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Enhancing the lives and outcomes
of Canadian individuals and families
affected by Barth syndrome."

Grants Awarded 

 

Since the 2007 season, the Barth Syndrome Foundation of Canada (BSFCa) has actively participated in the Barth Syndrome Foundation (BSF) Research Grant program. 

Following is a list of grants funded or partially funded by BSFCa.  For a complete list of grants awarded by BSF and affiliates please follow this link.

2017 

Richard Epand, PhD, Professor, McMaster University, Hamilton, Ontario, Canada

      The cause and consequences of plasmalogen depletion in Barth syndrome 

This research is currently on-going

*Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada

 


2016 

Christina Pacak, PhD, Assistant Professor, University of Florida, Gainesville, FL

       Optimization of AAV-mediated gene therapy for Barth syndrome

This research is currently on-going 

      *Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada 

 


2015 

Robin Duncan, Assistant Professor, University of Waterloo, Waterloo, Ontario

This research is currently on-going

*Funding for this award was provided by the Barth Syndrome Foundation and the Barth Syndrome Foundation of Canada 


 

2014

Valerian Kagan, PhD, Professor and Vice-Chairman, University of Pittsburgh, Pittsburgh, PA

Mechanism and role of cardiolipin oxidation and hydrolysis in Barth syndrome


Associated publications to date:

Kagan VE, Bayir H, Tyurina YY, Bolevich SB, Maguire JJ, Fadeel B, Balasubramanian K. Elimination of the unnecessary:  Intra- and extracellular signaling by anionic phospholipids. Review Article. Biochemical and Biophysical Research Communications, Volume 482, issue 3, 15 January 2017, Pages 482-490. (PubMed Abstract)* 

Kooijman EE, Swim LA, Graber ZT, Tyurina YY, Bayir H, Kagan VE. Magic angle spinning 31P NMR spectroscopy reveals two essentially identical ionization states for the cardiolipin phosphates in phospholipid liposomes. Biochim Biophys Acta. 2016 Oct 26. pii:  S0005-2736(16)30346-7. doi:10.1016/j.bbamem.2016.10.013. [Epub ahead of print] (PubMed Abstract)* 

Maguire JJ, Tyurina YY, Mohammadyani D, Kapralov AA, Anthonymuthu TS, Qu F, Amoscato AA, Sparvero LJ, Tyurin VA, Planas-Iglesias J, He RR, Klein-Seetharaman J, Bayir H, Kagan VE. Known unknowns of cardiolipin signaling:  The best is yet to come. Biochim Biophys Acta. 2016 Aug 4. pii:  S1388-1981(16)30221-9. doi:  10.1016/j.bbalip.2016.08.001. [Epub ahead of print] (PubMed Abstract)* 


  *Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada


 

2013

Grant Hatch, PhD, Professor, University of Manitoba, Winnipeg, Manitoba

 

 

Associated publications to date:

Mejia EM, Zinko JC, Hauff KD, Xu Fy, Ravandi A, Hatch GM. Glucose uptake and triacylglycerol synthesis are increased in Barth syndrome lymphoblasts. Lipids. 2017 Jan 17. doi:  10.1007/s11745-017-4232-7. [Epub ahead of print] (PubMed Abstract)* 

 

 

*Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada


 

2012

Richard Epand, PhD, Professor, McMaster University, Hamilton, Ontario

Associated Publications to Date:

Xu Y, Phoon CK, Berno B, D'Souza K, Hoedt E, Zhang G, Neubert TA, Epand RM, Ren M, Schlame M. Loss of protein association casuses cardiolipin degradation in Barth syndrome. Nat Chem Biol. 2016 Jun 27. doi:  10.1038/nchembio.2113. [Epub ahead of print] (PubMed Abstract) 

 

 
*Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada

 

2011

Grant Hatch, PhD, Professor, University of Manitoba, Winnipeg, Manitoba

 

Associated Publications To Date:

 Mejia EM, Nguyen H, Hatch GM. Mammalian cardiolipin biosynthesis. Chem Phys Lipids. 2013 Oct 18 [Epub ahead of print](PubMed Abstract)

 

Associated Presentations To Date:

Linking cardiolipin remodeling to mitochondrial beta-oxidation 

 Biological Function of Tafazzin and Cardiolipin ~ Brainstorming About Future Directions



2010

Christopher McMaster, PhD, Professor, Dalhousie University, Halifax, Nova Scotia

 

A screen for drugs, leads for the treatment of Barth syndrome

 

Associated Publications to Date:

Gaspard GJ, McMaster CR. Cardiolipin metabolism and its causal role in the etiology of the inherited cardiomyopathy Barth syndrome.  Chem Phys Lipids. 2015 Sep 25. pii:  S0009-3084(15)30054-2. doi:10.1016/j.chemphyslip.2018.09.005.[Epub ahead of print] Review. (PubMed Abstract)*

 

Gaspard GJ, McMaster CR. The mitochondrial quality control protein Yme1 is necessary to prevent defective mitophagy in a yeast model of Barth syndrome. J Biol Chem. 2015 Feb 16. pii:jbc.M115.41878.[Epub ahead of print] (PubMed - Open Access)*

 

Associated Presentations To Date:

Yeast genome-wide screens to assess the genetic landscape for Barth syndrome (PDF Presentation)    Audio File


2009

Grant Hatch, PhD, Professor, University of Manitoba, Winnipeg, Manitoba

Role of human monolysocardiolipin acyltransferase in Barth syndrome

Associated Publications To Date:

Taylor WA, Mejia EM, Mitchell RW, Choy PC, Sparagna GC, et al. Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidation. PLoS ONE 7(11): e48628.(PubMed ~ OpenAccess)

Saini-Chohan HK, Mitchell RW, Vaz FM, Zelinski T, Hatch GM. Delineating the role of alterations in lipid metabolism to the pathogenesis of inherited skeletal and cardiac muscle disorders. J Lipid Res. 2012 Jan;53(1):4-27. doi: 10.1194/jlr.R012120. Epub 2011 Nov 7.(PubMed ~ OpenAccess)

Saini-Chohan HK, Dakshinamurti S, Taylor WA, Shen GX, Murphy R, Sparagna GC, Hatch GM. Persistent pulmonary hypertension results in reduced tetralinoleoyl-cardiolipin and Complex II + III activity in neonatal pig heart. Am J Physiol Heart Circ Physiol. 2011 Oct;301(4):H1415-24. doi: 10.1152/ajpheart.00247.2011. Epub 2011 Aug 12.(PubMed ~ OpenAccess)

 


2008

Miriam Greenberg, PhD, Professor and Associate Dean, Wayne State University, Detroit, Michigan

The role of tafazzin in mitochondrial protein import – Implications for Barth syndrome

Associated Publications To Date:

Tyurina YY, Lou W, Qu F, tyurin VA, Mohammadyani D, Liu J, Hüttemann M, Frasso MA, Wipf P, Bayer H, Greenberg ML, Kagan VE. Lipidomics characterization of biosynthetic and remodeling pathways of cardiolipins in genetically and nutritionally manipulated yeast cells. ACS Chem Biol. 2016 Dec 16. [Epub ahead of print] (PubMed - OpenAccess)* 

Raja V, Joshi AS, Li G, Maddipati KR, Greenberg ML. Loss of cardiolipin leads to perturbation of acetyl-CoA synthesis. J Biol Chem. 2016 Dec 9. pii:  jbc.M116.753624. [Epub ahead of print] (PubMed Abstract)* 

Chen S, Liu D, Finley RL Jr, Greenberg ML. Loss of mitochondrial DNA in the yeast cardiolipin synthase crd1 mutant leads to up-regulation of the protein kinase Swe1p that regulates the G2/M transition. J Biol Chem. 2010 Apr 2;285(14):10397-407. doi: 10.1074/jbc.M110.100784. Epub 2010 Jan 19. (PubMed ~ OpenAccess)

Gebert N, Joshi AS, Kutik S, Becker T, McKenzie M, Guan XL, Mooga VP, Stroud DA, Kulkarni G, Wenk MR, Rehling P, Meisinger C, Ryan MT, Wiedemann N, Greenberg ML, Pfanner N. Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome. Curr Biol. 2009 Dec 29;19(24):2133-9. Epub 2009 Dec 3. (PubMed Abstract)

 

*Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada

2007

Richard Epand, PhD, Professor, McMaster University, Hamilton, Ontario

Consequences of the alteration of cardiolipin structure on the properties of the mitochondrial membranes

Associated Publications To Date:

Schlame M, Acehan D, Berno B, Xu Y, Valvo S, Ren M, Stokes DL, Epand RM. The physical state of lipid substrates provides transacylation specificity for tafazzinNat Chem Biol. 2012 Oct;8(10):862-9.(PubMed Abstract)

Schlattner U, Tokarska-Schlattner M, Ramirez S, Brückner A, Kay L, Polge C, Epand RF, Lee RM, Lacombe ML, Epand RM. Mitochondrial kinases and their molecular interaction with cardiolipin. Biochim Biophys Acta. 2009 Oct;1788(10):2032-47. Epub 2009 May 4. Review.  (PubMed Abstract)

Associated Presentations To Date:

U.Schlattner, M.Tokarska-Schlattner, S.Ramirez, A.Bruckner, L.Kay, C.Polge, R.F.Epand, R.M.Lee, M.L.Lacombe, and R.M.Epand, Mitochondrial kinases and their molecular interaction with cardiolipin. Biochim Biophys Acta 1788 (2009) 2032-2047.

T. H. Haines, R. F. Epand and R. M. Epand. The pK´s of cardiolipins are altered by the number and position of their specific fatty acid esters. Biophysical Society – 52th Annual Meeting, Long Beach, CA (February, 2008).

 

And

 

Christopher R. McMaster, PhD, Assistant Professor, Dalhousie University, Halifax, Nova Scotia

Synthetic genetics toward understanding Barth syndrome cell biology

Associated Publications to Date:

Gaspard GJ, McMaster CR. Cardiolipin metabolism and its causal role in the etiology of the inherited cardiomyopathy Barth syndrome.  Chem Phys Lipids. 2015 Sep 25. pii:  S0009-3084(15)30054-2. doi:10.1016/j.chemphyslip.2018.09.005.[Epub ahead of print] Review. (PubMed Abstract)* 

Gaspard GJ, McMaster CR. The mitochondrial quality control protein Yme1 is necessary to prevent defective mitophagy in a yeast model of Barth syndrome. J Biol Chem. 2015 Feb 16. pii:jbc.M115.41878.[Epub ahead of print] (PubMed - Open Access)* 

*Funding for this award was provided by the Barth Syndrome Foundation, and the Barth Syndrome Foundation of Canada 

 


2006

Grant M. Hatch, PhD, Professor, Pharmacology and Therapeutics, Acting Associate Dean of Medicine for Research 

University of Manitoba,  Winnipeg, Manitoba

Cholesterol Metabolism in Barth syndrome

Associated Publications To Date:

Hauff K, Hatch GM. Reduction in cholesterol synthesis in response to serum starvation in lymphoblasts of a patient with Barth syndrome. Biochem Cell Biol. 2010 Aug;88(4):595-602. (PubMed Abstract)

Hauff KD, Choi SY, Frohman MA, Hatch GM. Cardiolipin synthesis is required to support human cholesterol biosynthesis from palmitate upon serum removal in Hela cells. Can J Physiol Pharmacol. 2009 Oct;87(10):813-20. (PubMed Abstract)

Hauff KD, Hatch, GM. Cardiolipin metabolism and Barth Syndrome.Prog Lipid Res. 2006 Mar;45(2):91-101. Epub 2006 Jan 18.  (PubMed Abstract)


 



 

 


 

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