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Author

  • Aleksandra Filipovska
2018

Modular ssDNA binding and inhibition of telomerase activity by designer PPR proteins

Spåhr, H., Chia, T., Lingford, J. P., Siira, S. J., Cohen, S. B., Filipovska, A. & Rackham, O., 7 Jun 2018, In : Nature Communications. 9, 1, p. 2212 2212.

Research output: Contribution to journalArticle

Open Access
6 Citations (Scopus)
2019

Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome

Lake, N. J., Webb, B. D., Stroud, D. A., Richman, T. R., Ruzzenente, B., Compton, A. G., Mountford, H. S., Pulman, J., Zangarelli, C., Rio, M., Bodaert, N., Assouline, Z., Sherpa, M. D., Schadt, E. E., Houten, S. M., Byrnes, J., McCormick, E. M., Zolkipli-Cunningham, Z., Haude, K., Zhang, Z. & 11 others, Retterer, K., Bai, R., Calvo, S. E., Mootha, V. K., Christodoulou, J., Rotig, A., Filipovska, A., Cristian, I., Falk, M. J., Metodiev, M. D. & Thorburn, D. R., Jul 2019, In : European Journal of Human Genetics. 27, p. 181-182 2 p.

Research output: Contribution to journalAbstract/Meeting Abstract

2020

Cardiolipin is required for membrane docking of mitochondrial ribosomes and protein synthesis

Lee, R. G., Gao, J., Siira, S. J., Shearwood, A. M., Ermer, J. A., Hofferek, V., Mathews, J. C., Zheng, M., Reid, G. E., Rackham, O. & Filipovska, A., 24 Jul 2020, In : Journal of Cell Science. 133, 14

Research output: Contribution to journalArticle

Open Access
1 Citation (Scopus)
2014

A bifunctional protein regulates mitochondrial protein synthesis

Richman, T., Davies, S., Shearwood, A-M., Ermer, J., Scott, L., Hibbs, M., Rackham, O. & Filipovska, A., 5 Mar 2014, In : Nucleic Acids Research. 42, 9, p. 5483-5494

Research output: Contribution to journalArticle

Open Access
13 Citations (Scopus)
2017

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

Lake, N. J., Webb, B. D., Stroud, D. A., Richman, T. R., Ruzzenente, B., Compton, A. G., Mountford, H. S., Pulman, J., Zangarelli, C., Rio, M., Bodaert, N., Assouline, Z., Sherpa, M. D., Schadt, E. E., Houten, S. M., Byrnes, J., McCormick, E. M., Zolkipli-Cunningham, Z., Haude, K., Zhang, Z. & 11 others, Retterer, K., Bai, R., Calvo, S. E., Mootha, V. K., Christodoulou, J., Rötig, A., Filipovska, A., Cristian, I., Falk, M. J., Metodiev, M. D. & Thorburn, D. R., 3 Aug 2017, In : American Journal of Human Genetics. 101, 2, p. 239-254 16 p.

Research output: Contribution to journalArticle

Open Access
26 Citations (Scopus)

LRPPRC-mediated folding of the mitochondrial transcriptome

Siira, S. J., Spåhr, H., Shearwood, A. M. J., Ruzzenente, B., Larsson, N. G., Rackham, O. & Filipovska, A., 1 Dec 2017, In : Nature Communications. 8, 1, 1532.

Research output: Contribution to journalArticle

Open Access
29 Citations (Scopus)
2014

Founder p.Arg 446* mutation in the PDHX gene explains over half of cases with congenital lactic acidosis in Roma children

Ivanov, I. S., Azmanov, D., Ivanova, M. B., Chamova, T., Pacheva, I. H., Panova, M. V., Song, S., Morar, B., Yordanova, R. V., Galabova, F. K., Sotkova, I. G., Linev, A. J., Bitchev, S., Shearwood, A-M., Kancheva, D., Gabriková, D., Karcagi, V., Guergueltcheva, V., Geneva, I. E., Bozhinova, V. & 9 others, Stoyanova, V. K., Kremensky, I. M., Jordanova, A. G., Savov, A. S., Horváth, R., Brown, M. A., Tournev, I., Filipovska, A. & Kalaydjieva, L., Sep 2014, In : Molecular Genetics and Metabolism. 113, 1-2, p. 76-83

Research output: Contribution to journalArticle

Open Access
7 Citations (Scopus)
2018

Shining LIGHT on the metabolic role of the cytokine TNFSF14 and the implications on hepatic IL-6 production

Saunders, B. M., Rudnicka, C., Filipovska, A., Davies, S., Ward, N., Hricova, J., Schlaich, M. P. & Matthews, V. B., 1 Jan 2018, In : Immunology and Cell Biology. 96, 1, p. 41-53 13 p.

Research output: Contribution to journalArticle

3 Citations (Scopus)

Tighter Ligand Binding Can Compensate for Impaired Stability of an RNA-Binding Protein

Wallis, C. P., Richman, T. R., Filipovska, A. & Rackham, O., 15 Jun 2018, In : ACS Chemical Biology. 13, 6, p. 1499-1505 7 p.

Research output: Contribution to journalArticle

3 Citations (Scopus)

A modified yeast three-hybrid system enabling both positive and negative selections

Wallis, C. P., Filipovska, A. & Rackham, O., Jul 2018, In : Biotechnology Letters. 40, 7, p. 1127-1134 8 p.

Research output: Contribution to journalArticle

Open Access
3 Citations (Scopus)
2013
26 Citations (Scopus)

Organelle transcriptomes: products of a deconstructed genome

Small, I., Rackham, O. & Filipovska, A., Oct 2013, In : Current Opinion in Microbiology. 16, 5, p. 652-658

Research output: Contribution to journalReview article

Open Access
File
36 Citations (Scopus)
248 Downloads (Pure)
2018

Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria

Matic, S., Jiang, M., Nicholls, T. J., Uhler, J. P., Dirksen-Schwanenland, C., Polosa, P. L., Simard, M. L., Li, X., Atanassov, I., Rackham, O., Filipovska, A., Stewart, J. B., Falkenberg, M., Larsson, N. G. & Milenkovic, D., 1 Dec 2018, In : Nature Communications. 9, 1, 1202.

Research output: Contribution to journalArticle

Open Access
15 Citations (Scopus)

PTCD1 Is Required for 16S rRNA Maturation Complex Stability and Mitochondrial Ribosome Assembly

Perks, K. L., Rossetti, G., Kuznetsova, I., Hughes, L. A., Ermer, J. A., Ferreira, N., Busch, J. D., Rudler, D. L., Spahr, H., Schöndorf, T., Shearwood, A. M. J., Viola, H. M., Siira, S. J., Hool, L. C., Milenkovic, D., Larsson, N. G., Rackham, O. & Filipovska, A., 3 Apr 2018, In : Cell Reports. 23, 1, p. 127-142 16 p.

Research output: Contribution to journalArticle

Open Access
13 Citations (Scopus)
2017

Adult-onset obesity is triggered by impaired mitochondrial gene expression

Perks, K. L., Ferreira, N., Richman, T. R., Ermer, J. A., Kuznetsova, I., Shearwood, A-M. J., Lee, R. G., Viola, H. M., Johnstone, V. P. A., Matthews, V., Hool, L. C., Rackham, O. & Filipovska, A., Aug 2017, In : Science Advances. 3, 8, p. e1700677

Research output: Contribution to journalArticle

Open Access
16 Citations (Scopus)
2016

Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly

Rackham, O., Busch, J. D., Matic, S., Siira, S., Kuznetsova, I., Atanassov, I., Ermer, J., Shearwood, A-M., Richman, T., Stewart, J. B., Mourier, A., Milenkovic, D., Larsson, N. G. & Filipovska, A., 16 Aug 2016, In : Cell Reports. 16, 7, p. 1874-1890

Research output: Contribution to journalArticle

Open Access
38 Citations (Scopus)
2018
14 Citations (Scopus)
2017

Defects in RNA metabolism in mitochondrial disease

Siira, S. J., Shearwood, A. M. J., Bracken, C. P., Rackham, O. & Filipovska, A., 1 Apr 2017, In : International Journal of Biochemistry and Cell Biology. 85, p. 106-113 8 p.

Research output: Contribution to journalArticle

Open Access
File
2 Citations (Scopus)
287 Downloads (Pure)
2019

Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation

Ferreira, N., Perks, K. L., Rossetti, G., Rudler, D. L., Hughes, L. A., Ermer, J. A., Scott, L. H., Kuznetsova, I., Richman, T. R., Narayana, V. K., Abudulai, L. N., Shearwood, A-M. J., Cserne Szappanos, H., Tull, D., Yeoh, G. C., Hool, L. C., Filipovska, A. & Rackham, O., 16 Dec 2019, In : The EMBO Journal. 38, 24, 19 p., e102155.

Research output: Contribution to journalArticle

Open Access
File
4 Citations (Scopus)
178 Downloads (Pure)
2013
Open Access
File
30 Citations (Scopus)
425 Downloads (Pure)
2017

Review of machine learning algorithms in differential expression analysis

Kuznetsova, I., Karpievitch, Y. V., Filipovska, A., Lugmayr, A. & Holzinger, A., 2017, Proceedings of the 9th Workshop on Semantic Ambient Media Experiences : Visualisation, Emerging Media, and User Experience. Lugmayr, A., Seale, R., Woods, A., Sari, E. & Tedjasaputra, A. (eds.). Australia: International Ambient Media Association (iAMEA), p. 11-24 14 p. ( International Series on Information Systems and Management in Creative eMedia; vol. 2016, no. 2).

Research output: Chapter in Book/Conference paperConference paper

Open Access
2019

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

Nicholls, T. J., Spåhr, H., Jiang, S., Siira, S. J., Koolmeister, C., Sharma, S., Kauppila, J. H. K., Jiang, M., Kaever, V., Rackham, O., Chabes, A., Falkenberg, M., Filipovska, A., Larsson, N. G. & Gustafsson, C. M., 5 Dec 2019, In : Molecular Cell. 76, 5, p. 784-796.e6

Research output: Contribution to journalArticle

Open Access
1 Citation (Scopus)
2018

Is mitochondrial gene expression coordinated or stochastic?

Lee, R. G., Rudler, D. L., Rackham, O. & Filipovska, A., 19 Oct 2018, In : Biochemical Society Transactions. 46, 5, p. 1239-1246 8 p.

Research output: Contribution to journalReview article

6 Citations (Scopus)
2012

Modular recognition of nucleic acids by PUF, TALE and PPR proteins

Filipovska, A. & Rackham, O., 2012, In : Molecular Biosystems. 8, p. 699-708

Research output: Contribution to journalArticle

50 Citations (Scopus)
2014

Supernumerary proteins of mitochondrial ribosomes

Rackham, O. & Filipovska, A., Apr 2014, In : Biochimica et Biophysica Acta - General Subjects. 1840, 4, p. 1227-1232

Research output: Contribution to journalReview article

9 Citations (Scopus)
2018

An Artificial Yeast Genetic Circuit Enables Deep Mutational Scanning of an Antimicrobial Resistance Protein

Scott, L. H., Mathews, J. C., Flematti, G. R., Filipovska, A. & Rackham, O., 17 Aug 2018, In : ACS Synthetic Biology. 7, 8, p. 1907-1917 11 p.

Research output: Contribution to journalArticle

2 Citations (Scopus)
2019

Endoplasmic reticulum mediates mitochondrial transfer within the osteocyte dendritic network

Gao, J., Qin, A., Liu, D., Ruan, R., Wang, Q., Yuan, J., Cheng, T. S., Filipovska, A., Papadimitriou, J. M., Dai, K., Jiang, Q., Gao, X., Feng, J. Q., Takayanagi, H., Zhang, C. & Zheng, M. H., 20 Nov 2019, In : Science Advances. 5, 11, eaaw7215.

Research output: Contribution to journalArticle

Open Access
1 Citation (Scopus)
2020

Murine cytomegalovirus infection exacerbates complex IV deficiency in a model of mitochondrial disease

Ferreira, N., Andoniou, C. E., Perks, K. L., Ermer, J. A., Rudler, D. L., Rossetti, G., Periyakaruppiah, A., Wong, J. K. Y., Rackham, O., Noakes, P. G., Degli-Esposti, M. A. & Filipovska, A., Mar 2020, In : PLoS Genetics. 16, 3, e1008604.

Research output: Contribution to journalArticle

Open Access
2019

Modulation of miRNA function by natural and synthetic RNA-binding proteins in cancer

Vos, P. D., Leedman, P. J., Filipovska, A. & Rackham, O., Oct 2019, In : Cellular and Molecular Life Sciences. 76, 19, p. 3745–3752

Research output: Contribution to journalReview article

10 Citations (Scopus)
2018

Early-onset Parkinson disease caused by a mutation in CHCHD2 and mitochondrial dysfunction

Lee, R. G., Sedghi, M., Salari, M., Shearwood, A-M. J., Stentenbach, M., Kariminejad, A., Goullee, H., Rackham, O., Laing, N. G., Tajsharghi, H. & Filipovska, A., Oct 2018, In : Neurology: Genetics. 4, 5, 10 p., 276.

Research output: Contribution to journalArticle

Open Access
2016

Transcriptome-wide effects of a POLR3A gene mutation in patients with an unusual phenotype of striatal involvement

Azmanov, D. N., Siira, S. J., Chamova, T., Kaprelyan, A., Guergueltcheva, V., Shearwood, A-M. J., Liu, G., Morar, B., Rackham, O., Bynevelt, M., Grudkova, M., Kamenov, Z., Svechtarov, V., Tournev, I., Kalaydjieva, L. & Filipovska, A., 2016, In : Human Molecular Genetics. 25, 19, p. 4302-4314 13 p.

Research output: Contribution to journalArticle

15 Citations (Scopus)
2019

TEFM regulates both transcription elongation and RNA processing in mitochondria

Jiang, S., Koolmeister, C., Misic, J., Siira, S., Kühl, I., Silva Ramos, E., Miranda, M., Jiang, M., Posse, V., Lytovchenko, O., Atanassov, I., Schober, F. A., Wibom, R., Hultenby, K., Milenkovic, D., Gustafsson, C. M., Filipovska, A. & Larsson, N. G., 1 Jun 2019, In : EMBO Reports. 20, 6, 18 p., e48101.

Research output: Contribution to journalArticle

Open Access
6 Citations (Scopus)
2016

The Role of the L-Type Ca2+ Channel in Altered Metabolic Activity in a Murine Model of Hypertrophic Cardiomyopathy

Viola, H. M., Johnstone, V. P. A., Cserne Szappanos, H., Richman, T. R., Tsoutsman, T., Filipovska, A., Semsarian, C., Seidman, J. G. G., Seidman, C. E. E. & Hool, L. C., 2016, In : JACC: Basic to Translational Science. 1, 1-2, p. 61-72 12 p.

Research output: Contribution to journalArticle

Open Access
9 Citations (Scopus)
2017

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals

Kühl, I., Miranda, M., Atanassov, I., Kuznetsova, I., Hinze, Y., Mourier, A., Filipovska, A. & Larsson, N. G., 14 Nov 2017, In : eLife. 6, e30952.

Research output: Contribution to journalArticle

Open Access
41 Citations (Scopus)
2012

Structure of the heterodimer of human NONO and paraspeckle protein component 1 and analysis of its role in subnuclear body formation

Passon, D., Lee, M., Rackham, O., Stanley, W., Sadowska, A., Filipovska, A., Fox, A. & Bond, C., 27 Mar 2012, In : NATIONAL ACADEMY OF SCIENCES. PROCEEDINGS. 109, 13, p. 4846-4850

Research output: Contribution to journalArticle

Open Access
83 Citations (Scopus)

MRPS27 is a pentatricopeptide repeat domain protein required for the translation of mitochondrially encoded proteins

Davies, S., Lopez Sanchez, M. I., Narsai, R., Shearwood, A-M., Mohamad Razif, M. F., Small, I., Whelan, J., Rackham, O. & Filipovska, A., 19 Oct 2012, In : FEBS Letters. 586, p. 3555-3561

Research output: Contribution to journalArticle

Open Access
36 Citations (Scopus)
2014
Open Access
28 Citations (Scopus)
2017
Open Access
16 Citations (Scopus)
2016

POLRMT regulates the switch between replication primer formation and gene expression of mammalian mtDNA

Kühl, I., Miranda, M., Posse, V., Milenkovic, D., Mourier, A., Siira, S. J., Bonekamp, N. A. A., Neumann, U., Filipovska, A., Loguercio Polosa, P., Gustafsson, C. M. M. & Larsson, N-G. G., 2016, In : Science Advances. 2, 8, 14 p., e1600963.

Research output: Contribution to journalArticle

Open Access
32 Citations (Scopus)
2015

Mutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunction

Richman, T., Ermer, J., Davies, S., Perks, K., Viola, H., Shearwood, A-M., Hool, L., Rackham, O. & Filipovska, A., 27 Mar 2015, In : PLoS Genetics. 11, 3, p. e1005089 22 p., e1005089.

Research output: Contribution to journalArticle

Open Access
File
18 Citations (Scopus)
396 Downloads (Pure)
2013

Mapping of Mitochondrial RNA-Protein Interactions by Digital RNase Footprinting

Liu, G., Mercer, T. R., Shearwood, A-M., Siira, S., Hibbs, M., Mattick, J. S., Rackham, O. & Filipovska, A., 14 Nov 2013, In : Cell Reports. 5, 3, p. 839-848

Research output: Contribution to journalArticle

Open Access
File
23 Citations (Scopus)
331 Downloads (Pure)
2019

Circularized visualisation of genetic interactions

Kuznetsova, I., Lugmayr, A., Filipovska, A., Holzinger, A. & Rackham, O., 1 Jan 2019, Proceedings of the 26th International Conference on World Wide Web Companion. International World Wide Web Conferences Steering Committee, p. 225-226 2 p. (26th International World Wide Web Conference 2017, WWW 2017 Companion).

Research output: Chapter in Book/Conference paperConference paper

Open Access
2014

Synthetic biology with RNA

Filipovska, A. & Rackham, O., 2014, Synthetic Biology. Ryadnov, M., Brunsveld, L. & Suga, H. (eds.). United Kingdom: The Royal Society of Chemistry, Vol. 1. p. 359

Research output: Chapter in Book/Conference paperChapter

Analysis of the Human Mitochondrial Transcriptome Using Directional Deep Sequencing and Parallel Analysis of RNA Ends

Rackham, O. & Filipovska, A., 2014, Polyadenylation: Methods and Protocols. Rorbach, J. & Bobrowicz, A. J. (eds.). United States: Springer Science + Business Media, Vol. 1125. p. 263-275

Research output: Chapter in Book/Conference paperChapter

9 Citations (Scopus)
2013

Redox activation of Fe(III)–thiosemicarbazones and Fe(III)–bleomycin by thioredoxin reductase:specificity of enzymatic redox centers and analysis of reactive species formation by ESR spin trapping

Myers, J. M., Cheng, Q., Antholine, W. E., Kalyanaraman, B., Filipovska, A., Arnér, E. S. J. & Myers, C. R., 2013, In : Free Radical Biology and Medicine. 60, p. 183-194

Research output: Contribution to journalArticle

16 Citations (Scopus)
2019

Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome

Lake, N. J., Webb, B. D., Stroud, D. A., Richman, T. R., Ruzzenente, B., Compton, A. G., Mountford, H. S., Pulman, J., Zangarelli, C., Rio, M., Bodaert, N., Assouline, Z., Sherpa, M. D., Schadt, E. E., Houten, S. M., Byrnes, J., McCormick, E. M., Zolkipli-Cunningham, Z., Haude, K., Zhang, Z. & 11 others, Retterer, K., Bai, R., Calvo, S. E., Mootha, V. K., Christodoulou, J., Rotig, A., Filipovska, A., Cristian, I., Falk, M. J., Metodiev, M. D. & Thorburn, D. R., Jul 2019, In : European Journal of Human Genetics. 27, p. 860-861 2 p.

Research output: Contribution to journalAbstract/Meeting Abstract

2013

Specialization from synthesis: How ribosome diversity can customize protein function

Filipovska, A. & Rackham, O., 2013, In : FEBS Letters. 587, 8, p. 1189-1197

Research output: Contribution to journalArticle

Open Access
28 Citations (Scopus)

Pentatricopeptide repeats: Modular blocks for building RNA-binding proteins

Filipovska, A. & Rackham, O., 2013, In : RNA Biology. 10, 9, p. 1243-1249

Research output: Contribution to journalReview article

29 Citations (Scopus)
2014
6 Citations (Scopus)
2018
4 Citations (Scopus)