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Items: 1 to 50 of 86

1.

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

Lee RG, Sedghi M, Salari M, Shearwood AJ, Stentenbach M, Kariminejad A, Goullee H, Rackham O, Laing NG, Tajsharghi H, Filipovska A.

Neurol Genet. 2018 Oct 5;4(5):e276. doi: 10.1212/NXG.0000000000000276. eCollection 2018 Oct.

PMID:
30338296
2.

Is mitochondrial gene expression coordinated or stochastic?

Lee RG, Rudler DL, Rackham O, Filipovska A.

Biochem Soc Trans. 2018 Oct 8. pii: BST20180174. doi: 10.1042/BST20180174. [Epub ahead of print] Review.

PMID:
30301847
3.

Concerted regulation of mitochondrial and nuclear non-coding RNAs by a dual-targeted RNase Z.

Siira SJ, Rossetti G, Richman TR, Perks K, Ermer JA, Kuznetsova I, Hughes L, Shearwood AJ, Viola HM, Hool LC, Rackham O, Filipovska A.

EMBO Rep. 2018 Oct;19(10). pii: e46198. doi: 10.15252/embr.201846198. Epub 2018 Aug 20.

PMID:
30126926
4.

Unique features of mammalian mitochondrial translation initiation revealed by cryo-EM.

Kummer E, Leibundgut M, Rackham O, Lee RG, Boehringer D, Filipovska A, Ban N.

Nature. 2018 Aug;560(7717):263-267. doi: 10.1038/s41586-018-0373-y. Epub 2018 Aug 8.

PMID:
30089917
5.

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

Scott LH, Mathews JC, Flematti GR, Filipovska A, Rackham O.

ACS Synth Biol. 2018 Aug 17;7(8):1907-1917. doi: 10.1021/acssynbio.8b00121. Epub 2018 Jul 17.

PMID:
29979580
6.

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

Spåhr H, Chia T, Lingford JP, Siira SJ, Cohen SB, Filipovska A, Rackham O.

Nat Commun. 2018 Jun 7;9(1):2212. doi: 10.1038/s41467-018-04388-1.

7.

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

Wallis CP, Richman TR, Filipovska A, Rackham O.

ACS Chem Biol. 2018 Jun 15;13(6):1499-1505. doi: 10.1021/acschembio.8b00424. Epub 2018 May 29.

PMID:
29808990
8.

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

Wallis CP, Filipovska A, Rackham O.

Biotechnol Lett. 2018 Jul;40(7):1127-1134. doi: 10.1007/s10529-018-2567-7. Epub 2018 May 18.

PMID:
29777514
9.

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

Lake NJ, Webb BD, Stroud DA, Richman TR, Ruzzenente B, Compton AG, Mountford HS, Pulman J, Zangarelli C, Rio M, Boddaert N, Assouline Z, Sherpa MD, Schadt EE, Houten SM, Byrnes J, McCormick EM, Zolkipli-Cunningham Z, Haude K, Zhang Z, Retterer K, Bai R, Calvo SE, Mootha VK, Christodoulou J, Rötig A, Filipovska A, Cristian I, Falk MJ, Metodiev MD, Thorburn DR.

Am J Hum Genet. 2018 Apr 5;102(4):713. doi: 10.1016/j.ajhg.2018.03.015. No abstract available.

10.

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

Perks KL, Rossetti G, Kuznetsova I, Hughes LA, Ermer JA, Ferreira N, Busch JD, Rudler DL, Spahr H, Schöndorf T, Shearwood AJ, Viola HM, Siira SJ, Hool LC, Milenkovic D, Larsson NG, Rackham O, Filipovska A.

Cell Rep. 2018 Apr 3;23(1):127-142. doi: 10.1016/j.celrep.2018.03.033.

11.

Expression patterns of regulatory RNAs, including lncRNAs and tRNAs, during postnatal growth of normal and dystrophic (mdx) mouse muscles, and their response to taurine treatment.

Butchart LC, Terrill JR, Rossetti G, White R, Filipovska A, Grounds MD.

Int J Biochem Cell Biol. 2018 Jun;99:52-63. doi: 10.1016/j.biocel.2018.03.016. Epub 2018 Mar 22.

PMID:
29578051
12.

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

Matic S, Jiang M, Nicholls TJ, Uhler JP, Dirksen-Schwanenland C, Polosa PL, Simard ML, Li X, Atanassov I, Rackham O, Filipovska A, Stewart JB, Falkenberg M, Larsson NG, Milenkovic D.

Nat Commun. 2018 Mar 23;9(1):1202. doi: 10.1038/s41467-018-03552-x.

13.

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

Saunders BM, Rudnicka C, Filipovska A, Davies S, Ward N, Hricova J, Schlaich MP, Matthews VB.

Immunol Cell Biol. 2018 Jan;96(1):41-53. doi: 10.1111/imcb.1002. Epub 2017 Nov 23.

PMID:
29359470
14.

LRPPRC-mediated folding of the mitochondrial transcriptome.

Siira SJ, Spåhr H, Shearwood AJ, Ruzzenente B, Larsson NG, Rackham O, Filipovska A.

Nat Commun. 2017 Nov 16;8(1):1532. doi: 10.1038/s41467-017-01221-z.

15.

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 NG.

Elife. 2017 Nov 14;6. pii: e30952. doi: 10.7554/eLife.30952.

16.

The First Mitochondrial Genomics and Evolution SMBE-Satellite Meeting: A New Scientific Symbiosis.

Ostersetzer-Biran O, Lane N, Pomiankowski A, Burton R, Arnqvist G, Filipovska A, Huchon D, Mishmar D.

Genome Biol Evol. 2017 Nov 1;9(11):3054-3058. doi: 10.1093/gbe/evx227.

17.

Regulation of a minimal transcriptome by repeat domain proteins.

Ferreira N, Rackham O, Filipovska A.

Semin Cell Dev Biol. 2018 Apr;76:132-141. doi: 10.1016/j.semcdb.2017.08.037. Epub 2017 Aug 24. Review.

PMID:
28843979
18.

Adult-onset obesity is triggered by impaired mitochondrial gene expression.

Perks KL, Ferreira N, Richman TR, Ermer JA, Kuznetsova I, Shearwood AJ, Lee RG, Viola HM, Johnstone VPA, Matthews V, Hool LC, Rackham O, Filipovska A.

Sci Adv. 2017 Aug 16;3(8):e1700677. doi: 10.1126/sciadv.1700677. eCollection 2017 Aug.

19.

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

Lake NJ, Webb BD, Stroud DA, Richman TR, Ruzzenente B, Compton AG, Mountford HS, Pulman J, Zangarelli C, Rio M, Boddaert N, Assouline Z, Sherpa MD, Schadt EE, Houten SM, Byrnes J, McCormick EM, Zolkipli-Cunningham Z, Haude K, Zhang Z, Retterer K, Bai R, Calvo SE, Mootha VK, Christodoulou J, Rötig A, Filipovska A, Cristian I, Falk MJ, Metodiev MD, Thorburn DR.

Am J Hum Genet. 2017 Aug 3;101(2):239-254. doi: 10.1016/j.ajhg.2017.07.005. Erratum in: Am J Hum Genet. 2018 Apr 5;102(4):713.

20.

Simultaneous processing and degradation of mitochondrial RNAs revealed by circularized RNA sequencing.

Kuznetsova I, Siira SJ, Shearwood AJ, Ermer JA, Filipovska A, Rackham O.

Nucleic Acids Res. 2017 May 19;45(9):5487-5500. doi: 10.1093/nar/gkx104.

21.

Defects in RNA metabolism in mitochondrial disease.

Siira SJ, Shearwood AJ, Bracken CP, Rackham O, Filipovska A.

Int J Biochem Cell Biol. 2017 Apr;85:106-113. doi: 10.1016/j.biocel.2017.02.003. Epub 2017 Feb 9.

PMID:
28189843
22.

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 SJ, Bonekamp NA, Neumann U, Filipovska A, Polosa PL, Gustafsson CM, Larsson NG.

Sci Adv. 2016 Aug 5;2(8):e1600963. doi: 10.1126/sciadv.1600963. eCollection 2016 Aug.

23.

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

Azmanov DN, Siira SJ, Chamova T, Kaprelyan A, Guergueltcheva V, Shearwood AJ, Liu G, Morar B, Rackham O, Bynevelt M, Grudkova M, Kamenov Z, Svechtarov V, Tournev I, Kalaydjieva L, Filipovska A.

Hum Mol Genet. 2016 Oct 1;25(19):4302-4314. doi: 10.1093/hmg/ddw263. Epub 2016 Aug 9.

PMID:
27506977
24.

Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly.

Rackham O, Busch JD, Matic S, Siira SJ, Kuznetsova I, Atanassov I, Ermer JA, Shearwood AM, Richman TR, Stewart JB, Mourier A, Milenkovic D, Larsson NG, Filipovska A.

Cell Rep. 2016 Aug 16;16(7):1874-90. doi: 10.1016/j.celrep.2016.07.031. Epub 2016 Aug 4.

25.

Dinuclear Au(i) N-heterocyclic carbene complexes derived from unsymmetrical azolium cyclophane salts: potential probes for live cell imaging applications.

Wedlock LE, Barnard PJ, Filipovska A, Skelton BW, Berners-Price SJ, Baker MV.

Dalton Trans. 2016 Jul 26;45(30):12221-36. doi: 10.1039/c6dt01409g.

PMID:
27426282
26.

Recessive Mutations in TRMT10C Cause Defects in Mitochondrial RNA Processing and Multiple Respiratory Chain Deficiencies.

Metodiev MD, Thompson K, Alston CL, Morris AA, He L, Assouline Z, Rio M, Bahi-Buisson N, Pyle A, Griffin H, Siira S, Filipovska A, Munnich A, Chinnery PF, McFarland R, Rötig A, Taylor RW.

Am J Hum Genet. 2016 Jul 7;99(1):246. doi: 10.1016/j.ajhg.2016.06.013. No abstract available.

27.

Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice.

Richman TR, Spåhr H, Ermer JA, Davies SM, Viola HM, Bates KA, Papadimitriou J, Hool LC, Rodger J, Larsson NG, Rackham O, Filipovska A.

Nat Commun. 2016 Jun 20;7:11884. doi: 10.1038/ncomms11884.

28.

Recessive Mutations in TRMT10C Cause Defects in Mitochondrial RNA Processing and Multiple Respiratory Chain Deficiencies.

Metodiev MD, Thompson K, Alston CL, Morris AAM, He L, Assouline Z, Rio M, Bahi-Buisson N, Pyle A, Griffin H, Siira S, Filipovska A, Munnich A, Chinnery PF, McFarland R, Rötig A, Taylor RW.

Am J Hum Genet. 2016 May 5;98(5):993-1000. doi: 10.1016/j.ajhg.2016.03.010. Epub 2016 Apr 28.

29.

The L-type Ca(2+) channel facilitates abnormal metabolic activity in the cTnI-G203S mouse model of hypertrophic cardiomyopathy.

Viola H, Johnstone V, Cserne Szappanos H, Richman T, Tsoutsman T, Filipovska A, Semsarian C, Hool L.

J Physiol. 2016 Jul 15;594(14):4051-70. doi: 10.1113/JP271681. Epub 2016 Jun 12.

30.

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

Viola HM, Johnstone VPA, Cserne Szappanos H, Richman TR, Tsoutsman T, Filipovska A, Semsarian C, Seidman JG, Seidman CE, Hool LC.

JACC Basic Transl Sci. 2016 Feb 13;1(1-2):61-72. doi: 10.1016/j.jacbts.2015.12.001. eCollection 2016 Jan-Feb. Review.

31.

A mutation in MT-TW causes a tRNA processing defect and reduced mitochondrial function in a family with Leigh syndrome.

Duff RM, Shearwood AM, Ermer J, Rossetti G, Gooding R, Richman TR, Balasubramaniam S, Thorburn DR, Rackham O, Lamont PJ, Filipovska A.

Mitochondrion. 2015 Nov;25:113-9. doi: 10.1016/j.mito.2015.10.008. Epub 2015 Oct 30.

PMID:
26524491
32.

SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation.

Lagouge M, Mourier A, Lee HJ, Spåhr H, Wai T, Kukat C, Silva Ramos E, Motori E, Busch JD, Siira S; German Mouse Clinic Consortium, Kremmer E, Filipovska A, Larsson NG.

PLoS Genet. 2015 Aug 6;11(8):e1005423. doi: 10.1371/journal.pgen.1005423. eCollection 2015 Aug.

33.

Mutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunction.

Richman TR, Ermer JA, Davies SM, Perks KL, Viola HM, Shearwood AM, Hool LC, Rackham O, Filipovska A.

PLoS Genet. 2015 Mar 27;11(3):e1005089. doi: 10.1371/journal.pgen.1005089. eCollection 2015 Mar.

34.

Age-related proteostasis and metabolic alterations in Caspase-2-deficient mice.

Wilson CH, Shalini S, Filipovska A, Richman TR, Davies S, Martin SD, McGee SL, Puccini J, Nikolic A, Dorstyn L, Kumar S.

Cell Death Dis. 2015 Jan 22;6:e1615. doi: 10.1038/cddis.2014.567.

35.

An artificial PPR scaffold for programmable RNA recognition.

Coquille S, Filipovska A, Chia T, Rajappa L, Lingford JP, Razif MF, Thore S, Rackham O.

Nat Commun. 2014 Dec 17;5:5729. doi: 10.1038/ncomms6729.

PMID:
25517350
36.

Estrogen-mediated regulation of mitochondrial gene expression.

Sanchez MI, Shearwood AM, Chia T, Davies SM, Rackham O, Filipovska A.

Mol Endocrinol. 2015 Jan;29(1):14-27. doi: 10.1210/me.2014-1077.

37.

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

Ivanov IS, Azmanov DN, Ivanova MB, Chamova T, Pacheva IH, Panova MV, Song S, Morar B, Yordanova RV, Galabova FK, Sotkova IG, Linev AJ, Bitchev S, Shearwood AM, Kancheva D, Gabrikova D, Karcagi V, Guergueltcheva V, Geneva IE, Bozhinova V, Stoyanova VK, Kremensky I, Jordanova A, Savov A, Horvath R, Brown MA, Tournev I, Filipovska A, Kalaydjieva L.

Mol Genet Metab. 2014 Sep-Oct;113(1-2):76-83. doi: 10.1016/j.ymgme.2014.07.017. Epub 2014 Jul 21.

38.

Impaired functional communication between the L-type calcium channel and mitochondria contributes to metabolic inhibition in the mdx heart.

Viola HM, Adams AM, Davies SM, Fletcher S, Filipovska A, Hool LC.

Proc Natl Acad Sci U S A. 2014 Jul 15;111(28):E2905-14. doi: 10.1073/pnas.1402544111. Epub 2014 Jun 26.

39.

Mitochondria: Unusual features of the mammalian mitoribosome.

Richman TR, Rackham O, Filipovska A.

Int J Biochem Cell Biol. 2014 Aug;53:115-20. doi: 10.1016/j.biocel.2014.05.011. Epub 2014 May 16.

PMID:
24842111
40.

A bifunctional protein regulates mitochondrial protein synthesis.

Richman TR, Davies SM, Shearwood AM, Ermer JA, Scott LH, Hibbs ME, Rackham O, Filipovska A.

Nucleic Acids Res. 2014 May;42(9):5483-94. doi: 10.1093/nar/gku179. Epub 2014 Mar 5.

41.

Analysis of the human mitochondrial transcriptome using directional deep sequencing and parallel analysis of RNA ends.

Rackham O, Filipovska A.

Methods Mol Biol. 2014;1125:263-75. doi: 10.1007/978-1-62703-971-0_21.

PMID:
24590795
42.

Mapping of mitochondrial RNA-protein interactions by digital RNase footprinting.

Liu G, Mercer TR, Shearwood AM, Siira SJ, Hibbs ME, Mattick JS, Rackham O, Filipovska A.

Cell Rep. 2013 Nov 14;5(3):839-48. doi: 10.1016/j.celrep.2013.09.036. Epub 2013 Oct 31.

43.

Supernumerary proteins of mitochondrial ribosomes.

Rackham O, Filipovska A.

Biochim Biophys Acta. 2014 Apr;1840(4):1227-32. doi: 10.1016/j.bbagen.2013.08.010. Epub 2013 Aug 17. Review.

PMID:
23958563
44.

Organelle transcriptomes: products of a deconstructed genome.

Small ID, Rackham O, Filipovska A.

Curr Opin Microbiol. 2013 Oct;16(5):652-8. doi: 10.1016/j.mib.2013.07.011. Epub 2013 Aug 7. Review.

PMID:
23932204
45.

Pentatricopeptide repeats: modular blocks for building RNA-binding proteins.

Filipovska A, Rackham O.

RNA Biol. 2013;10(9):1426-32. doi: 10.4161/rna.24769. Epub 2013 Apr 23.

46.

Specialization from synthesis: how ribosome diversity can customize protein function.

Filipovska A, Rackham O.

FEBS Lett. 2013 Apr 17;587(8):1189-97. doi: 10.1016/j.febslet.2013.02.032. Epub 2013 Feb 26. Review.

47.

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 JM, Cheng Q, Antholine WE, Kalyanaraman B, Filipovska A, Arnér ES, Myers CR.

Free Radic Biol Med. 2013 Jul;60:183-94. doi: 10.1016/j.freeradbiomed.2013.02.016. Epub 2013 Feb 26.

48.

L-type Ca(2+) channel contributes to alterations in mitochondrial calcium handling in the mdx ventricular myocyte.

Viola HM, Davies SM, Filipovska A, Hool LC.

Am J Physiol Heart Circ Physiol. 2013 Mar 15;304(6):H767-75. doi: 10.1152/ajpheart.00700.2012. Epub 2013 Jan 18.

49.

Engineered rRNA enhances the efficiency of selenocysteine incorporation during translation.

Thyer R, Filipovska A, Rackham O.

J Am Chem Soc. 2013 Jan 9;135(1):2-5. doi: 10.1021/ja3069177. Epub 2012 Dec 27.

PMID:
23256865
50.

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

Davies SM, Lopez Sanchez MI, Narsai R, Shearwood AM, Razif MF, Small ID, Whelan J, Rackham O, Filipovska A.

FEBS Lett. 2012 Oct 19;586(20):3555-61. doi: 10.1016/j.febslet.2012.07.043. Epub 2012 Jul 25.

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