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Brendan Battersby, Research Director

  • PL 56 (Viikinkaari 5)

    00014

    Finland

  • Viikinkaari 5, Biocenter 2

    00790 Helsinki

    Finland

19962019
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Publications 1996 2019

2019

A variant in MRPS14 (uS14m) causes perinatal hypertrophic cardiomyopathy with neonatal lactic acidosis, growth retardation, dysmorphic features and neurological involvement

Jackson, C. B., Huemer, M., Bolognini, R., Martin, F., Szinnai, G., Donner, B. C., Richter, U., Battersby, B. J., Nuoffer, J-M., Suomalainen, A. & Schaller, A., 15 Feb 2019, In : Human Molecular Genetics. 28, 4, p. 639-649 11 p.

Research output: Contribution to journalArticleScientificpeer-review

Mitochondrial Nascent Chain Quality Control Determines Organelle Form and Function

Battersby, B., Richter, U. & Safronov, O., 23 Sep 2019, In : ACS Chemical Biology.

Research output: Contribution to journalArticleScientificpeer-review

Mitochondrial stress response triggered by defects in protein synthesis quality control

Richter, U., Ng, K. Y., Suomi, F., Marttinen, P., Turunen, T., Jackson, C., Suomalainen, A., Vihinen, H., Jokitalo, E., Nyman, T. A., Isokallio, M. A., Stewart, J. B., Mancini, C., Brusco, A., Seneca, S., Lombes, A., Taylor, R. W. & Battersby, B. J., Feb 2019, In : Life Science Alliance. 2, 1, 17 p., 201800219.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2018

Mice harbouring a SCA28 patient mutation in AFG3L2 develop late-onset ataxia associated with enhanced mitochondrial proteotoxicity

Mancini, C., Hoxha, E., Iommarini, L., Brussino, A., Richter, U., Montarolo, F., Cagnoli, C., Parolisi, R., Gondor Morosini, D. I., Nicolò, V., Maltecca, F., Muratori, L., Ronchi, G., Geuna, S., Arnaboldi, F., Donetti, E., Giorgio, E., Cavalieri, S., Di Gregorio, E., Pozzi, E. & 11 othersFerrero, M., Riberi, E., Casari, G., Altruda, F., Turco, E., Gasparre, G., Battersby, B. J., Porcelli, A. M., Ferrero, E., Brusco, A. & Tempia, F., 1 Oct 2018, In : Neurobiology of Disease. 124, p. 14-28 15 p.

Research output: Contribution to journalArticleScientificpeer-review

Mitochondrial diseases: the contribution of organelle stress responses to pathology

Suomalainen, A. & Battersby, B. J., 1 Feb 2018, In : Nature Reviews. Molecular Cell Biology. 19, 2, p. 77-92 16 p.

Research output: Contribution to journalArticleScientificpeer-review

OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect

Thompson, K., Mai, N., Oláhová, M., Scialó, F., Formosa, L. E., Stroud, D. A., Garrett, M., Lax, N. Z., Robertson, F. M., Jou, C., Nascimento, A., Ortez, C., Jimenez-Mallebrera, C., Hardy, S. A., He, L., Brown, G. K., Marttinen, P., McFarland, R., Sanz, A., Battersby, B. J. & 5 othersBonnen, P. E., Ryan, M. T., Chrzanowska-Lightowlers, Z. M., Lightowlers, R. N. & Taylor, R. W., 1 Nov 2018, In : EMBO molecular medicine. 10, 11, 13 p., e9060.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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RNA modification landscape of the human mitochondrial tRNA(LYs) regulates protein synthesis

Richter, U., Evans, M. E., Clark, W. C., Marttinen, P., Shoubridge, E. A., Suomalainen, A., Wredenberg, A., Wedell, A., Pan, T. & Battersby, B. J., 27 Sep 2018, In : Nature Communications. 9, 11 p., 3966.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2017

A novel mitochondrial ATP6 frameshift mutation causing isolated complex V deficiency, ataxia and encephalomyopathy

Jackson, C. B., Hahn, D., Schroter, B., Richter, U., Battersby, B. J., Schmitt-Mechelke, T., Marttinen, P., Nuoffer, J-M. & Schaller, A., Jun 2017, In : European Journal of Medical Genetics. 60, 6, p. 345-351 7 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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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

Tissue-specific modulation of mitochondrial DNA segregation by a defect in mitochondrial division

Jokinen, R., Marttinen, P., Stewart, J. B., Dear, T. N. & Battersby, B. J., 15 Feb 2016, In : Human Molecular Genetics. 25, 4, p. 706-714 9 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
2015

Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness

Richter, U., Lahtinen, T., Marttinen, P., Suomi, F. & Battersby, B. J., 26 Oct 2015, In : Journal of Cell Biology. 211, 2, p. 373-389 17 p.

Research output: Contribution to journalArticleScientificpeer-review

Quantitative Changes in Gimap3 and Gimap5 Expression Modify Mitochondrial DNA Segregation in Mice

Jokinen, R., Lahtinen, T., Marttinen, P., Myohanen, M., Ruotsalainen, P., Yeung, N., Shvetsova, A., Kastaniotis, A. J., Hiltunen, J. K., Ohman, T., Nyman, T. A., Weiler, H. & Battersby, B. J., May 2015, In : Genetics. 200, 1, p. 221-U419 20 p.

Research output: Contribution to journalArticleScientificpeer-review

2014

No recombination of mtDNA after heteroplasmy for 50 generations in the mouse maternal germline

Hagstrom, E., Freyer, C., Battersby, B. J., Stewart, J. B. & Larsson, N-G., Jan 2014, In : Nucleic Acids Research. 42, 2, p. 1111-1116 6 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2013

A Mitochondrial Ribosomal and RNA Decay Pathway Blocks Cell Proliferation

Richter, U., Lahtinen, T., Marttinen, P., Myohanen, M., Greco, D., Cannino, G., Jacobs, H. T., Lietzen, N., Nyman, T. A. & Battersby, B. J., 18 Mar 2013, In : Current Biology. 23, 6, p. 535-541 7 p.

Research output: Contribution to journalArticleScientificpeer-review

Insight into mammalian mitochondrial DNA segregation

Jokinen, R. & Battersby, B. J., 1 Mar 2013, In : Annals of Medicine. 45, 2, p. 149-155 7 p.

Research output: Contribution to journalArticleScientificpeer-review

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

2011

Gimap3: a foot-in-the-door to tissue-specific regulation of mitochondrial DNA genetics

Jokinen, R., Junnila, H. & Battersby, B., 2011, In : Small GTPases. 2, 1, p. 31-35 5 p.

Research output: Contribution to journalArticleScientificpeer-review

2010

Gimap3 Regulates Tissue-Specific Mitochondrial DNA Segregation

Jokinen, R., Marttinen, P., Sandell, H. K., Manninen, T., Teerenhovi, H., Wai, T., Teoli, D., Loredo-Osti, J. C., Shoubridge, E. A. & Battersby, B. J., 2010, In : PLoS Genetics. 6, 10 , p. e1001161 9 p.

Research output: Contribution to journalArticleScientificpeer-review

Open Access
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2007

Reactive oxygen species and the segregation of mtDNA sequence variants

Battersby, B. J. & Shoubridge, E. A., 2007, In : Nature Genetics. 39, 5, p. 571-572 2 p.

Research output: Contribution to journalArticleScientificpeer-review

2005

Mitochondrial DNA segregation in hematopoietic lineages does not depend on MHC presentation of mitochondrially-encoded peptides

Battersby, B., Redpath, M. E. & Shoubridge, E. A., 2005, In : Human Molecular Genetics. 14, 17, p. 2587-2594

Research output: Contribution to journalArticleScientificpeer-review

Rapid directional shift of mitochondrial DNA heteroplasmy in animal tissues by a mitochondrially targeted restriction endonuclease

Bayona-Bafaluy, M. P., Blits, B., Battersby, B. J., Shoubridge, E. A. & Moraes, C. T., 2005, In : Proceedings of the National Academy of Sciences of the United States of America. 102, 40, p. 14392-14397 6 p.

Research output: Contribution to journalArticleScientificpeer-review

2003

Nuclear genetic control of mitochondrial DNA segregation

Battersby, BJ., Loredo-Osti, JC. & Shoubridge, EA., Feb 2003, In : Nature Genetics. 33, 2, p. 183-186 4 p.

Research output: Contribution to journalArticleScientificpeer-review

Prospect of preimplantation genetic diagnosis for heritable mitochondrial DNA diseases

Dean, NL., Battersby, BJ., Ao, A., Gosden, RG., Tan, SL. & Shoubridge, EA., 1 Oct 2003, In : Molecular Human Reproduction. 9, 10, p. 631-638 8 p.

Research output: Contribution to journalArticleScientificpeer-review

2001

Correlations of plasma lipid metabolites with hibernation and lactation in wild black bears Ursus americanus

LeBlanc, PJ., Obbard, M., Battersby, BJ., Felskie, AK., Brown, L., Wright, PA. & Ballantyne, JS., May 2001, In : Journal of comparative physiology. B, Biochemical, systems, and environmental physiology. 171, 4, p. 327-334 8 p.

Research output: Contribution to journalArticleScientificpeer-review

Selection of a mtDNA sequence variant in hepatocytes of heteroplasmic mice is not due to differences in respiratory chain function or efficiency of replication

Battersby, BJ. & Shoubridge, EA., 15 Oct 2001, In : Human Molecular Genetics. 10, 22, p. 2469-2479 11 p.

Research output: Contribution to journalArticleScientificpeer-review

1999

Lactation during hibernation in wild black bears: Effects on plasma amino acids and nitrogen metabolites

Wright, PA., Obbard, ME., Battersby, BJ., Felskie, AK., LeBlanc, PJ. & Ballantyne, JS., 1999, In : Physiological and Biochemical Zoology. 72, 5, p. 597-604 8 p.

Research output: Contribution to journalArticleScientificpeer-review

1998

Are there distinct subcellular populations of mitochondria in rainbow trout red muscle?

Battersby, BJ. & Moyes, CD., Aug 1998, In : Journal of Experimental Biology. 201, 16, p. 2455-2460 6 p.

Research output: Contribution to journalArticleScientificpeer-review

Influence of acclimation temperature on mitochondrial DNA, RNA, and enzymes in skeletal muscle

Battersby, BJ. & Moyes, CD., Sep 1998, In : American Journal of Physiology: Regulatory, Integrative and Comparative Physiology. 275, 3, p. R905-R912 8 p.

Research output: Contribution to journalArticleScientificpeer-review

Inter-tissue differences in mitochondrial enzyme activity, RNA and DNA in rainbow trout (Oncorhynchus mykiss)

Leary, SC., Battersby, BJ. & Moyes, CD., Dec 1998, In : Journal of Experimental Biology. 201, 24, p. 3377-3384 8 p.

Research output: Contribution to journalArticleScientificpeer-review

Regulation of muscle mitochondrial design

Moyes, CD., Battersby, BJ. & Leary, SC., Feb 1998, In : Journal of Experimental Biology. 201, 3, p. 299-307 9 p.

Research output: Contribution to journalReview ArticleScientificpeer-review

1997

Short-term effects of 3,5,3'-triiodothyronine on the activity of selected enzymes of intermediary metabolism in the liver of the Atlantic hagfish, Myxine glutinosa

Leary, SC., Reddy, K., Battersby, BJ., Dugan, S. & Ballantyne, JS., 1 Aug 1997, In : Journal of Experimental Zoology. 278, 5, p. 333-337 5 p.

Research output: Contribution to journalArticleScientificpeer-review

1996

Short-term effects of 3,5,3'-triiodothyronine on the intermediary metabolism of the dogfish shark Squalus acanthias: Evidence from enzyme activities

Battersby, BJ., McFarlane, WJ. & Ballantyne, JS., 15 Feb 1996, In : Journal of Experimental Zoology. 274, 3, p. 157-162 6 p.

Research output: Contribution to journalArticleScientificpeer-review