Dr Matthew McKenzie - Researcher Profile

Matthew McKenzie

Address

Monash Institute of Medical Research
Centre for Reproduction & Development

Contact Details

Tel: +61 3 990 24806

Email: Matthew.McKenzie@monash.edu


Biography

Matthew works in the Centre for Reproduction and Development, Monash Institute of Medical Research, as an ARC Future Fellow and Research Group Leader.

The central aim of Dr. McKenzie’s research is to define the pathogenic mechanisms which underlie mitochondrial disease. Following the completion of his PhD in 2002, Dr. McKenzie worked in the laboratory of Prof. Michael Duchen, University College London, where he investigated mitochondrial membrane potential, calcium handling and ATP generation in mitochondrial disorders. In 2004 Dr. McKenzie returned to La Trobe University, Melbourne, where he was supported by an NHMRC Peter Doherty Fellowship to undertake research on the biogenesis of mitochondrial oxidative phosphorylation (OXPHOS) complexes. In particular, his research focused on the assembly of mitochondrial respiratory chain complex I (NADH:ubiquinone oxidoreductase) and how inherited defects can disrupt this process.

In April 2011 Dr. McKenzie was recruited to Monash Institute of Medical Research as a Group Leader in the Centre for Reproduction and Development. His current research focuses on mitochondrial fatty acid b-oxidation (FAO) and how this metabolic pathway interacts with mitochondrial OXPHOS. He is currently supported by an NHMRC CDA Fellowship and project grants from the NHMRC, ARC and the James and Vera Lawson Trust.

In 2012 Dr. McKenzie was awarded an ARC Future Fellowship to continue his research on mitochondrial disease

Related Links:

Keywords

mitochondrial disease, fatty acid oxidation, , oxidative phosphorylation, protein complex assembly

Qualifications

PHD (DEPARTMENT OF MEDICINE)
Institution: University of Melbourne
Year awarded: 2003
SCIENCE (HONS)
Institution: University of Melbourne
Year awarded: 1996

Publications

Book Chapters

McKenzie, M., Lazarou, M., Ryan, M., 2009, Analysis of respiratory chain complex assembly with radiolabeled nuclear- and mitochondrial-encoded subunits, in Methods in Enzymology: Mitochondrial Function, Part A, eds Abelson, John N. & Simon, Melvin I., Academic Press, United States, pp. 321-339.

Journal Articles

Kelly, R.D.W., Mahmud, A., McKenzie, M., Trounce, I., St John, J.C., 2012, Mitochondrial DNA copy number is regulated in a tissue specific manner by DNA methylation of the nuclear-encoded DNA polymerase gamma A, Nucleic Acids Research [P], vol 40, issue 20, Oxford University Press, United Kingdom, pp. 10124-10138.

Tucker, E., Mimaki, M., Crompton, A., McKenzie, M., Ryan, M., Thorburn, D., 2012, Next-generation sequencing in molecular diagnosis: NUBPL mutations highlight the challenges of variant detection and interpretation, Human Mutation [P], vol 33, issue 2, John Wiley & Sons, Inc., United States, pp. 411-418.

Leong, D., Komen, J.C., Hewitt, C.A., Arnaud, E., McKenzie, M., Phipson, B., Bahlo, M., Laskowski, A., Kinkel, S.A., Davey, G.M., Heath, W.R., Voss, A.K., Zahedi, R.P., Pitt, J., Chrast, R., Sickmann, A., Ryan, M.T., Smyth, G.K., Thorburn, D.R., Scott, H.S., 2012, Proteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) gene, Journal Of Biological Chemistry [P], vol 287, issue 24, American Society for Biochemistry and Molecular Biology, Inc., United States, pp. 20652-20663.

Mimaki, M., Wang, X., McKenzie, M., Thorburn, D., Ryan, M., 2012, Understanding mitochondrial complex I assembly in health and disease, Biochimica et Biophysica Acta [P], vol 1817, issue 6, Elsevier BV, Netherlands, pp. 851-862.

Tucker, E., Hershman, S., Kohrer, C., Belcher-Timme, C., Patel, J., Goldberger, O., Christodoulou, J., Silberstein, J., McKenzie, M., Ryan, M., Compton, A., Jaffe, J., Carr, S., Calvo, S., RajBhandary, U., Thorburn, D., Mootha, V., 2011, Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation, Cell Metabolism [P], vol 14, issue 3, Cell Press, United States, pp. 428-434.

McKenzie, M., Tucker, E., Compton, A., Lazarou, M., George, C., Thorburn, D., Ryan, M., 2011, Mutations in the gene encoding C8orf38 block complex I assembly by inhibiting production of the mitochondria-encoded subunit ND1, Journal of Molecular Biology [P], vol 414, issue 3, Academic Press, United Kingdom, pp. 413-426.

McKenzie, M., Ryan, M., 2010, Assembly factors of human mitochondrial complex I and their defects in disease, Iubmb Life [P], vol 62, issue 7, Wiley-Blackwell Publishing Ltd., United Kingdom, pp. 497-502.

Riley, L., Cooper, S., Hickey, P., Rudinger-Thirion, J., McKenzie, M., Compton, A., Lim, S., Thorburn, D., Ryan, M., Giege, R., Bahlo, M., Christodoulou, J., 2010, Mutation of the mitochondrial tyrosyl-tRNA synthetase gene, YARS2, causes myopathy, lactic acidosis, and sideroblastic anemia - MLASA syndrome, American Journal Of Human Genetics [P], vol 87, issue 1, Cell Press, United States, pp. 52-59.

Lazarou, M., Thorburn, D., Ryan, M., McKenzie, M., 2009, Assembly of mitochondrial complex I and defects in disease, Biochimica et Biophysica Acta [P], vol 1793, issue 1, Elsevier BV, Netherlands, pp. 78-88.

Lazarou, M., Smith, S., Thorburn, D., Ryan, M., McKenzie, M., 2009, Assembly of nuclear DNA-encoded subunits into mitochondrial complex IV, and their preferential integration into supercomplex forms in patient mitochondria, Febs Journal [P], vol 276, issue 22, Wiley-Blackwell Publishing Ltd., United Kingdom, pp. 6701-6713.

Gebert, N., Joshi, A., Kutik, S., Becker, T., McKenzie, M., Guan, X., Mooga, V., Stroud, D., Kulkarni, G., Wenk, M., Rehling, P., Meisinger, C., Ryan, M., Wiedemann, N., Greenburg, M., Pfanner, N., 2009, Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome, Current Biology [P], vol 19, issue 24, Cell Press, United Kingdom, pp. 2133-2139.

Sugiana, C., Pagliarini, D., McKenzie, M., Kirby, D., Salemi, R., Abu-Amero, K., Dahl, H., Hutchison, W., Vascotto, K., Smith, S., Newbold, R., Christodoulou, J., Calvo, S., Mootha, V., Ryan, M., Thorburn, D., 2008, Mutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease, American Journal Of Human Genetics [P], vol 83, issue 4, Cell Press, United States, pp. 468-478.

McKenzie, M., Lazarou, M., Thorburn, D., Ryan, M., 2007, Analysis of mitochondrial subunit assembly into respiratory chain complexes using Blue Native polyacrylamide gel electrophoresis, Analytical Biochemistry [P], vol 364, issue 2, Academic Press, United States, pp. 128-137.

Lazarou, M., McKenzie, M., Ohtake, A., Thorburn, D., Ryan, M., 2007, Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex I, Molecular and Cellular Biology [P], vol 27, issue 12, American Society for Microbiology, United States, pp. 4228-4237.

Dunning, C., McKenzie, M., Sugiana, C., Lazarou, M., Silke, J., Connelly, A., Fletcher, J., Kirby, D., Thorburn, D., Ryan, M., 2007, Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease, Embo Journal [P], vol 26, issue 13, Nature Publishing Group, United Kingdom, pp. 3227-3237.

McKenzie, M., Liolitsa, D., Akinshina, N., Campanella, M., Sisodiya, S., Hargreaves, I., Nirmalananthan, N., Sweeney, M., Abou-Sleiman, P., Wood, N., Hanna, M., Duchen, M., 2007, Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption, Journal Of Biological Chemistry [P], vol 282, issue 51, American Association for Biochemistry and Molecular Biology, United States, pp. 36845-36852.

McKenzie, M., Lazarou, M., Thorburn, D., Ryan, M., 2006, Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients, Journal of Molecular Biology [P], vol 361, issue 3, Academic Press, United Kingdom, pp. 462-469.

Trounce, I., McKenzie, M., Cassar, C., Ingraham, C., Lerner, C., Dunn, D., Donegan, C., Takeda, K., Pogozelski, W., Howell, R., Pinkert, C., 2004, Development and initial characterization of xenomitochondrial mice, Journal Of Bioenergetics And Biomembranes [P], vol 36, issue 4, Springer New York LLC, United States, pp. 421-427.

McKenzie, M., Liolitsa, D., Hanna, M., 2004, Mitochondrial disease: mutations and mechanisms, Neurochemical Research [P], vol 29, issue 3, Springer New York LLC, United States, pp. 589-600.

McKenzie, M., Trounce, I., Cassar, C., Pinkert, C., 2004, Production of homoplasmic xenomitochondrial mice, Proceedings Of The National Academy Of Sciences Of The United States Of America [P], vol 101, issue 6, National Academy of Sciences, United States, pp. 1685-1690.

McKenzie, M., Chiotis, M., Pinkert, C., Trounce, I., 2003, Functional respiratory chain analyses in murid xenomitochondrial cybrids expose coevolutionary constraints of cytochrome b and nuclear subunits of complex III, Molecular Biology And Evolution [P], vol 20, issue 7, Oxford University Press, United States, pp. 1117-1124.

Babon, J., McKenzie, M., Cotton, R., 2003, The use of resolvases T4 endonuclease VII and T7 endonuclease I in mutation detection, Applied Biochemistry and Biotechnology - Part B Molecular Biotechnology [P], vol 23, issue 1, Humana Press, United States, pp. 73-81.

Activities

Assessments of applications for research

From: 06/03/2010 To: 08/03/2010
The Netherlands Organization for Scientific Research (NWO)
Project Grant Reviewer

From: 08/06/2011 To: 29/05/2012
NHMRC
External Grant Reviewer

From: 12/08/2006 To: 14/08/2006
Singapore National Medical Research Council.
Project Grant Reviewer

From: 12/08/2007 To: 14/08/2007
Singapore National Medical Research Council.
Project Grant Reviewer

From: 17/05/2007 To: 19/05/2007
Barth Syndrome Foundation, USA.
Project Grant Reviewer

Editing special issues of journals

From: 30/11/2012 To: 31/05/2013
Biochim Biophys Acta - General Subjects
‘Frontiers of Mitochondrial Biology'

Invitations to meetings, seminars, plenary sessions, or exhibitions

From: 02/12/2010 To: 03/12/2010
AussieMit Conference
Oral presentation selected from abstracts
Sydney, Australia

From: 07/06/2010 To: 07/06/2010
Monash Institute of Medical Research
Invited Seminar

From: 09/07/1998 To: 09/07/1998
kConFab Conference
Guest Speaker
Melbourne, Australia.

From: 10/02/2008 To: 14/02/2008
Lorne Conference on Protein Structure and Function
Junior Investigator Awardee, oral presentation
Lorne, Australia.

From: 10/12/2001 To: 10/12/2001
University of Rochester Medical Center, Rochester, USA.
Invited Seminar

From: 11/07/2010 To: 16/07/2010
Gordon Conference, Mitochondria and Chloroplasts
Oral presentation selected from abstracts
Barga, Italy.

From: 12/07/2000 To: 12/07/2000
University of Newcastle upon Tyne, UK.
Invited Seminar

From: 19/09/2001 To: 23/09/2001
European Meeting on Mitochondrial Pathology (Euromit)
Oral presentation selected from abstracts
Euromit V, Venice, Italy.

From: 29/06/2010 To: 29/06/2010
Saitama University, Tokyo, Japan.
Invited Seminar

Manuscript refereeing

From: 01/01/0013 To: 30/09/2012
Multiple Journals
18 reviews since 2006

Organised major conferences outside the Faculty

From: 10/12/2012 To: 12/12/2012
AussieMit Conference
Treasurer and Organizing Committee Member

From: 17/05/2012 To: 17/05/2012
Monash Health Translation Precinct
Organizing Committee Chair, Symposium on Reproduction and Development

From: 27/11/2008 To: 28/11/2008
AussieMit Conference
Co-founder and Secretary of Organizing Committee

From: 29/09/2010 To: 29/09/2010
OzBio 2010 Conference
Co-Chair of Colloquia Session on Molecular Basis of Disease

Professional organisation memberships

From: 01/01/0007 To: 31/12/2012
United Mitochondrial Disease Foundation
Member

From: 01/01/0008 To: 31/12/0012
Australian Society for Biochemistry and Molecular Biology
Member

Research - internally funded

From: 01/01/2013 To: 31/12/2014
Monash University
Monash Research Accelerator Program

Visiting professorships, fellowships, or other appointments

From: 01/01/2006 To: 31/12/2008
LA TROBE UNIVERSITY
NHMRC Peter Doherty Fellowship

From: 01/01/2009 To: 31/03/2011
LA TROBE UNIVERSITY
NHMRC CDA Fellowship

From: 01/01/2013 To: 31/12/2016
Monash University
ARC Future Fellowship

From: 01/04/2011 To: 31/12/2012
Monash Institute of Medical Research
NHMRC CDA Fellowship

Awards

Academic awards (national, international)

From: 05/07/0009 To: 10/07/0009
FASEB Conference
Poster prize, 1st place

From: 10/02/0008 To: 14/02/0008
Lorne Conference on Protein Structure and Function
Junior Investigator Award

From: 24/08/0002 To: 27/08/0002
St Vincents Hospital Melbourne
Junior Investigator Award, St. Vincent's Hospital Research Week

Grants

Title:
Generating new induced Pluripotent Stem (iPS) Cells models of human Metabolic Disease.
Investigators:
McKenzie, M
Funding:
(2013 - 2016). Philanthropy, ANZ Trustees.
Title:
New models of mitochondrial fatty acid oxidation disorders.
Investigators:
McKenzie, M
Funding:
(2012 - 2016). Australian Research Council (ARC).
Title:
Assembly of mitochondria respiratory chain complexes and their defects associated with disease.
Investigators:
McKenzie, M
Funding:
(2011 - 2015). National Health & Medical Research Council (NHMRC).