My research focusses on developing new diagnostic tools for investigating inborn errors of metabolism using clinical applications of mass spectrometry, investigating pathophysiology of metabolic diseases and studying immunometabolism in patients with inborn errors of metabolism.
A simple method modification to increase separation of 2- and 3-hydroxyglutaric acid by GC–MS for clinical urine organic acids analysis Clinical Biochemistry Dubland, J.A. and Sinclair, G. and Vallance, H. and Raki?, B. DOI: 10.1016/j.clinbiochem.2022.04.016 2022
Diagnostic yield from routine metabolic screening tests in evaluation of global developmental delay and intellectual disability Paediatrics and Child Health (Canada) Vallance, H. and Sinclair, G. and Rakic, B. and Stockler-Ipsiroglu, S. DOI: 10.1093/pch/pxaa112 2021
Molecular diagnosis of pompe disease in the genomic era: Correlation with acid alpha-glucosidase activity in dried blood spots Journal of Clinical Medicine Thuriot, F. and Gravel, E. and Hodson, K. and Ganopolsky, J. and Rakic, B. and Waters, P.J. and Gravel, S. and Lévesque, S. DOI: 10.3390/jcm10173868 2021
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex I deficiency Molecular Genetics and Metabolism Reports Apatean, D. and Rakic, B. and Brunel-Guitton, C. and Hendson, G. and Bai, R. and Sargent, M.A. and Lavoie, P.M. and Patel, M. and Stockler-Ipsiroglu, S. DOI: 10.1016/j.ymgmr.2018.12.006 2019
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency Molecular Genetics and Metabolism Reports Apatean, D. and Rakic, B. and Brunel-Guitton, C. and Hendson, G. and Bai, R. and Sargent, M.A. and Lavoie, P.M. and Patel, M. and Stockler-Ipsiroglu, S. DOI: 10.1016/j.ymgmr.2019.100472 2019
Gene Expression Profiling of Endoplasmatic Reticulum Stress in Hepatitis C Virus-Containing Cells Treated with an Inhibitor of Protein Disulfide Isomerases ACS Omega Özcelik, D. and Seto, A. and Rakic, B. and Farzam, A. and Supek, F. and Pezacki, J.P. DOI: 10.1021/acsomega.8b02676 2018
Sialic acid catabolism by N-acetylneuraminate pyruvate lyase is essential for muscle function JCI insight Wen, X.-Y. and Tarailo-Graovac, M. and Brand-Arzamendi, K. and Willems, A. and Rakic, B. and Huijben, K. and Da Silva, A. and Pan, X. and El-Rass, S. and Ng, R. and Selby, K. and Philip, A.M. and Yun, J. and Ye, X.C. and Ross, C.J. and Lehman, A.M. and Zijlstra, F. and Abu Bakar, N. and Drögemöller, B. and Moreland, J. and Wasserman, W.W. and Vallance, H. and van Scherpenzeel, M. and Karbassi, F. and Hoskings, M. and Engelke, U. and de Brouwer, A. and Wevers, R.A. and Pshezhetsky, A.V. and van Karnebeek, C.D. and Lefeber, D.J. DOI: 10.1172/jci.insight.122373 2018
Consensus recommendations for the diagnosis, treatment and follow-up of inherited methylation disorders Journal of Inherited Metabolic Disease Bari?, I. and Staufner, C. and Augoustides-Savvopoulou, P. and Chien, Y.-H. and Dobbelaere, D. and Grünert, S.C. and Opladen, T. and Petkovi? Ramad?a, D. and Raki?, B. and Wedell, A. and Blom, H.J. DOI: 10.1007/s10545-016-9972-7 2017
Exome sequencing and the management of neurometabolic disorders New England Journal of Medicine Tarailo-Graovac, M. and Shyr, C. and Ross, C.J. and Horvath, G.A. and Salvarinova, R. and Ye, X.C. and Zhang, L.-H. and Bhavsar, A.P. and Lee, J.J.Y. and Drögemöller, B.I. and Abdelsayed, M. and Alfadhel, M. and Armstrong, L. and Baumgartner, M.R. and Burda, P. and Connolly, M.B. and Cameron, J. and Demos, M. and Dewan, T. and Dionne, J. and Evans, A.M. and Friedman, J.M. and Garber, I. and Lewis, S. and Ling, J. and Mandal, R. and Mattman, A. and McKinnon, M. and Michoulas, A. and Metzger, D. and Ogunbayo, O.A. and Rakic, B. and Rozmus, J. and Ruben, P. and Sayson, B. and Santra, S. and Schultz, K.R. and Selby, K. and Shekel, P. and Sirrs, S. and Skrypnyk, C. and Superti-Furga, A. and Turvey, S.E. and Van Allen, M.I. and Wishart, D. and Wu, J. and Wu, J. and Zafeiriou, D. and Kluijtmans, L. and Wevers, R.A. and Eydoux, P. and Lehman, A.M. and Vallance, H. and Stockler-Ipsiroglu, S. and Sinclair, G. and Wasserman, W.W. and Van Karnebeek, C.D. DOI: 10.1056/NEJMoa1515792 2016
Structure-based mutagenic analysis of mechanism and substrate specificity in mammalian glycosyltransferases: Porcine ST3Gal-I Glycobiology Raki?, B. and Rao, F.V. and Freimann, K. and Wakarchuk, W. and Strynadka, N.C.J. and Withers, S.G. DOI: 10.1093/glycob/cwt001 2013
Structure and mechanism of the lipooligosaccharide sialyltransferase from Neisseria meningitidis Journal of Biological Chemistry Lin, L.Y.-C. and Rakic, B. and Chiu, C.P.C. and Lameignere, E. and Wakarchuk, W.W. and Withers, S.G. and Strynadka, N.C.J. DOI: 10.1074/jbc.M111.249920 2011
Structural and kinetic characterizations of the polysialic acid O-acetyltransferase OatWY from Neisseria meningitidis Journal of Biological Chemistry Lee, H.J. and Rakic, B. and Gilbert, M. and Wakarchuk, W.W. and Withers, S.G. and Strynadka, N.C.J. DOI: 10.1074/jbc.M109.006049 2009
Benzofuran-derived cyclic ß-amino acid scaffold for building a diverse set of flavonoid-like probes and the discovery of a cell motility inhibitor Organic Letters Nandy, J.P. and Rakic, B. and Sarma, B.V.N.B. and Babu, N. and Lefrance, M. and Enright, G.D. and Leek, D.M. and Daniel, K. and Sabourin, L.A. and Arya, P. DOI: 10.1021/ol800050g 2008
Synthesis and bioorthogonal coupling chemistry of a novel cyclopentenone-containing unnatural tyrosine analogue Bioorganic Chemistry Lorello, G.R. and Legault, M.C.B. and Raki?, B. and Bisgaard, K. and Pezacki, J.P. DOI: 10.1016/j.bioorg.2007.12.006 2008
A Small-Molecule Probe for Hepatitis C Virus Replication that Blocks Protein Folding Chemistry and Biology Rakic, B. and Clarke, J. and Tremblay, T.-L. and Taylor, J. and Schreiber, K. and Nelson, K.M. and Abrams, S.R. and Pezacki, J.P. DOI: 10.1016/j.chembiol.2006.08.010 2006
Peroxisome proliferator-activated receptor a antagonism inhibits hepatitis C virus replication Chemistry and Biology Rakic, B. and Sagan, S.M. and Noestheden, M. and Bélanger, S. and Nan, X. and Evans, C.L. and Xie, X.S. and Pezacki, J.P. DOI: 10.1016/j.chembiol.2005.10.006 2006
Bleomycin is a potent small-molecule inhibitor of hepatitis C virus replication ChemBioChem Raki?, B. and Brûlotte, M. and Rouleau, Y. and Bélanger, S. and Pezacki, J.P. DOI: 10.1002/cbic.200600180 2006
Synthesis of (-)-cytoxazone and (+)-epi-cytoxazone: The chiral pool approach Synthetic Communications Tokic-Vujosevic, Z. and Petrovic, G. and Rakic, B. and Matovic, R. and Saicic, R.N. DOI: 10.1081/SCC-200048953 2005
Synthesis of (2)-Cytoxazone and (1)-epi-Cytoxazone: the chiral pool approach Synthetic Communications 2005
Clinical Research Development Award (CRSU), 2019. “Next generation urine metabolic profiling for the diagnosis and monitoring of Inborn Errors of Metabolism.” (PI) - Bojana Rakic, PhD FCCMG. CO-PIs - Graham Sinclair, PhD FCCMG, Joshua Dubland, PhD, Ramona Salvarinova-Zivkovic, MD MHSc FRCPC FCCMG PhD
Childhood Diseases Team Grant, 2018. “T-cell function, susceptibility to infection, and a fatty acid oxidation variant common to coastal BC First Nations.” (PI) - Bojana Rakic, PhD FCCMG. CO-PI - Graham Sinclair, PhD FCCMG
Rare Diseases - Models and Mechanisms Network. N-Acetylneuraminate pyruvate lyase (NPL) deficiency Functional validation in mouse, $20,000. Coinvestigators - Dr. Clara van Karnebeek and Dr. Alexey Pshezhetsky
Rare Disease Foundation and BC Children’s Hospital Foundation (BCCHF). GBA2 validation, $3500. Coinvestigator - Dr. Gabriella Horvath
Honours & Awards
2009-2012 - NSERC Postdoctoral Fellowship
2005 - Pfizer Award for Outstanding Achievement, Bio-Organic Gordon Research Conference
2014 - Linda Stevens Memorial Fund Garrod Conference Travel Award 2014
A pan-Canadian team has developed a new way to quickly find personalized treatments for young cancer patients, by growing their tumours in chicken eggs and analyzing their proteins.
The team, led by researchers from BC Children’s Hospital Research Institute and the University of British Columbia, is the first in Canada to combine these two techniques to identify and test a drug for a young patient's tumour in time for their treatment.
Congratulations to the investigators and their teams who were awarded funding through the Canadian Institutes of Health Research (CIHR) Fall 2024 Project Grant competition!
The findings from a BCCHR study played a part in integrating the multiple breath washout test into recent recommendations to screen children for pulmonary chronic graft-versus-host disease, a lung complication that can affect children who undergo a bone marrow transplant.
New research published in Nature Communications, identifies a key molecule that might be critical for the development of stem cell-based therapies for treating type 1 diabetes.
Dr. Kirk Schultz, an investigator at BC Children’s Hospital Research Institute, has been awarded the 2024 Geoffrey L. Hammond Lectureship in recognition of his leadership and significant contributions to improving the health and well-being of children in British Columbia and around the world.
We believe there’s nothing we can’t do with your support. It can take years to turn scientific breakthrough into new interventions and treatments. Funding helps speed the pace of change. When given the resources, we can bring transformative therapies – and hope – out of the laboratory and into the clinic to save and improve children’s lives.