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  • PL 63 (Haartmaninkatu 8)

    00014

    Finland

20072019
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Publications 2007 2019

2019

Instability of the mitochondrial alanyl-tRNA synthetase underlies fatal infantile-onset cardiomyopathy

Sommerville, E. W., Zhou, X-L., Olahova, M., Jenkins, J., Euro, L., Konovalova, S., Hilander, T., Pyle, A., He, L., Habeebu, S., Saunders, C., Kelsey, A., Morris, A. A. M., McFarland, R., Suomalainen, A., Gorman, G. S., Wang, E-D., Thiffault, I., Tyynismaa, H. & Taylor, R. W., 15 Jan 2019, In : Human Molecular Genetics. 28, 2, p. 258-268 11 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2018

Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria

Hilander, T., Zhou, X-L., Konovalova, S., Zhang, F-P., Euro, L., Shilov, D., Poutanen, M., Chihade, J., Wang, E-D. & Tyynismaa, H., 2018, In : Nucleic Acids Research. 46, 2, p. 849–860 12 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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Loss of mtDNA activates astrocytes and leads to spongiotic encephalopathy

Ignatenko, O., Chilov, D., Paetau, I., de Miguel, E., Jackson, C. B., Capin, G., Paetau, A., Terzioglu, M., Euro, L. & Suomalainen, A., 4 Jan 2018, In : Nature Communications. 9, 12 p., 70.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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Redox regulation of GRPEL2 nucleotide exchange factor for mitochondrial HSP70 chaperone

Konovalova, S., Liu, X., Manjunath, P., Baral, S., Neupane, N., Hilander, T., Yang, Y., Balboa, D., Terzioglu, M., Euro, L., Varjosalo, M. & Tyynismaa, H., Oct 2018, In : Redox biology. 19, p. 37-45 9 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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Simultaneous measurement of folate cycle intermediates in different biological matrices using liquid chromatography-tandem mass spectrometry

Nandania, J., Kokkonen, M., Euro, L. & Velagapudi, V., 15 Aug 2018, In : Journal of Chromatography. B. 1092, p. 168-178 11 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2017

Atomistic Molecular Dynamics Simulations of Mitochondrial DNA Polymerase gamma: Novel Mechanisms of Function and Pathogenesis

Euro, L., Haapanen, O., Rog, T., Vattulainen, I., Suomalainen, A. & Sharma, V., 7 Mar 2017, In : Biochemistry. 56, 9, p. 1227-1238 12 p.

Research output: Contribution to journalArticleScientificpeer-review

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ATPase-deficient mitochondrial inner membrane protein ATAD3A disturbs mitochondrial dynamics in dominant hereditary spastic paraplegia

Cooper, H. M., Yang, Y., Ylikallio, E., Khairullin, R., Woldegebriel, R., Lin, K-L., Euro, L., Palin, E., Wolf, A., Trokovic, R., Isohanni, P., Kaakkola, S., Auranen, M., Lonnqvist, T., Wanrooij, S. & Tyynismaa, H., 15 Apr 2017, In : Human Molecular Genetics. 26, 8, p. 1432-1443 12 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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Defective mitochondrial RNA processing due to PNPT1 variants causes Leigh syndrome

Matilainen, S., Carroll, C. J., Richter, U., Euro, L., Pohjanpelto, M., Paetau, A., Isohanni, P. & Suomalainen, A., 1 Sep 2017, In : Human Molecular Genetics. 26, 17, p. 3352-3361 10 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access

SDHA Mutation with Dominant Transmission Results in Complex II Deficiency with Ocular, Cardiac, and Neurologic Involvement

Courage, C., Jackson, C. B., Hahn, D., Euro, L., Nuoffer, J-M., Gallati, S. & Schaller, A., Jan 2017, In : American Journal of Medical Genetics. Part A. 173, 1, p. 225-230 6 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
2016

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

Nikkanen, J., Forsstrom, S., Euro, L., Paetau, I., Kohnz, R. A., Wang, L., Chilov, D., Viinamaki, J., Roivainen, A., Marjamaki, P., Liljenback, H., Ahola, S., Buzkova, J., Terzioglu, M., Khan, N. A., Pirnes-Karhu, S., Paetau, A., Lonnqvist, T., Sajantila, A., Isohanni, P. & 6 othersTyynismaa, H., Nomura, D. K., Battersby, B. J., Velagapudi, V., Carroll, C. J. & Suomalainen, A., 12 Apr 2016, In : Cell Metabolism. 23, 4, p. 635-648 14 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access

The Rare Costello Variant HRAS c.173C > T (p.T58I) with Severe Neonatal Hypertrophic Cardiomyopathy

Hiippala, A., Vasilescu, C., Tallila, J., Alastalo, T-P., Paetau, A., Tyni, T., Suomalainen, A., Euro, L. & Ojala, T., Jun 2016, In : American Journal of Medical Genetics. Part A. 170, 6, p. 1433-1438 6 p.

Research output: Contribution to journalArticleScientificpeer-review

Structural modeling of tissue-specific mitochondrial alanyl-tRNA synthetase (AARS2) defects predicts differential effects on aminoacylation.

Euro, L., Konovalova, S., Asin-Cayuela, J., Tulinius, M., Griffin, H., Horvath, R., Taylor, R. W., Chinnery, P. F., Schara, U., Thorburn, D. R., Wartiovaara, A., Chihade, J. & Tyynismaa, H., 2015, In : Frontiers in Genetics. 6, 12 p., 21.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2014

Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3

Khan, N. A., Auranen, M., Paetau, I., Pirinen, E., Euro, L., Forsström, S., Pasila, L., Velagapudi, V., Carroll, C. J., Auwerx, J. & Suomalainen, A., Jun 2014, In : EMBO molecular medicine. 6, 6, p. 721-731 11 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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Mitochondrial EFTs defects in juvenile-onset Leigh disease, ataxia, neuropathy, and optic atrophy

Ahola, S., Isohanni, P., Euro, L., Brilhante, V., Palotie, A., Pihko, H., Lonnqvist, T., Lehtonen, T., Laine, J., Tyynismaa, H. & Suomalainen, A., 19 Aug 2014, In : Neurology. 83, 8, p. 743-751 9 p.

Research output: Contribution to journalArticleScientificpeer-review

2013

Whole-exome sequencing identifies a mutation in the mitochondrial ribosome protein MRPL44 to underlie mitochondrial infantile cardiomyopathy

Carroll, C. J., Isohanni, P., Poyhonen, R., Euro, L., Richter, U., Brilhante, V., Gotz, A., Lahtinen, T., Paetau, A., Pihko, H., Battersby, B. J., Tyynismaa, H. & Suomalainen, A., Mar 2013, In : Journal of Medical Genetics. 50, 3, p. 151-159 9 p.

Research output: Contribution to journalArticleScientificpeer-review

2012

Biochemical Genetics of the Mitochondrial Replicase: Clustering of Pathogenic Mutations into Five Functional Modules in Human DNA Polymerase gamma

Kaguni, L. S., Farnum, G. A., Euro, L., Palin, E. & Wartiovaara, A., Apr 2012, In : FASEB Journal. 26, 1 p.

Research output: Contribution to journalArticleScientificpeer-review

Carrier Frequency of a Common Mutation of Carnitine Palmitoyltransferase 1A Deficiency and Long-Term Follow-Up in Finland

Roomets, E., Polinati, P. P., Euro, L., P., E., A., P. & Tyni, T., 2012, In : The Journal of Pediatrics. 160, 3, p. 473-479.e.1 8 p.

Research output: Contribution to journalArticleScientificpeer-review

Mitochondrial phenylalanyl-tRNA synthetase mutations underlie fatal infantile Alpers encephalopathy

Elo, J. M., Yadavalli, S. S., Euro, L., Isohanni, P., Götz, A., Carroll, C. J., Valanne, L., Alkuraya, F. S., Uusimaa, J., Paetau, A., Caruso, E. M., Pihko, H., Ibba, M., Tyynismaa, H. & Suomalainen, A., 15 Oct 2012, In : Human Molecular Genetics. 21, 20, p. 4521-4529 9 p.

Research output: Contribution to journalArticleScientificpeer-review

2011

Clustering of Alpers disease mutations and catalytic defects in biochemical variants reveal new features of molecular mechanism of hte human mitochondrial replicase, Pol gamma

Euro, L., Farnum, G. A., Palin, E., Suomalainen, A. & Kaguni, L. S., 2011, In : Nucleic Acids Research. 39, 21, p. 9072-9084 13 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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Cys377 Residue in NqrF Subunit Confers Ag+ Sensitivity of Na+-Translocating NADH:quinone Oxidoreductase from Vibrio harveyi

Fadeeva, M. S., Bertsova, Y. V., Euro, L. & Bogachev, A. V., 2011, In : Biochemistry (Moscow). 76, 2, p. 186-195 10 p.

Research output: Contribution to journalArticleScientificpeer-review

Exome Sequencing Identifies Mitochondrial Alanyl-tRNA Synthetase Mutations in Infantile Mitochondrial Cardiomyopathy

Götz, A., Tyynismaa, H., Euro, L., Ellonen, P., Hyötyläinen, T., Ojala, T., Hämäläinen, R. H., Tommiska, J., Raivio, T., Oresic, M., Karikoski, R., Tammela, O., Simola, K. O. J., Paetau, A., Tyni, T. & Suomalainen, A., 2011, In : American Journal of Human Genetics. 88, 5, p. 635-642 8 p.

Research output: Contribution to journalArticleScientificpeer-review

POLG1 manifestations in childhood: A study of 136 children

Isohanni, P., Hakonen, A. H., Euro, L., Paetau, I., Linnankivi, T., Liukkonen, E., Wallden, T., Luostarinen, L., Valanne, L., Paetau, A., Uusimaa, J., Lönnqvist, T., Suomalainen, A. & Pihko, H., 2011, In : Neurology. 76, 9, p. 811-815 5 p.

Research output: Contribution to journalArticleScientificpeer-review

2010

Functional analysis of H-sapiens DNA polymerase gamma spacer mutation W748S with and without common variant E1143G

Palin, E. J. H., Lesonen, A., Farr, C. L., Euro, L., Suomalainen, A. & Kaguni, L. S., 2010, In : Biochimica et Biophysica Acta. Molecular Basis of Disease. 1802, 6, p. 545-551 7 p.

Research output: Contribution to journalArticleScientificpeer-review

High affinity cation-binding sites in Complex I from Escherichia coli

Euro, L., Belevich, G., Wikström, M. & Verkhovskaya, M., 2009, In : Biochimica et Biophysica Acta. Bioenergetics. 1787, 8, p. 1024-1028 5 p.

Research output: Contribution to journalArticleScientificpeer-review

The role of the invariant glutamate 95 in the catalytic site of Complex I from Escherichia coli

Euro, L., Belevich, G., Bloch, D. A., Verkhovsky, M. I., Wikstrom, M. & Verkhovskaya, M., 2009, In : Biochimica et Biophysica Acta. Bioenergetics. 1787, 1, p. 68-73 6 p.

Research output: Contribution to journalArticleScientificpeer-review

2008

Conserved lysine residues of the membrane subunit NuoM are involved in energy conversion by the proton-pumping NADH:ubiquinone oxidoreductase (Complex I)

Euro, L., Belevich, G., Verkhovsky, M., Wikström, M. & Verkhovskaya, M., 2008, In : Biochimica et Biophysica Acta. Bioenergetics. 1777, 9, p. 1166-1172 7 p.

Research output: Contribution to journalArticleScientificpeer-review

Electrostatic interactions between FeS clusters in NADH:ubiquinone oxidoreductase (complex I) from Escherichia coli

Euro, L., Bloch, D. A., Wikström, M., Verkhovsky, M. & Verkhovskaya, M., 2008, In : Biochemistry. 47, 10, p. 3185-3193 9 p.

Research output: Contribution to journalArticleScientificpeer-review

Real-time electron transfer in respiratory complex I

Verkhovskaya, M., Belevich, N., Euro, L., Wikstrom, M. & Verkhovsky, M. I., 2008, In : Proceedings of the National Academy of Sciences of the United States of America. 105, 10, p. 3763-3767 5 p.

Research output: Contribution to journalArticleScientificpeer-review

2007

Role of the conserved arginine 274 and histidine 224 and 228 residues in the NuoCD subunit of complex I from Escherichia coli

Belevich, G., Euro, L., Wikström, M. & Verkhovskaya, M., 2007, In : Biochemistry. 46, 2, p. 526-533 8 p.

Research output: Contribution to journalArticleScientificpeer-review