autio KJ, Jiang G, Chen Z, Glumoff T.

Mol Cell Endocrinol. 2019 Jun 1;489:107-118. doi: 10.1016/j.mce.2018.11.012. Epub 2018 Nov 30. Review.

PMID:
30508570
4.

Impaired Mitochondrial Fatty Acid Synthesis Leads to Neurodegeneration in Mice.

Nair RR, Koivisto H, Jokivarsi K, Miinalainen IJ, Autio KJ, Manninen A, Poutiainen P, Tanila H, Hiltunen JK, Kastaniotis AJ.

J Neurosci. 2018 Nov 7;38(45):9781-9800. doi: 10.1523/JNEUROSCI.3514-17.2018. Epub 2018 Sep 28.

5.

Different opinion on the reported role of Poldip2 and ACSM1 in a mammalian lipoic acid salvage pathway controlling HIF-1 activation.

Bailey PSJ, Hiltunen JK, Dieckmann CL, Kastaniotis AJ, Nathan JA.

Proc Natl Acad Sci U S A. 2018 Aug 7;115(32):E7458-E7459. doi: 10.1073/pnas.1804041115. Epub 2018 Jul 24. No abstract available.

6.

Structural enzymology comparisons of multifunctional enzyme, type-1 (MFE1): the flexibility of its dehydrogenase part.

Kasaragod P, Midekessa GB, Sridhar S, Schmitz W, Kiema TR, Hiltunen JK, Wierenga RK.

FEBS Open Bio. 2017 Nov 6;7(12):1830-1842. doi: 10.1002/2211-5463.12337. eCollection 2017 Dec.

7.

Non-electron transfer chain mitochondrial defects differently regulate HIF-1α degradation and transcription.

Shvetsova AN, Mennerich D, Kerätär JM, Hiltunen JK, Kietzmann T.

Redox Biol. 2017 Aug;12:1052-1061. doi: 10.1016/j.redox.2017.05.003. Epub 2017 May 8.

8.

Genetic modifications of Mecr reveal a role for mitochondrial 2-enoyl-CoA/ACP reductase in placental development in mice.

Nair RR, Kerätär JM, Autio KJ, Masud AJ, Finnilä MAJ, Autio-Harmainen HI, Miinalainen IJ, Nieminen PA, Hiltunen JK, Kastaniotis AJ.

Hum Mol Genet. 2017 Jun 1;26(11):2104-2117. doi: 10.1093/hmg/ddx105.

PMID:
28369354
9.

MECR Mutations Cause Childhood-Onset Dystonia and Optic Atrophy, a Mitochondrial Fatty Acid Synthesis Disorder.

Heimer G, Kerätär JM, Riley LG, Balasubramaniam S, Eyal E, Pietikäinen LP, Hiltunen JK, Marek-Yagel D, Hamada J, Gregory A, Rogers C, Hogarth P, Nance MA, Shalva N, Veber A, Tzadok M, Nissenkorn A, Tonduti D, Renaldo F; University of Washington Center for Mendelian Genomics, Kraoua I, Panteghini C, Valletta L, Garavaglia B, Cowley MJ, Gayevskiy V, Roscioli T, Silberstein JM, Hoffmann C, Raas-Rothschild A, Tiranti V, Anikster Y, Christodoulou J, Kastaniotis AJ, Ben-Zeev B, Hayflick SJ.

Am J Hum Genet. 2016 Dec 1;99(6):1229-1244. doi: 10.1016/j.ajhg.2016.09.021. Epub 2016 Nov 3.

10.

Mitochondrial fatty acid synthesis, fatty acids and mitochondrial physiology.

Kastaniotis AJ, Autio KJ, Kerätär JM, Monteuuis G, Mäkelä AM, Nair RR, Pietikäinen LP, Shvetsova A, Chen Z, Hiltunen JK.

Biochim Biophys Acta Mol Cell Biol Lipids. 2017 Jan;1862(1):39-48. doi: 10.1016/j.bbalip.2016.08.011. Epub 2016 Aug 21. Review.

PMID:
27553474
11.

Dual targeted poplar ferredoxin NADP(+) oxidoreductase interacts with hemoglobin 1.

Jokipii-Lukkari S, Kastaniotis AJ, Parkash V, Sundström R, Leiva-Eriksson N, Nymalm Y, Blokhina O, Kukkola E, Fagerstedt KV, Salminen TA, Läärä E, Bülow L, Ohlmeier S, Hiltunen JK, Kallio PT, Häggman H.

Plant Sci. 2016 Jun;247:138-49. doi: 10.1016/j.plantsci.2016.03.013. Epub 2016 Mar 26.

PMID:
27095407
12.

Trim37-deficient mice recapitulate several features of the multi-organ disorder Mulibrey nanism.

Kettunen KM, Karikoski R, Hämäläinen RH, Toivonen TT, Antonenkov VD, Kulesskaya N, Voikar V, Hölttä-Vuori M, Ikonen E, Sainio K, Jalanko A, Karlberg S, Karlberg N, Lipsanen-Nyman M, Toppari J, Jauhiainen M, Hiltunen JK, Jalanko H, Lehesjoki AE.

Biol Open. 2016 May 15;5(5):584-95. doi: 10.1242/bio.016246.

13.

A monoclonal antibody raised against bacterially expressed MPV17 sequences shows peroxisomal, endosomal and lysosomal localisation in U2OS cells.

Weiher H, Pircher H, Jansen-Dürr P, Hegenbarth S, Knolle P, Grunau S, Vapola M, Hiltunen JK, Zwacka RM, Schmelzer E, Reumann K, Will H.

BMC Res Notes. 2016 Feb 27;9:128. doi: 10.1186/s13104-016-1939-0.

14.

Peroxisomal Pex11 is a pore-forming protein homologous to TRPM channels.

Mindthoff S, Grunau S, Steinfort LL, Girzalsky W, Hiltunen JK, Erdmann R, Antonenkov VD.

Biochim Biophys Acta. 2016 Feb;1863(2):271-83. doi: 10.1016/j.bbamcr.2015.11.013. Epub 2015 Nov 17.

15.

Phytol is lethal for Amacr-deficient mice.

Selkälä EM, Nair RR, Schmitz W, Kvist AP, Baes M, Hiltunen JK, Autio KJ.

Biochim Biophys Acta. 2015 Oct;1851(10):1394-405. doi: 10.1016/j.bbalip.2015.07.008. Epub 2015 Aug 4.

PMID:
26248199
16.

The Human Mitochondrial DNA Depletion Syndrome Gene MPV17 Encodes a Non-selective Channel That Modulates Membrane Potential.

Antonenkov VD, Isomursu A, Mennerich D, Vapola MH, Weiher H, Kietzmann T, Hiltunen JK.

J Biol Chem. 2015 May 29;290(22):13840-61. doi: 10.1074/jbc.M114.608083. Epub 2015 Apr 10.

17.

Quantitative changes in Gimap3 and Gimap5 expression modify mitochondrial DNA segregation in mice.

Jokinen R, Lahtinen T, Marttinen P, Myöhänen M, Ruotsalainen P, Yeung N, Shvetsova A, Kastaniotis AJ, Hiltunen JK, Öhman T, Nyman TA, Weiler H, Battersby BJ.

Genetics. 2015 May;200(1):221-35. doi: 10.1534/genetics.115.175596. Epub 2015 Mar 25.

18.

Insights into mitochondrial fatty acid synthesis from the structure of heterotetrameric 3-ketoacyl-ACP reductase/3R-hydroxyacyl-CoA dehydrogenase.

Venkatesan R, Sah-Teli SK, Awoniyi LO, Jiang G, Prus P, Kastaniotis AJ, Hiltunen JK, Wierenga RK, Chen Z.

Nat Commun. 2014 Sep 9;5:4805. doi: 10.1038/ncomms5805.

PMID:
25203508
19.

Role of AMACR (α-methylacyl-CoA racemase) and MFE-1 (peroxisomal multifunctional enzyme-1) in bile acid synthesis in mice.

Autio KJ, Schmitz W, Nair RR, Selkälä EM, Sormunen RT, Miinalainen IJ, Crick PJ, Wang Y, Griffiths WJ, Reddy JK, Baes M, Hiltunen JK.

Biochem J. 2014 Jul 1;461(1):125-35. doi: 10.1042/BJ20130915.

PMID:
24735479
20.

Dithiothreitol-capped fluorescent gold nanoclusters: an efficient probe for detection of copper(II) ions in aqueous solution.

Ding H, Liang C, Sun K, Wang H, Hiltunen JK, Chen Z, Shen J.

Biosens Bioelectron. 2014 Sep 15;59:216-20. doi: 10.1016/j.bios.2014.03.045. Epub 2014 Mar 31.

PMID:
24727608
21.

Peroxisomal membrane channel Pxmp2 in the mammary fat pad is essential for stromal lipid homeostasis and for development of mammary gland epithelium in mice.

Vapola MH, Rokka A, Sormunen RT, Alhonen L, Schmitz W, Conzelmann E, Wärri A, Grunau S, Antonenkov VD, Hiltunen JK.

Dev Biol. 2014 Jul 1;391(1):66-80. doi: 10.1016/j.ydbio.2014.03.022. Epub 2014 Apr 12.

22.

Dietary intake of n-3 long-chain polyunsaturated fatty acids and risk of myocardial infarction in coronary artery disease patients with or without diabetes mellitus: a prospective cohort study.

Strand E, Pedersen ER, Svingen GF, Schartum-Hansen H, Rebnord EW, Bjørndal B, Seifert R, Bohov P, Meyer K, Hiltunen JK, Nordrehaug JE, Nilsen DW, Berge RK, Nygård O.

BMC Med. 2013 Oct 8;11:216. doi: 10.1186/1741-7015-11-216.

23.

Defects in mitochondrial fatty acid synthesis result in failure of multiple aspects of mitochondrial biogenesis in Saccharomyces cerevisiae.

Kursu VA, Pietikäinen LP, Fontanesi F, Aaltonen MJ, Suomi F, Raghavan Nair R, Schonauer MS, Dieckmann CL, Barrientos A, Hiltunen JK, Kastaniotis AJ.

Mol Microbiol. 2013 Nov;90(4):824-40. doi: 10.1111/mmi.12402. Epub 2013 Oct 10.

24.

Metabolic adaptation allows Amacr-deficient mice to remain symptom-free despite low levels of mature bile acids.

Selkälä EM, Kuusisto SM, Salonurmi T, Savolainen MJ, Jauhiainen M, Pirilä PL, Kvist AP, Conzelmann E, Schmitz W, Alexson SE, Kotti TJ, Hiltunen JK, Autio KJ.

Biochim Biophys Acta. 2013 Aug;1831(8):1335-43. doi: 10.1016/j.bbalip.2013.05.002. Epub 2013 May 13.

PMID:
23680781
25.

Nutritional countermeasures targeting reactive oxygen species in cancer: from mechanisms to biomarkers and clinical evidence.

Samoylenko A, Hossain JA, Mennerich D, Kellokumpu S, Hiltunen JK, Kietzmann T.

Antioxid Redox Signal. 2013 Dec 10;19(17):2157-96. doi: 10.1089/ars.2012.4662. Epub 2013 Apr 15. Review.

26.

The isomerase and hydratase reaction mechanism of the crotonase active site of the multifunctional enzyme (type-1), as deduced from structures of complexes with 3S-hydroxy-acyl-CoA.

Kasaragod P, Schmitz W, Hiltunen JK, Wierenga RK.

FEBS J. 2013 Jul;280(13):3160-75. doi: 10.1111/febs.12150. Epub 2013 Feb 15.

27.

Quaternary structure of human, Drosophila melanogaster and Caenorhabditis elegans MFE-2 in solution from synchrotron small-angle X-ray scattering.

Mehtälä ML, Haataja TJ, Blanchet CE, Hiltunen JK, Svergun DI, Glumoff T.

FEBS Lett. 2013 Feb 14;587(4):305-10. doi: 10.1016/j.febslet.2012.12.014. Epub 2013 Jan 8.

28.

On the molecular basis of D-bifunctional protein deficiency type III.

Mehtälä ML, Lensink MF, Pietikäinen LP, Hiltunen JK, Glumoff T.

PLoS One. 2013;8(1):e53688. doi: 10.1371/journal.pone.0053688. Epub 2013 Jan 7.

29.

Channel-forming activities in the glycosomal fraction from the bloodstream form of Trypanosoma brucei.

Gualdron-López M, Vapola MH, Miinalainen IJ, Hiltunen JK, Michels PA, Antonenkov VD.

PLoS One. 2012;7(4):e34530. doi: 10.1371/journal.pone.0034530. Epub 2012 Apr 10.

30.

The enolization chemistry of a thioester-dependent racemase: the 1.4 Å crystal structure of a reaction intermediate complex characterized by detailed QM/MM calculations.

Sharma S, Bhaumik P, Schmitz W, Venkatesan R, Hiltunen JK, Conzelmann E, Juffer AH, Wierenga RK.

J Phys Chem B. 2012 Mar 22;116(11):3619-29. doi: 10.1021/jp210185m. Epub 2012 Mar 7.

PMID:
22360758
31.

Transfer of metabolites across the peroxisomal membrane.

Antonenkov VD, Hiltunen JK.

Biochim Biophys Acta. 2012 Sep;1822(9):1374-86. doi: 10.1016/j.bbadis.2011.12.011. Epub 2011 Dec 22. Review.

32.

Apicoplast and endoplasmic reticulum cooperate in fatty acid biosynthesis in apicomplexan parasite Toxoplasma gondii.

Ramakrishnan S, Docampo MD, Macrae JI, Pujol FM, Brooks CF, van Dooren GG, Hiltunen JK, Kastaniotis AJ, McConville MJ, Striepen B.

J Biol Chem. 2012 Feb 10;287(7):4957-71. doi: 10.1074/jbc.M111.310144. Epub 2011 Dec 16.

33.

Measurements of intracellular ATP provide new insight into the regulation of glycolysis in the yeast Saccharomyces cerevisiae.

Ytting CK, Fuglsang AT, Hiltunen JK, Kastaniotis AJ, Özalp VC, Nielsen LJ, Olsen LF.

Integr Biol (Camb). 2012 Jan;4(1):99-107. doi: 10.1039/c1ib00108f. Epub 2011 Dec 2.

PMID:
22134619
34.

Peroxisomal multifunctional enzyme type 2 from the fruitfly: dehydrogenase and hydratase act as separate entities, as revealed by structure and kinetics.

Haataja TJ, Koski MK, Hiltunen JK, Glumoff T.

Biochem J. 2011 May 1;435(3):771-81. doi: 10.1042/BJ20101661.

PMID:
21320074
35.

Protein phosphorylation in mitochondria --a study on fermentative and respiratory growth of Saccharomyces cerevisiae.

Ohlmeier S, Hiltunen JK, Bergmann U.

Electrophoresis. 2010 Sep;31(17):2869-81. doi: 10.1002/elps.200900759.

PMID:
20715123
36.

Identification of a substrate-binding site in a peroxisomal beta-oxidation enzyme by photoaffinity labeling with a novel palmitoyl derivative.

Kashiwayama Y, Tomohiro T, Narita K, Suzumura M, Glumoff T, Hiltunen JK, Van Veldhoven PP, Hatanaka Y, Imanaka T.

J Biol Chem. 2010 Aug 20;285(34):26315-25. doi: 10.1074/jbc.M110.104547. Epub 2010 Jun 21.

37.

Crystal structure of liganded rat peroxisomal multifunctional enzyme type 1: a flexible molecule with two interconnected active sites.

Kasaragod P, Venkatesan R, Kiema TR, Hiltunen JK, Wierenga RK.

J Biol Chem. 2010 Jul 30;285(31):24089-98. doi: 10.1074/jbc.M110.117606. Epub 2010 May 12.

38.

Mitochondrial fatty acid synthesis and respiration.

Hiltunen JK, Autio KJ, Schonauer MS, Kursu VA, Dieckmann CL, Kastaniotis AJ.

Biochim Biophys Acta. 2010 Jun-Jul;1797(6-7):1195-202. doi: 10.1016/j.bbabio.2010.03.006. Epub 2010 Mar 11. Review.

39.

Peroxisomes are oxidative organelles.

Antonenkov VD, Grunau S, Ohlmeier S, Hiltunen JK.

Antioxid Redox Signal. 2010 Aug 15;13(4):525-37. doi: 10.1089/ars.2009.2996. Review.

PMID:
19958170
40.

An involvement of yeast peroxisomal channels in transmembrane transfer of glyoxylate cycle intermediates.

Antonenkov VD, Mindthoff S, Grunau S, Erdmann R, Hiltunen JK.

Int J Biochem Cell Biol. 2009 Dec;41(12):2546-54. doi: 10.1016/j.biocel.2009.08.014. Epub 2009 Aug 25.

PMID:
19712748
41.

Mitochondrial fatty acid synthesis--an adopted set of enzymes making a pathway of major importance for the cellular metabolism.

Hiltunen JK, Chen Z, Haapalainen AM, Wierenga RK, Kastaniotis AJ.

Prog Lipid Res. 2010 Jan;49(1):27-45. doi: 10.1016/j.plipres.2009.08.001. Epub 2009 Aug 15. Review.

PMID:
19686777
42.

Avoiding unscheduled transcription in shared promoters: Saccharomyces cerevisiae Sum1p represses the divergent gene pair SPS18-SPS19 through a midsporulation element (MSE).

Gurvitz A, Suomi F, Rottensteiner H, Hiltunen JK, Dawes IW.

FEMS Yeast Res. 2009 Sep;9(6):821-31. doi: 10.1111/j.1567-1364.2009.00527.x. Epub 2009 May 6.

43.

Mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis.

Miinalainen IJ, Schmitz W, Huotari A, Autio KJ, Soininen R, Ver Loren van Themaat E, Baes M, Herzig KH, Conzelmann E, Hiltunen JK.

PLoS Genet. 2009 Jul;5(7):e1000543. doi: 10.1371/journal.pgen.1000543. Epub 2009 Jul 3.

44.

17beta-hydroxysteroid dehydrogenase type 8 and carbonyl reductase type 4 assemble as a ketoacyl reductase of human mitochondrial FAS.

Chen Z, Kastaniotis AJ, Miinalainen IJ, Rajaram V, Wierenga RK, Hiltunen JK.

FASEB J. 2009 Nov;23(11):3682-91. doi: 10.1096/fj.09-133587. Epub 2009 Jul 1.

PMID:
19571038
45.

Lipoic acid synthesis and attachment in yeast mitochondria.

Schonauer MS, Kastaniotis AJ, Kursu VA, Hiltunen JK, Dieckmann CL.

J Biol Chem. 2009 Aug 28;284(35):23234-42. doi: 10.1074/jbc.M109.015594. Epub 2009 Jul 1.

46.

Myocardial overexpression of Mecr, a gene of mitochondrial FAS II leads to cardiac dysfunction in mouse.

Chen Z, Leskinen H, Liimatta E, Sormunen RT, Miinalainen IJ, Hassinen IE, Hiltunen JK.

PLoS One. 2009;4(5):e5589. doi: 10.1371/journal.pone.0005589. Epub 2009 May 18.

47.

Pxmp2 is a channel-forming protein in Mammalian peroxisomal membrane.

Rokka A, Antonenkov VD, Soininen R, Immonen HL, Pirilä PL, Bergmann U, Sormunen RT, Weckström M, Benz R, Hiltunen JK.

PLoS One. 2009;4(4):e5090. doi: 10.1371/journal.pone.0005090. Epub 2009 Apr 7.

48.

Characterization of two cotton cDNAs encoding trans-2-enoyl-CoA reductase reveals a putative novel NADPH-binding motif.

Song WQ, Qin YM, Saito M, Shirai T, Pujol FM, Kastaniotis AJ, Hiltunen JK, Zhu YX.

J Exp Bot. 2009;60(6):1839-48. doi: 10.1093/jxb/erp057. Epub 2009 Mar 13.

49.

Channel-forming activities of peroxisomal membrane proteins from the yeast Saccharomyces cerevisiae.

Grunau S, Mindthoff S, Rottensteiner H, Sormunen RT, Hiltunen JK, Erdmann R, Antonenkov VD.

FEBS J. 2009 Mar;276(6):1698-708. doi: 10.1111/j.1742-4658.2009.06903.x. Epub 2009 Feb 13.

50.

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