Wellcome Trust Centre For Mitochondrial Research

Francesco Bruni

Biography

 

I am a molecular biologist and my expertise lies in the field of mitochondrial gene expression. Currently, I am working as a Scientific Officer in the Wellcome Trust Centre for Mitochondrial Research. My responsibilities include having personal research projects but also acting as the liaison between PIs/executive administrators and the scientists and technicians who make up the Mitochondrial Research division.

The aims of the research project are to characterize the function of candidate proteins in the mitochondrial translation process and to determine the role of the pathogenic mutations underlying mitochondrial protein synthesis defects in available patient cell lines.

Research Project

Understanding critical factors and pathogenic defects underlying mitochondrial gene expression

Principal Investigators: Professor R.N. Lightowlers, Professor Z.M.A Chrzanowska-Lightowlers and Professor R. Taylor

Project Details

Currently, I am investigating the ways in which mitochondria can exert a level of ‘quality control’ between mitochondrial transcription and translation processes and the protein factors that are likely to be responsible for this activity. Within the Wellcome Trust Centre for Mitochondrial Research the link between pure and clinical research is very strong, providing the opportunity to study the mitochondrial translation mechanisms in controls versus disease.

Email: francesco.bruni@ncl.ac.uk

Sponsor/Funder: Wellcome Trust

Publications

Bruni F, Lightowlers RN, Chrzanowska-Lightowlers ZM. Human mitochondrial nucleases. The FEBS Journal 2016. In Press.

Boczonadi V, Meyer K, Kaspar B, Bartsakoulia M, Bansagi B, Bruni F, Boycott K, Mueller J, Horvath R. Mutations in glycyl-tRNA-synthetase impair mitochondrial function in neurons. In: Journal of the Peripheral Nervous System. 2016

Besse A, Wu P, Bruni F, Donti T, Graham BH, Craigen WJ, McFarland R, Moretti P, Lalani S, Scott KL, Taylor RW, Bonnen PE. The GABA Transaminase, ABAT, Is Essential for Mitochondrial Nucleoside Metabolism. Cell Metabolism 2015, 21(3), 417-427.

Besse A, Wu P, Bruni F, Donti T, Graham BH, Craigen WJ, McFarland R, Lalani S, Scott KL, Taylor RW, Bonnen PE. ABAT is a novel human mitochondrial DNA depletion syndrome gene linking gamma-aminobutyric acid (GABA) catabolism and mitochondrial nucleoside metabolism. In: Molecular Genetics and Metabolism 2015

Wilson WC, Hornig-Do HT, Bruni F, Chang JC, Jourdain AA, Martinou JC, Falkenberg M, Spåhr H, Larsson NG, Lewis RJ, Hewitt L, Baslé A, Cross HE, Tong L, Lebel RR, Crosby AH, Chrzanowska-Lightowlers ZMA, Lightowlers RN. A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression. Human Molecular Genetics 2014, 23(23), 6345-6355.

Yarham JW, Lamichhane TN, Pyle A, Mattijssen S, Baruffini E, Bruni F, Donnini C, Vassilev A, He L, Blakely EL, Griffin H, Santibanez-Koref M, Bindoff LA, Ferrero I, Chinnery PF, McFarland R, Maraia RJ, Taylor RW. Defective i6A37 Modification of Mitochondrial and Cytosolic tRNAs Results from Pathogenic Mutations in TRIT1 and its Substrate tRNA. PLoS Genetics 2014, 10(6), e1004424.

Bruni F, Gramegna P, Oliveira JM, Lightowlers RN, Chrzanowska-Lightowlers ZMA. REXO2 is an oligoribonuclease active in human mitochondria. PLoS One 2013, 8(5), e64670.

Fernández-Moreno MA, Hernández R, Adán C, Roberti M, Bruni F, Loguercio-Polosa P, Cantatore P, Matsushima Y, Kaguni LS, Garesse R. Drosophila nuclear factor DREF regulates the expression of the mitochondrial DNA helicase and mitochondrial transcription factor B2 but not the mitochondrial translation factor B1. Biochimica et Biophysica Acta: Gene Regulatory Mechanisms 2013, 1829(10), 1136-1146.

Bruni F, Manzari C, Filice M, Loguercio Polosa P, Colella M, Carmone C, Hambardjieva E, Garcia-Diaz M, Cantatore P, Roberti M. D-MTERF5 is a novel factor modulating transcription in Drosophila mitochondria. Mitochondrion 2012, 12(5), 492-499.

Bruni F, Gramegna P, Lightowlers RN, Chrzanowska-Lightowlers ZMA. The mystery of mitochondrial RNases. Biochemical Society Transactions 2012, 40(4), 865-869.

Roberti M, Loguerciopolosa P, Bruni F, Manzari C, Filice M, Cantatore P. Characterization of an MTERF family protein acting in Drosophila mitochondrial transcription. In: 36th FEBS Congress: Biochemistry for Tomorrow’s Medicine. 2011, Torino, Italy.

Bruni F, Lightowlers RN. Designing an Organellar Postal Service: Delivery of Macromolecules to Mitochondria in Intact Cells. Molecular Therapy 2011, 19(8), 1404-1405.

BRUNI F, LOGUERCIOPOLOSA P, GADALETA MN, CANTATORE P, ROBERTI M. Human mitochondrial transcription and replication proteins are regulated via differential pathways. 2010, FEBS Journal.

Roberti M, LoguercioPolosa P, Bruni F, Deceglie S, Gadaleta MN, Cantatore P. MTERF factors: a multifunction protein family. BioMolecular Concepts 2010, 1(2), 215-224.

Bruni F, Loguercio Polosa P, Gadaleta MN, Cantatore P, Roberti M. Nuclear Respiratory Factor 2 Induces the Expression of Many but Not All Human Proteins Acting in Mitochondrial DNA Transcription and Replication. Journal of Biological Chemistry 2010, 285(6), 3939-3948.

Fernández-Moreno MA, Bruni F, Adán C, HernándezSierra R, LoguercioPolosa P, Cantatore P, Garesse R, Roberti M. The Drosophila nuclear factor DREF positively regulates the expression of the mitochondrial transcription termination factor DmTTF. Biochemical Journal 2009, 418, 453-462.

Roberti M, LoguercioPolosa P, Bruni F, Manzari c, Deceglie S, Gadaleta MN, Cantatore P. The MTERF family proteins: Mitochondrial transcription regulators and beyond. Biochimica et Biophysica Acta. Bioenergetics 2009, 1787(5), 303-311.

Bruni F, FernandezMoreno MA, LoguercioPolosa P, Garesse R, Cantatore P, Gadaleta MN, Roberti M. Transcriptional regulation of MTERF family genes in humans and Drosophila. 2009, FEBS Journal.

Roberti M, Bruni F, Loguercio Polosa P, Manzari C, Gadaleta MN, Cantatore P. MTERF3, the most conserved member of the mTERF-family, is a modular factor involved in mitochondrial protein synthesis. Biochimica et Biophysica Acta 2006, 1757(9-10), 1199-1206.

Roberti M, Bruni F, Loguercio Polosa P, Gadaleta MN, Cantatore P. The Drosophila termination factor DmTTF regulates in vivo mitochondrial transcription. Nucleic Acids Research 2006, 34(7), 2109-2116.

Roberti M, Fernandez-Silva P, Loguercio Polosa P, Fernandez-Vizarra E, Bruni F, Deceglie S, Montoya J, Gadaleta MN, Cantatore P. In vitro transcription termination activity of the Drosophila mitochondrial DNA-binding protein DmTTF. Biochemical and Biophysical Research Communications 2005, 331(1), 357-362.

Roberti M, Loguercio Polosa P, Bruni F, Musicco C, Gadaleta MN, Cantatore P. DmTTF, a novel mitochondrial transcription termination factor that recognises two sequences of Drosophila melanogaster mitochondrial DNA. Nucleic Acids Research 2003, 31(6), 1597-1604.

MUSICCO C, ROBERTI M, BRUNI F, LOGUERCIOPOLOSA P, GADALETA MN, CANTATORE P. IDENTIFICATION AND CHARACTERIZATION OF DmTTF, THE DROSOPHILA MELANOGASTER HOMOLOG OF THE SEA URCHIN MITOCHONDRIAL TRANSCRIPTION TERMINATION FACTOR mtDBP. 2003, Protein Science.