Selected Publications

Hiltunen, J.K., Kastaniotis, A.J., Autio, K.J., Jiang, G.,1,  Chen, Z. & Glumoff, T. (2018) 17B-Hydroxysteroid dehydrogenases as acyl thioester metabolizing enzymes. Mol. Cell Endocrin. doi: 10.1016/j.mce.2018.11.01

Nair, R.R., Koivisto, H., Jokivarsi, K., Miinalainen, I.J., Autio, K.J., Manninen, A., Poutiainen, P., Tanila, H., Hiltunen, J.K. & Kastaniotis, A.J. (2018) Impaired Mitochondrial Fatty Acid Synthesis Leads to Neurodegeneration in Mice. J. Neurosci. 38, 9781-9800

Nair, R.R., Kerätär, J.M., Autio, K.J., Masud, A.J., Finnilä, M.A.J., Autio-Harmainen, H.I., Miinalainen, I.J. Nieminen, P.A., Hiltunen, J.K., Kastaniotis, A.J. (2017) Genetic medifications of Mecr reveal a role for mitochondrial 2-enoyl-CoA/ACP reductase in placental development in mice. Human Mol. Genetic, 26, 2104-2117

Kastaniotis, A.J., Autio, K.J., Kerätär, J.M,, Monteuuis, G., Mäkelä, A.M., Nair, R.R., Pietikäinen, L.P., Shvetsova, A., Chen, Z. & Hiltunen, J.K. (2017) Mitochondrial fatty acid synthesis, fatty acids and mitochondrial physiology. Biochim Biophys Acta. 1862, 39-48

Shvetsova, A., Mennerich, D., Kerätär, J. M. Hiltunen, J.K., & Kietzmann, T. (2017) Non-electron transfer chain mitochondrial defects differently regulate HIF-1α degradation and transcription. Redox Biol. 12, 1052-1061

Jenkins, B.J., Seyssel, K., Chiu, S., Pan, P.H., Lin, S.Y., Stanley, E., Ament, Z., West, J.A., Summerhill, K., Griffin, J.L., Vetter, W., Autio, K.J., Hiltunen, K., Hazebrouck, S., Stepankova, R., Chen, C.J,, Alligier, M., Laville, M., Moore, M., Kraft, G., Cherrington, A., King, S., Krauss, R.M,, de Schryver, E., Van Veldhoven, P.P., Ronis, M. &Koulman, A. (2017) Odd Chain Fatty Acids; New Insights of the Relationship Between the Gut Microbiota, Dietary Intake, Biosynthesis and Glucose Intolerance. Sci. Rep. 7, 44845, doi: 10.1038/srep44845

Heimer, G., Kerätär, J.M,, Riley, L.G., Balasubramaniam, S., Eyal, E., Pietikäinen, L.P., Hiltunen, J.K., Marek-Yagel, D., Hamada, J., Gregory, A., Rogers, C., Hogarth, P., Nance, M.A., 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, M.J., Gayevskiy, V., Roscioli, T., Silberstein JM, Hoffmann C, Raas-Rothschild A, Tiranti V, Anikster Y, Christodoulou J., Kastaniotis, A.J., Ben-Zeev, B. & Hayflick, S.J.. (2016) MECR Mutations Cause Childhood-Onset Dystonia and Optic Atrophy, a Mitochondrial Fatty Acid Synthesis Disorder. Am. J. Hum. Genet. 99, 1229-1224

Jokipii-Lukkari, S., Kastaniotis A.J., Nymalm, Y., Sundström, R., Kallio, P.T., Fagerstedt, K.V., Salminen, T.A., Hiltunen, J.K. &  Häggman,  H. (2016) Coexpression of hybrid aspen PttHb1 and ferredoxin NADP+ oxidoreductase alleviates nitric oxide sensitivity in vivo. Plant Sci. 247, 138-149

Kettunen, K.M., Karikoski, R., Hämäläinen, R.H., Toivonen, T.T., Antonenkov, V.D., Kulesskaya, N., Voikar, V., Holtta-Vuori, M., Ikonen, E., Sainio, K., Jalanko, A., Karlberg, S., Karlberg, N., Lipsanen-Nyman, M., Toppari, J.,  Jauhiainen, M.,  Hiltunen, J.K., Jalanko, H. & Lehesjoki, A.-E. (2016) Trim37-deficient Mice Recapitulate Several Features of the Multi-organ Disorder Mulibrey Nanism. BiolOpen. 5, 584-595

Mindthoff, S., Grunau, S., Steinfort, L., Girzalsky, W., Hiltunen, J. K., Erdmann, R. & Antonenkov, V.D.  (2016) Peroxisomal Pex11 is a pore-forming protein homologous to TRPM channels. BBA - Molecular Cell Research, 1863, 271-283

Liu P, Wang X, Hiltunen K, Chen Z. (2015) Controllable Drug Release System in Living Cells Triggered by Enzyme-Substrate Recognition. ACS Appl. Mater. Interfaces. 7, 26811-26818

Selkälä, E.M., Nair, R.R., Schmitz, W., Kvist, A.-P., Baes, M.,  Hiltunen, J.K. & Autio, K.J. (2015) Phytol is lethal for Amacr-deficient mice BBA – Molec. Cell Biol. Lipids 1851, 1394-13405

Liu, P.C., Wang, H., Hiltunen, J.K., Chen, Z.J., Shen, J.C., (2015) Cross-Linked Proteins with Gold Nanoclusters: A Dual-Purpose pH-Responsive Material for Controllable Cell Imaging and Antibiotic Delivery  Part. Part. Syst. Char. 32, 749-755

Battersby, B. J. (2015) Quantitative changes in Gimap3 and Gimap5 expression modify mitochondrial DNA segregation in mice. Genetics, 200, 221-235

Antonenkov, V.D., Isomursu, A. Mennerich, D., Vapola, M.H., Weiher, H., Kietzmann, T. & Hiltunen, J. K. (2015) The human mitochondrial mtDNA depletion syndrome gene Mpv17 encodes a sensitive to mitochondrial conditions non-selective channel that modulates membrane protential. J. Biol. Chem. 290, 13840-13861

Venkatesan, R., Sah-Teli, S.K., Awoniyi, L.O., Jiang, G., Prus, P., Kastaniotis, A.J., Hiltunen, J.K., Wierenga, R.K. & Chen, Z. (2014) Insight into mitochondrial fatty acid synthesis from the structure of heterotetrameric 3-ketoacyl-ACP reductase/3R-hydorxyacyl-CoA dehydrogenase. Nature Comm. DOI: 10.1038/ncomms5805

Vapola, M.H., Rokka, A., Sormunen, R.T., Alhonen, L., Schmitz, W., Conzelmann, E., Wärri, A., Grunau, S., Antonenkov, V.D. & Hiltunen, J.K. (2014) Peroxisomal membrane channel Pxmp2 in the mammary gland fat pad is essential for stromal lipid homeostasis and development of mammary gland epithelium in mice. Dev. Biol. 391, 66-80

Ding, H, Zhou, M., Liu, P., Li H., Wu, Y., Hiltunen, J. K. Chen, Z. & Shen, J. (2014) Bacterial recombinant protein-directed synthesis of gold nanoclusters. Microchim. Acta, 181, 1029-1034

Autio, K. J., Schmitz, W., Nair, R.R.,  Selkälä, E.M., Sormunen R.T., Miinalainen, I.J., Crick, P.J., Wang, Y., Griffiths, W.J., Reddy, J.K., Baes, M.  &  Hiltunen. J.K. (2014)  Role of α-Methylacyl-CoA Racemase (Amacr) and peroxisomal Multifunctional Enzyme 1 (Mfe-1) in bile acid synthesis in mice. Biochem J. 461, 125-135

Ding, H., Liang, C., Sun, K., Wang, H., Hiltunen, J. K., Chen, Z. & Shen, J.  (2014) Dithiothreitol-capped fluorescent gold nanoclusters: an efficient probe for detection of copper(II) ions in aqueous solution. Biosens. Bioelectron, 59, 216-220

Strand, E., Pedersen, E.R., Ebbing, M., Svingen, G. F.T., Schartum-Hansen, H., Bjørndal, B., Seifert, R., Bohov, P., Meyer, K., Hiltunen, J.K., Nordrehaug, J. E., Nilsen, D. W.T., Berge, R.K. & Nygård, O. (2013)  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. BMC Med. doi: 10.1186/1741-7015-11-216

Kursu, V.A,, Pietikäinen, L.P., Fontanesi, F., Aaltonen, M.J., Suomi, F., Raghavan Nair, R., Schonauer, M.S., Dieckmann, C.L., Barrientos, A., Hiltunen, J.K., Kastaniotis, A.J. (2013) Defects in mitochondrial fatty acid synthesis result in failure of multiple aspects of mitochondrial biogenesis in Saccharomyces cerevisiae. Mol Microbiol. 90, 824-840

Samoylenko, A., AL Hossain, J.A, Kellokumpu, S., Hiltunen, J.K. & Kietzmann, T. (2013) Nutritional Countermeasures Targeting Reactive Oxygen Species in Cancer: From Mechanisms to Biomarkers and Clinical Evidence. Antioxid. Redox Signal. 19, 2157-2196

Selkälä, E.M., Kuusisto, S.M., Salonurmi, T., Savolainen, M.J., Jauhiainen, M., Pirilä, P.L., Kvist, A.-P., Conzelmann, E., Schmitz, W., Alexson, S.E., Kotti, T. J., Hiltunen, J.K. &. Autio, K. J. (2013) Metabolic adaptation allows Amacr-deficient mice to remain symptom-free despite low levels of mature bile acids. Biochim. Biophys Acta - Mol. Cell Biol. Lipids 1831, 1335-1345

Hiltunen, J.K., Chen, Z., Haapalainen, A.M., Wierenga, R.K. & Kastaniotis, A.J. (2010) Mitochondrial fatty acid synthesis –an adopted set of enzymes making a pathway of major importance for cellular metabolism. Prog. Lipid Res. 49, 27-45

Sharma, S., Bhaumik, P., Schmitz, W., Venkatesan, R., Hiltunen, J.K., Conzelmann, E., Juffer, A. & Wierenga, R.K. 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. (2012) J. Physical. Chem. B 116, 3619-3629

Glualdron-Lopez, M., Vapola, M.H. Miinalainen, I.J., Hiltunen, J.K., Michels, P.A.M. & Antonenkov, V.D. (2012) Channel-forming activities in the glycosomal farction from bloodstream form of Trypanosoma brucei. Plos One 7, e34530

Ytting, C. K., Fuglsang, A.T., Hiltunen, J. K., Kastaniotis, A. J., Özalp, V. C., Junker Nielsen, L. & Olsen, L. F. (2012) Measurements of intracellular ATP provide new insight into the regulation of glycolysis in the yeast Saccharomyces cerevisiae. Integrat. Biol. 4, 99-107

Antonenkov, V.D, Grunau, S., Ohlmeier, S. & Hiltunen, J.K. (2010) Peroxisomes are oxidative organelles. Antioxid Redox Signal. 13, 525-537

Ramakrishnan, S., Docampo, M.D., MacRae, J.I., Pujol, F.M., Brooks, C.F., vanDooren, G.G., Hiltunen, J.K., Kastaniotis, A.J., McConville, M. & Striepen, B. (2012) Apicoplast and endoplastic reticulum cooperate infatty acid biosynthesis in the apicocomplexan parasite Toxoplasma gondii. J. Biol. Chem. 287, 4957-4971

Haataja, T.J.K., Koski, M.K., Hiltunen, J.K. & Glumoff, T. (2011) Peroxisomal multifunctional enzyme type 2 from fruit fly: dehydrogenase and hydrotase act as separate entities as revealed by structure and kinetics. Biochem J. 435, 771-781

Kasaragod, P., Venkatesan, R., Keima, T. R., Hiltunen, J.K. & Wierenga, R.K. (2010) Crystal structure of liganded rat peroxisomal multifunctional enzyme type 1: a flexible structure with two active sites. J. Biol. Chem. 285, 24089-24098

Chen, Z.-J., Kastaniotis, A. J., Miinalainen, I. J., Rajaram,V., Wierenga, R. K. & Hiltunen, J. K. (2009) Heteromeric (17β-HSD8)2(CBR4)2 is the ketoacyl reductase (KAR) of mitochondrial FAS in human. FASEB J. 23, 3682-3691

Antonenkov V.D., Mindfhoff, S., Grunau, S., Erdmann, R. & Hiltunen, J.K. (2009) On the role of yest peroxisomal channels in transmembrane transfer of glyoxylate cycle intermediates. Intl. J. Biochem. Cell Biol. 41, 2546-2554

Miinalainen, I.J., Schmitz, W., Soininen, R., Autio, K.J., Huotari, A., Ver Loren van Themaat, E., Baes, M., Herzig, K.-H., Conzelmann, E. & Hiltunen, J.K. (2009) Mitochondrial 2,4-dienoyl-CoA reductase-deficiency in mice results in severe hypoglycaemia with stress intolerance and unimpaired ketogenesis. Plos Genetics 5, e1000543

Chen, Z.-J., Leskinen, H., Liimatta, E., Sormunen, R.T, Miinalainen, I. J., Hassinen, I., E. & Hiltunen, J. K. (2009) Overexpression of 2-enoyl thioester reductase of mitochondrial FAS II in myocardial leads to cardiac dysfunction in mouse. Plos One 4, e5589

Rokka, A., Antonenkov, V.D, Soininen, R., Immonen, H.L., Pirilä, P.L., Bergmann, U., Sormunen, R.T., Weckström, M., Benz, R. & Hiltunen, J.K. (2009) Pxmp2 is a channe-forming protein in mammalian peroxisomal membrane. Plos One 4, e5090

Viimeksi päivitetty: 9.1.2019