Team

Photo of Dr. Fon's team


PRINCIPAL INVESTIGATOR

Photo of Edward FonEdward Fon, MD, FRCP (C)

ted.fon [at] mcgill.ca

Dr. Edward Fon, MD, FRCP(C) is a neurologist-scientist at the Montreal Neurological Institute-Hospital (The Neuro) specializing in movement disorders and a Professor in the Department of Neurology and Neurosurgery at McGill University. He is the Director of the FRQS Quebec Parkinson Network and the Scientific Director of The Neuro. He was trained at the Université de Montréal, McGill University and the University of California, San Francisco (UCSF). His research focuses on the molecular and cellular events leading to Parkinson's disease (PD). His laboratory has made contributions to understanding the function and cell biology of PD genes and in understanding how defects in these genes lead to PD. In particular, his lab has focused on understanding the function of Parkin, PINK1, α-synuclein, GBA, LRRK2, TMEM175 and DJ1, and has established the infrastructure at The Neuro to study these genes and pathways in human patient-derived induced pluripotent stem cells (iPSCs), which are differentiated into neurons, glia and 3D brain organoids. The lab is currently using these iPSC systems to establish a pipeline to better understand the role of lesser-studied PD genes, identified in recent genome-wide association studies (GWAS), in PD pathogenesis. In addition, Dr. Fon helped establish the Tanenbaum Open Science Institute (TOSI) at The Neuro and oversees its platforms including the Clinical Biological Imaging and Genetic repository (C-BIGr) and iPSC/CRISPR Early Drug Discovery Unit (EDDU).

PDF icon Curriculum Vitae

PDF icon List of publications   -  NCBI bibliography

 


POSTDOCTORAL FELLOWS

Photo of Thomas GoiranThomas Goiran, PhD

thomas.goiran [at] mail.mcgill.ca

My research focuses on the effect induced by toxic accumulation of protein aggregates essentially composed of the protein Alpha-Synuclein. These aggregates are tought to be involved in neuronal decay in the brains of patients with Parkinson's disease, leading to loss of cognition and death. We do not know the genetic actors contributing to this neuronal toxicity. In order to identify and validate new target genes for the development of new therapeutic treatments, I combine the increased growth and division ability of cultured cell lines and the physiological relevance of a dopaminergic neuron model generated from induced pluripotent stem cells from healthy individuals and Parkinson's patients. The goal of my project is to reduce or prevent this toxicity and excessive neuronal death characteristic of Parkinson's disease.

List of publications

Roberts R., Bayne AN.,  Goiran T., Lévesque D., Boisvert FM., Trempe JF., Fon EA., (2021). Proteomic Profiling of Mitochondrial-Derived Vesicles in Brain Reveals Enrichment of Respiratory Complex Sub-assemblies and Small TIM Chaperones. J Proteome Res ; 20(1):506-517.

Checler F, Goiran T, Alves da Costa C. (2018). Nuclear TP53: An unraveled function as transcriptional repressor of PINK1. Autophagy ; 14(6):1099-1101.

McLelland GL, Goiran T, Yi W, Dorval G, Chen CX, Lauinger ND, Krahn AI, Valimehr S, Rakovic A, Rouiller I, Durcan TM, Trempe JF, Fon EA. (2018). Mfn2 ubiquitination by PINK1/parkin gates the p97-dependent release of ER from mitochondria to drive mitophagy. Elife ; pii: e32866

Goiran T, Duplan E, Rouland L, El Manaa W, Lauritzen I, Dunys J, You H, Checler F, Alves da Costa C. (2018). Nuclear p53-mediated repression of autophagy involves PINK1 transcriptional down-regulation. Cell Death and Differentiation ; 25(5):873-884

Goiran T, Duplan E, Chami M, Bourgeois A, El Manaa W, Rouland L, Dunys J, Lauritzen I, You H, Stambolic V, Biféri MG, Barkats M, Pimplikar SW, Sergeant N, Colin M, Morais VA, Pardossi-Piquard R, Checler F, Alves da Costa C. (2018). β-Amyloid Precursor Protein Intracellular Domain Controls Mitochondrial Function by Modulating Phosphatase and Tensin Homolog-Induced Kinase 1 Transcription in Cells and in Alzheimer Mice Models. Biological Psychiatry ; 1;83(5):416-427

Checler F, Goiran T, Alves da Costa C. (2017). Presenilins at the crossroad of a functional interplay between PARK2/PARKIN and PINK1 to control mitophagy: Implication for neurodegenerative diseases. Autophagy ; 13(11):2004-2005

CLoubière C, Goiran T, Laurent K, Djabari Z, Tanti JF, Bost F. (2015). Metformin-induced energy deficiency leads to the inhibition of lipogenesis in prostate cancer cells. Oncotarget ; 20;6(17):15652-61

Viotti J, Duplan E, Caillava C, Condat J, Goiran T, Giordano C, Marie Y, Idbaih A, Delattre JY, Honnorat J, Checler F, Alves da Costa C. (2014). Glioma tumor grade correlates with parkin depletion in mutant p53- linked tumors and results from loss of function of p53 transcriptional activity. Oncogene ; 3;33(14):1764-75

Duplan E, Sevalle J, Viotti J, Goiran T, Bauer C, Renbaum P, Levy-Lahad E, Gautier CA, Corti O, Leroudier N, Checler F, da Costa CA. (2013). Parkin differently regulates presenilin-1 and presenilin-2 functions by direct control of their promoter transcription. Journal of Molecular Cell Biology ; Apr;5(2):132-42

 

 

Photo of Mohamed EldeebMohamed Eldeeb, PhD

mohamed.eldeeb [at] mcgill.ca

My research aims at deciphering some of the molecular and structural basis of mitochondrial-quality control pathways in mammalian cells. Specifically, I am interested in understanding the molecular dialogue between protein quality-control and mitochondrial import machinery. In addition, I am interested in delineating the molecular basis of protein degradation roles in cellular demise in the context of PD.

List of publications

Eldeeb MA, Fahlman RP, Mohamed Ragheb, Mansoore Esmili and Mohamed Salla. (2019) Monitoring protein degradation in Nerve cells. JNC. Under review.

Eldeeb MA, Fahlman RP, Mohamed Ragheb and Mansoore Esmaili. (2019) Does N-terminal acetylation lead to protein degradation? BioEssays. In revision.

Eldeeb MA, Fahlman RP, Mansoore Esmaili and Edward Fon. (2019) Formylation of Eukaryotic Cytoplasmic Proteins: Linking Stress to Degradation. Trends Biochem. Sci. doi.org/10.1016/j.tibs.2018.12.008.

Eldeeb MA, Fahlman RP, Mansoore Esmaili and Mohamed Ragheb. (2018) Regulating apoptosis by degradation: The N-end rule-mediated regulation of apoptotic proteolytic fragments. International Journal of Molecular Sciences. 19, 3414.

Eldeeb MA, Fahlman RP. (2018) Does Too Much MAGIC Lead to Mitophagy? Trends in biochemical Sciences. P485-487, 2018.

Eldeeb MA, Ragheb, M.A. (2018) Post-translational N-terminal arginylation of protein fragments: a pivotal portal to proteolysis. Curr. Protein Pept. Sci. 19, 1214-1223

Eldeeb MA, Luana Leitao, Richard Fahlman. (2017) Emerging branches of the N-end rule pathways are revealing the sequence complexities of N-termini dependent protein degradation. Biochem. Cell. Biol. 10.1139/bcb-2017-0274.

Hou J, Eldeeb MA, Wang X. (2017) Beyond deubiquitylation: USP30-mediated regulation of mitochondrial homeostasis. AIMS Molecular Science. 4:219-223.

Eldeeb MA. (2017) Aging: when the ubiquitin–proteasome machinery collapses. Adv. Exp. Med. Biol. 1038:133-148.

Kramer DA, Eldeeb MA, Wuest M, Mercer J, Fahlman RP. (2017) Proteomic characterization of EL4 lymphoma derived tumors upon chemotherapy treatment reveals potential roles for lysozomes and caspase6 during tumor cell death in vivo. Proteomics. 17, 12-17.

AlMatarM., Eldeeb MA, Makky, E.A. et al. (2017) Are there any other compounds isolated from Dermacoccus spp at all? Curr. Microbiol. 74:132-144.

Eldeeb MA, Fahlman RP. (2016) The N-end rule: The beginning determines the end.Protein and Peptide Letters.  23:343-348.

Eldeeb MA, Fahlman RP. (2016) Phosphorylation impacts then-end rule degradation of the proteolytically activated form of BMX kinase. J. Biol. Chem.  291:22757-22768.

Eldeeb MA, Fahlman RP. (2014) The anti-apoptotic form of tyrosine kinase Lyn that is generated by proteolysis is degraded by the N-end rule pathway. Oncotarget. 5:2714–22.

 

Photo of Nguyen-Vi MohamedNguyen-Vi Mohamed, PhD

nguyen-vi.mohamed [at] mail.mcgill.ca

Investigating the pathological mechanisms leading to the propagation of alpha-synuclein protein in human midbrain organoids derived from Parkinson’s disease patients’ iPSCs.

 

 

 

 

List of publications

Mohamed N.V, Larroquette F., et al. (2019) One step into the future: new iPSC tools to advance research in Parkinson’s disease and neurological disorders. Journal of Parkinson’s disease doi: 10.3233/JPD-181515

Mohamed N.V, Desjardins A, Leclerc N (2017) Tau secretion is correlated to an increase of Golgi dynamics. PLoS One. 12(5): e0178288. doi: 10.1371/journal.pone.0178288. 

Rodriguez L, Mohamed N.V, Desjardins A, Lippé R, Fon E, Leclerc N (2016) Rab7A regulates tau secretion. J Neurochem. doi: 10.1111/jnc.13994.

Aulas A, Caron G, Gkogkas CG, Mohamed NV, Destroismaisons L, Sonenberg N, Leclerc N, Parker JA, Vande Velde C. (2015) G3BP1 promotes stress-induced RNA granule interactions to preserve polyadenylated mRNA. J Cell Biol. 209(1):73-84. doi: 10.1083/jcb.201408092

Mohamed N.V, et al. (2013) Starvation and inhibition of lysosomal function increase tau secretion. Sci Rep. 4: 5715. doi: 10.1038/srep05715

Mohamed N.V, et al. (2013) Spreading of tau pathology in Alzheimer disease by cell-to-cell transmission. Eur J Neurosci. 37(12):1939-48. doi: 10.1111/ejn.12229.

Mohamed N. V, Plouffe, V., et al. (2012) Hyperphosphorylation and cleavage at D421 enhance tau secretion. PLoS One 7(5): e36873.

 

Photo of Rhalena ThomasRhalena Thomas, PhD

rhalena.thomas [at] mcgill.ca

I develop analysis tools for induced pluripotent stem cells derived neurons and 3D tissue models ("mini-brains"). My goal is to integrate transcriptional data from single cell sequencing and cellular imaging data to understand the pathological processes involved in Parkinson's disease.

List of publications

Del Cid-Pellitero E, Tavassoly O, Larroquette F, Cai E, Thomas RA, Soubannier V, Luo W, Durcan TM, Fon EA. (2021) Pharmacological Inhibition of Brain EGFR Activation By a BBB-penetrating Inhibitor, AZD3759, Attenuates α-synuclein Pathology in a Mouse Model of α-Synuclein Propagation. Neurotherapeutics. 

Vogel JW, La Joie R, Grothe MJ, Diaz-Papkovich A, Doyle A, Vachon-Presseau E, Lepage C, de Wael RV, Thomas RA, Iturria-Medina Y, Bernhardt B. (2020) A molecular gradient along the longitudinal axis of the human hippocampus informs large-scale behavioral systems. Nature communications 11(1):1-7

Thomas RA, Gibon J, De Leon, A, Chen, CXQ, Chierzi, S, Baulac, S, Murai, KK, Barker PA. (2018) The Nogo Receptor Ligand LGI1 Regulates Synapse Number and Synaptic Activity in Hippocampal and Cortical Neurons. eNeuro 0185-18

Thomas RA, Metzen MG and Chacron MJ. (2018) Weakly electric fish distinguish between envelope stimuli arising from different behavioral contexts. Journal of experimental biology 221: jeb178244

Gibon J, Unsain N, Gamache K, Thomas RA, De Leon A, Johnstone A, Nader K, Sequela P, Barker PA. (2016) The X-linked inhibitor of apoptosis regulates long-term depression and learning rate. FASEB J. 30(9):3083

Thomas RA, Ambalavana A, Rouleau GA, Barker PA. (2016) Identification of genetic variants of LGI1 and RTN4R (NgR1) linked to schizophrenia that are defective in NgR1–LGI1 signaling. Molecular Genetics and Genomic Medicine. 4(4): 447–456

Kommaddi R, Thomas R, Ceni C, Daigneault K, Barker PA. (2011) Trk-dependent ADAM17 activation facilitates neurotrophin survival signalling. FASEB J. 10-173740

Ceni C, Kommaddi R, Thomas R, Vereker E, McPherson PM, Ritter B, Liu X, Barker PA. (2010) The p75NTR intracellular domain is generated by neurotrophin-induced receptor cleavage to potentiate Trk signalling. Journal of Cell Science 123(13):2299-307

Thomas R, Favell K, Morante-Redolat JM, Pool, M, Kent C, Wright M, Daignault K, Ferraro G, Montcalm S, Durocher Y, Fournier A, Perez-Tur J, Barker PA. (2010) LGI1 is a Novel Nogo Receptor 1 ligand that antagonizes myelin-based growth inhibition. Journal of Neuroscience 30(19):6607-12

 

Empty portraitYogitha Thattikota, PhD

yogitha.thattikota [at] mcgill.ca

 

 

 

 

 

List of publications

Thattikota Y., Tollis S., Palou R., Vinet J., Tyers M., D'Amours D. (2018) Cdc48/VCP Promotes Chromosome Morphogenesis by Releasing Condensin from Self-Entrapment in Chromatin. Mol. Cell. 69, 664-676 e665.

Chatterjee G., Sankaranarayanan S.R., Guin K., Thattikota Y., Padmanabhan S., Siddharthan R., Sanyal K. (2016) Repeat-Associated Fission Yeast-Like Regional Centromeres in the Ascomycetous Budding Yeast Candida tropicalis. PLoS Genet. 12, e1005839.

Kanshin E., Kubiniok P., Thattikota Y., D'Amours D., Thibault P.(2015) Phosphoproteome dynamics of Saccharomyces cerevisiae under heat shock and cold stress. Mol. Syst. Biol. 11, 813

Robellet X*., Thattikota Y*., Wang F., Wee T.L., Pascariu M., Shankar S., Bonneil E., Brown C.M., D'Amours D.(2015) A high-sensitivity phospho-switch triggered by Cdk1 governs chromosome morphogenesis during cell division. Genes Dev. 29, 426-439. *equal contribution

 

Photo of Jace Jones-TabahJace Jones-Tabah, PhD

jace.jones-tabah [at] mail.mcgill.ca

 My research in the Fon lab is focused on the investigation of genetic risk factors for Parkinson’s disease that alter lysosome function. Mutations in the gene GBA, which encodes the lysosomal enzyme glucocerebrosidase, are among the most common genetic risk factors for Parkinson’s disease. However, recent genetic studies have revealed that mutations in several additional lysosomal proteins also increase the risk of developing the disease. In my research I use patient-derived stem cell models and CRISPR-Cas9 genome editing to study the mechanisms by which these disruptions in lysosomal function contribute to the loss of dopaminergic neurons in Parkinson’s disease, with the goal of identifying novel targets for Parkinson’s disease treatment.

List of publications

Jones-Tabah, J., Martin, R. D., Chen, J. J., Tanny, J. C., Clarke, P. B. S., and Hébert, T. E. (2021) Dopamine D1 receptor activation and cAMP/PKA signalling mediate Brd4 recruitment to chromatin to regulate gene expression in rat striatal neurons. bioRxiv. https://doi.org/10.1101/2021.07.01.450754

Jones-Tabah, J.,Martin, R. D., Tanny, J. C., Clarke, P. B. S., and Hebert, T. E. (2021) High-content single-cell FRET imaging of cultured striatal neurons reveals novel cross-talk in the regulation of nuclear signalling by PKA and ERK1/2. Molecular pharmacology. in press

Jones-Tabah, J., Mohammad, H., Clarke, P. B. S., and Hébert, T. E. (2021) In vivo detection of GPCR-dependent signaling using fiber photometry and FRET-based biosensors. Methods  in press

Jones-Tabah, J., Mohammad, H., Hadj-Youssef, S., Kim, L. E. H., Martin, R. D., Benaliouad, F., Tanny, J. C., Clarke, P. B. S., and Hébert, T. E. (2020) Dopamine D1 receptor signalling in dyskinetic Parkinsonian rats revealed by fiber photometry using FRET-based biosensors. Scientific reports. 10, 14426

Khan, S. M., Martin, R. D., Gora, S., Bouazza, C.,Jones-Tabah, J.,Zhang, A., MacKinnon, S., Trieu, P., Clarke, P. B. S., Tanny, J. C., and Hébert, T. E. (2020) An interaction between Gβγ and RNA polymerase II regulates transcription in cardiac fibroblasts. bioRxiv. https://doi.org/10.1101/415935

Bourque, K., Jones-Tabah, J., Devost, D., Clarke, P. B. S., and Hébert, T. E. (2020) Exploring functional consequences of GPCR oligomerization requires a different lens. Progress in molecular biology and translational science. 169, 181-211

Bourque, K., Jones-Tabah, J., Mnasri, N., Martin, R. D., and Hébert, T. E. (2018) Combining Optical Approaches with Human Inducible Pluripotent Stem Cells in G Protein-Coupled Receptor Drug Screening and Development. Biomolecules. 8

Jones-Tabah, J., Clarke, P. B., and Hébert, T. E. (2017) Measuring G protein-coupled receptor signalling in the brain with resonance energy transfer based biosensors. Current opinion in pharmacology. 32, 44-48

Wang, Y., Jones-Tabah, J., Chakravarty, P., Stewart, A., Muotri, A., Laposa, R. R., and Svejstrup, J. Q. (2016) Pharmacological Bypass of Cockayne Syndrome B Function in Neuronal Differentiation. Cell reports. 14, 2554-2561

Shen, M.Y., Perreault, M.L., Bambico, F.R., Jones-Tabah, J., Cheung, M., Fan, T., Nobrega, J.N. & George, S.R. (2015). Rapid anti-depressant and anxiolytic actions following dopamine D1–D2 receptor heteromer inactivation. European Neuropsychopharmacology. 10.1016/j.euroneuro.2015.09.004

Perreault, M.L., Jones-Tabah, J., O'Dowd, B.F. and George, S.R. (2012). A physiological role for the dopamine D5 receptor as a regulator of BDNF and Akt signaling in rodent prefrontal cortex.  The International Journal of Neuropsychopharmacology. 10.1017/S1461145712000685


PhD STUDENTS

Photo of Emma McDougallEmma MacDougall

emma.macdougall [at] mail.mcgill.ca

I study the role of LRRK2 and Rab GTPases in the pathogenesis of Parkinson's disease by using induced pluripotent stem cell (iPSC) based models. I focus particularly on understanding the role of LRRK2-Rab signaling in iPSC derived microglia, and co-culturing microglia and neurons in 3 dimensions. My goal is to unravel the mechanisms underlying LRRK2-Rab communication and neuroinflammation in Parkinson's disease.

List of publications

Yi, W., MacDougall, E. J., Tang, M. Y., Krahn, A. I., Gan-Or, Z., Trempe, J. Fon, E. A. (2019) The landscape of Parkin variants reveals pathogenic mechanisms and therapeutic targets in Parkinson’s disease. Hum. Mol. Genet. 28, 2811–2825

Fava, V. M., Xu, Y. Z., Lettre, G., Van Thuc, N., Orlova, M., Thai, V. H., Tao, S., Croteau, N., Eldeeb, M. A., MacDougall, E. J., Cambri, G., Lahiri, R., Adams, L., Fon, E. A., Trempe, J., Cobat, A., Alcaïs, A., Abel, L., Schurr, E. (2019) Pleiotropic effects for Parkin and LRRK2 in leprosy type-1 reactions and Parkinson’s disease. Proc. Natl. Acad. Sci. U.S.A. 116, 15616–15624

Eldeeb, M. A., MacDougall, E. J., Ragheb, M. A., Fon, E. A. (2019) Beyond ER: Regulating TOM-Complex-Mediated Import by Ubx2. Trends Cell Biol. 29, 687–689

 


MSc STUDENTS

Photo of Ghislaine DeyabGhislaine Deyab

ghislaine.deyab [at] mail.mcgill.ca

My research is focused on measuring neuronal activity in midbrain organoids derived from iPSCs. Neural organoids could represent a specific model for each patient. This could help develop individual therapy by specifically modeling disease progression and predicting response to treatments. However, in order to use this model, it needs to reliably model disease progression. Parkinson's disease development often goes together with changes in neuronal activity in the midbrain. By measuring the activity of neuronal networks in midbrain organoids, I could define and compare the caracteristics of these networks. This study could bring elements allowing us to conclude whether such model could be used as a reliable tool to study Parkinson's disease progression in patients.

List of publications

Khoo S.Y.-S., Lecocq M.,Deyab, G., Chaudhri, N. (2019) Context and topography determine the role of basolateral amygdala metabotropic glutamate receptor 5 in appetitive Pavlovian responding. Neuropsychopharmacology 44(9):1524-1533

 

Photo of Nathan KarpilovskyNathan Karpilovsky

nathan.karpilovsky [at] mail.mcgill.ca

 

List of publications

 

 


RESEARCH ASSOCIATES

Photo of Vincent SoubannierVincent Soubannier, PhD

vincent.soubannier [at] mcgill.ca

I work as a microscopy specialist in Dr. Fon’s lab. I perform basic training for users on routine and advanced microscopy on a widefield live imaging system. I work with researchers to determine appropriate equipment for experiments and to develop experimental designs to address specific research questions. This includes consultation on image processing and analysis and the development of automatized analytic tools through imageJ macros, for instance. I finally do regular maintenance of the microscopy equipment and initiate repairs if necessary.

List of publications

Del Cid-Pellitero E, Tavassoly O, Larroquette F, Cai E, Thomas RA,Soubannier V, Luo W, Durcan TM, Fon EA. (2021) Pharmacological Inhibition of Brain EGFR Activation By a BBB-penetrating Inhibitor, AZD3759, Attenuates α-synuclein Pathology in a Mouse Model of α-Synuclein Propagation. Neurotherapeutics. 

Jefri M, Bell S, Peng H, Hettige N, Maussion G, Soubannier V , Wu H, Silveira H, Theroux JF, Moquin L, Zhang X, Aouabed Z, Krishnan J, O'Leary LA, Antonyan L, Zhang Y, McCarty V, Mechawar N, Gratton A, Schuppert A, Durcan TM, Fon EA, Ernst C. (2020) Stimulation of L-type calcium channels increases tyrosine hydroxylase and dopamine in ventral midbrain cells induced from somatic cells. Stem Cells Transl Med.

Methot L, Soubannier V, Hermann R, Campos E, Li S, Stifani S. (2018) Nuclear factor-kappaB regulates multiple steps of gliogenesis in the developing murine cerebral cortex. Glia

Thomas RA, Gibon J, Chen CXQ, Chierzi S, Soubannier V, Baulac S, Séguéla P, Murai K, Barker PA. (2018) The Nogo Receptor Ligand LGI1 Regulates Synapse Number and Synaptic activity in Hippocampal and Cortical Neurons. eNeuro

Bell S, Maussion G, Jefri M, Peng H, Theroux JF, Silveira H, Soubannier V, Wu H, Hu P, Galat E, Torres-Platas SG, Boudreau-Pinsonneault C, O'Leary LA, Galat V, Turecki G, Durcan TM, Fon EA, Mechawar N, Ernst C. (2018) Disruption of GRIN2B Impairs Differentiation in Human Neurons. Stem Cell Reports.

Soubannier V, Stifani S. (2017) NF-κB Signalling in Glioblastoma. Biomedicines.

Gaub P, de Léon A, Gibon J, Soubannier V, Dorval G, Séguéla P, Barker PA. (2016) HBpF-proBDNF: A New Tool for the Analysis of Pro-Brain Derived Neurotrophic Factor Receptor Signaling and Cell Biology. PloS One.

Patten DA, Wong J, Khacho M, Soubannier V, Mailloux RJ, Pilon-Larose K, MacLaurin JG, Park DS, McBride HM, Trinkle-Mulcahy L, Harper ME, Germain M, Slack RS. (2014) OPA1-dependent cristae modulation is essential for cellular adaptation to metabolic demand. EMBO J.

McLelland GL, Soubannier V, Chen CX, McBride HM, Fon EA. (2014) Parkin and PINK1 function in a vesicular trafficking pathway regulating mitochondrial quality control. EMBO J.

Soubannier V, Rippstein P, Kaufman BA, Shoubridge EA, McBride HM. (2012) Reconstitution of mitochondria derived vesicle formation demonstrates selective enrichment of oxidized cargo. PloS One.

Soubannier V,McLelland GL, Zunino R, Braschi E, Rippstein P, Fon EA, McBride HM. (2012) A vesicular transport pathway shuttles cargo from mitochondria to lysosomes. Curr. Biol.

McBride H, Soubannier V (2010) Mitochondrial function: OMA1 and OPA1, the grandmasters of mitochondrial health. Curr. Biol.

Rabl R, Soubannier V, Scholz R, Vogel F, Mendl N, Vasiljev-Neumeyer A, Körner C, Jagasia R, Keil T, Baumeister W, Cyrklaff M, Neupert W, Reichert AS. (2009) Formation of cristae and crista junctions in mitochondria depends on antagonism between Fcj1 and Su e/g. J. Cell. Biol.

Soubannier V, McBride HM. (2009) Positioning mitochondrial plasticity within cellular signaling cascades. Biochim. Biophys. Acta.

Giraud MF, Paumard P, Soubannier V, Vaillier J, Arselin G, Salin B, Schaeffer J, Brèthes D, di Rago JP, Velours J. (2002) Is there a relationship between the supramolecular organization of the mitochondrial ATP synthase and the formation of cristae? Biochim. Biophys. Acta.

Paumard P, Vaillier J, Coulary B, Schaeffer J, Soubannier V, Mueller DM, Brèthes D, di Rago JP, Velours J. (2002) The ATP synthase is involved in generating mitochondrial cristae morphology. EMBO J.

Soubannier V, Vaillier J, Paumard P, Coulary B, Schaeffer J, Velours J. (2002) In the absence of the first membrane-spanning segment of subunit 4(b), the yeast ATP synthase is functional but does not dimerize or oligomerize. J. Biol. Chem.

Velours J, Vaillier J, Paumard P, Soubannier V, Lai-Zhang J, Mueller DM. (2001) Bovine coupling factor 6, with just 14.5% shared identity, replaces subunit h in the yeast ATP synthase. J. Biol. Chem.

Velours J, Paumard P, Soubannier V, Spannagel C, Vaillier J, Arselin G, Graves PV. (2000) Organisation of the yeast ATP synthase F(0):a study based on cysteine mutants, thiol modification and cross-linking reagents. Biochim. Biophys. Acta.

Soubannier V, Rusconi F, Vaillier J, Arselin G, Chaignepain S, Graves PV, Schmitter JM, Zhang JL, Mueller D, Velours J. (1999) The second stalk of the yeast ATP synthase complex: identification of subunits showing cross-links with known positions of subunit 4 (subunit b). Biochemistry

 

Photo of Roxanne LariviereRoxanne Larivière, PhD

roxanne.lariviere [at] mcgill.ca

My research interest lies in better understanding the role of GWAS-associated PD genes and characterize the lesser-studied PD genes in genetically-modified induced pluripotent stem cell derived midbrain organoids. With the use of PD phenotypic assays and unbiased multi-omics, we hope to disentangle the PD GWAS-ome by uncovering new pathways or mechanisms in PD pathology and new key targets for therapy

 

 

 

List of publications

Choquet K, Pinard M, Yang S, Moir RD, Poitras C, Dicaire M-J, Sgarioto N, Larivière R, Kleinman CL, Willis IM, Gauthier M-S, Coulombe B, Brais B. (2019) The leukodystrophy mutation Polr3b R103H causes homozygote mouse embryonic lethality and impairs RNA Polymerase III biogenesis. Mol. Brain

Larivière R, Sgarioto N, Toscano Márquez B, Gaudet R, Choquet K, McKinney RA, Watt AJ, Brais B. (2019) Sacs R272C missense homozygous mice develop an ataxia phenotype. Mol. Brain

Walker CL, Uchida A, Li Y, Trivedi N, Fenn JD, Monsma PC, Larivière RC, Julien JP, Jung P, Brown A. (2019) Local Acceleration of Neurofilament Transport at Nodes of Ranvier. J Neurosci.

Gentil BJ, Lai GT, Menade M, Larivière R, Minotti S, Gehring K, Chapple JP, Brais B, Durham HD. (2018) Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics. FASEB J.

Ady V, Toscano-Márquez B, Nath M, Chang PK, Hui J, Cook A, Charron F, Larivière R, Brais B, McKinney RA, Watt AJ. (2018) Altered synaptic and firing properties of cerebellar Purkinje cells in a mouse model of ARSACS. J Physiol.

Duncan EJ, Larivière R , Bradshaw TY, Longo F, Sgarioto N, Hayes MJ, Romano LEL, Nethisinghe S, Giunti P, Bruntraeger MB, Durham HD, Brais B, Maltecca F, Gentil BJ, Chapple JP. (2017) Altered organization of the intermediate filament cytoskele-ton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin. Hum Mol Genet.

Choquet K, Yang S, Moir RD, Forget D, Larivière R , Bouchard A, Poitras C, Sgarioto N, Dicaire MJ, Noohi F, Kennedy TE, Roch-ford J, Bernard G, Teichmann M, Coulombe B, Willis IM, Klein-man CL, Brais B. (2017) Absence of neurological abnormalities in mice homozygous for the Polr3a G672E hypomyelinating leukodystrophy mutation. Mol Brain.

Larroquette F, Seto L, Gaub PL, Kamal B, Wallis D, Larivière R, Vallée J, Robitaille R, Tsuda H. (2015) Vapb/Amyotrophic lateral sclerosis 8 knock-in mice display slowly progressive motor behavior defects accompanying ER stress and autophagic response. Hum Mol Genet.

Larivière R, Gaudet R, Gentil BJ, Girard M, Conte TC, Minotti S, Leclerc-Desaulniers K, Gehring K, McKinney RA, Shoubridge EA, McPherson PS, Durham HD, Brais B. (2015) Sacs knockout mice present pathophysiological defects underlying autosomal recessive spastic ataxia of Charlevoix-Saguenay. Hum Mol Genet.

Thiffault I, Dicaire MJ, Tetreault M, Huang KN, Demers-La-marche J, Bernard G, Duquette A, Larivière R, Gehring K, Montpetit A, McPherson PS, Richter A, Montermini L, Mercier J, Mitchell GA, Dupré N, Prévost C, Bouchard JP, Mathieu J, Brais B. (2013) Diversity of ARSACS mutations in French-Canadians. Can J Neurol Sci.

Girard M1, Larivière R1, Parfitt DA, Deane EC, Gaudet R, Noss-ova N, Blondeau F, Prenosil G, Vermeulen EGM, Duchen MR, Richter A, Shoubridge EA, Gehring K, McKinney RA, Brais B, Chapple JP, and McPherson PS. (2012 10) Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) PNAS (1: co-authors)

Ezzi SA, Larivière R, Urushitani M, Julien JP. (2010) Neuronal over-ex-pression of chromogranin A accelerates disease onset in a mouse model of ALS. J. Neurochem.

Dequen F, Filali M, Larivière RC, Perrot R, Hisanaga S, Julien JP. (2010) Reversal of neuropathy phenotypes in conditional mouse model of Charcot-Marie-Tooth disease type 2E. Hum Mol Genet.

Gros-Louis F, Soucy G, Larivière R, Julien JP. (2010) Intracerebroven-tricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS. J Neurochem.

Eriksson KS, Zhang S, Lin L, Larivière RC, Julien JP, Mignot E. (2008) The type III neurofilament peripherin is expressed in the tubero-mammillary neurons of the mouse. BMC Neurosci.

Millecamps S, Robertson J, Lariviere R, Mallet J, Julien JP. (2006) Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin. J Neurochem.

Gros-Louis F, Larivière R, Gowing G, Laurent S, Camu W, Bouchard JP, Meininger V, Rouleau GA, Julien JP. (2004) A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis. J Biol Chem.

Nguyen MD, Shu T, Sanada K, Larivière RC, Tseng HC, Park SK, Julien JP, Tsai LH. (2004) A NUDEL¬dependent mechanism of neurofil-ament assembly regulates the integrity of CNS neurons. Nat Cell Biol.

Lariviere RC, Julien JP. (2004) Functions of intermediate filaments in neuronal development and disease. J Neurobiol. Review.

Larivière RC, Beaulieu JM, Nguyen MD, Julien JP. (2003) Peripherin is not a contributing factor to motor neuron disease in a mouse model of amyotrophic lateral sclerosis caused by mutant superoxide dismutase. Neurobiol Dis.

Larivière RC, Nguyen MD, Ribeiro-da-Silva A, Julien JP. (2002) Reduced number of unmyelinated sensory axons in peripher-in null mice. J Neurochem.

Nguyen MD, Larivière RC, Julien JP. (2001) Deregulation of Cdk5 in a mouse model of ALS: toxicity alleviated by perikaryal neurofil-ament inclusions. Neuron.

Nguyen MD, Larivière RC, Julien JP. (2000) Reduction of axonal caliber does not alleviate motor neuron disease caused by mutant superoxide dismutase 1. Proc Natl Acad Sci U S A.

 


RESEARCH ASSISTANTS

Photo of Frederique LarroquetteFrédérique Larroquette, MSc

frederique.larroquette [at] mcgill.ca

I am investigating the role of genetic factors and alpha-synuclein propagation in mouse models of Parkinson's disease. I am also managing the lab in its day to day organization.

 

 

 

 

List of publications

Del Cid-Pellitero E, Tavassoly O, Larroquette F, Cai E, Thomas RA, Soubannier V, Luo W, Durcan TM, Fon EA. (2021) Pharmacological Inhibition of Brain EGFR Activation By a BBB-penetrating Inhibitor, AZD3759, Attenuates α-synuclein Pathology in a Mouse Model of α-Synuclein Propagation. Neurotherapeutics. 

Larroquette F., Mohamed NV, Beitel LK, Fon EA, Durcan TM. (2019) One step into the future: new iPSC tools to advance research in Parkinson’s disease and neurological disorders. Journal of Parkinson’s disease doi: 10.3233/JPD-181515

Larroquette F, Seto L, Gaub PL, Kamal B, Wallis D, Larivière R, Vallée J, Robitaille R, Tsuda H. (2015) Vapb/Amyotrophic lateral sclerosis 8 knock-in mice display slowly progressive motor behavior defects accompanying ER stress and autophagic response. Human Molecular Genetics ; 24(22):6515-29.

 

Photo of Esther Del Cid PeliterroEsther Del Cid Pellitero, PhD

esther.delcidpellitero [at] mcgill.ca

In Dr. Fon lab, I am the manager of pre-clinical and research mouse projects. I study the implications α–synuclein propagation as well as Parkin, Pink 1 and related proteins in Parkinson Disease.

List of publications

Del Cid-Pellitero E, Tavassoly O, Larroquette F, Cai E, Thomas RA, Soubannier V, Luo W, Durcan TM, Fon EA. (2021) Pharmacological Inhibition of Brain EGFR Activation By a BBB-penetrating Inhibitor, AZD3759, Attenuates α-synuclein Pathology in a Mouse Model of α-Synuclein Propagation. Neurotherapeutics. 

Del Cid-Pellitero E, Jin JW, Fan X, Liu XX, Zhou L, Dai C, Gibbs E, He W, Li H, Wu X, Hill A, Leavitt BR, Cashman N, Liu L, Lu J, Durcan TM, Dong Z, Fon EA & Wang YT. (2021) Development of an α-synuclein knockdown peptide and evaluation of its efficacy in Parkinson’s disease models. Communications Biology. 4, 232

Toossi H, Del Cid-Pellitero E, Jones BE. (2018) Homeostatic Changes in GABA and Acetylcholine Muscarinic Receptors on GABAergic Neurons in the Mesencephalic Reticular Formation following Sleep Deprivation. eNeuro. 3;4(6)

Reynolds LM, Pokinko M, Torres-Berrío A, Cuesta S, Lambert LC, Del Cid-Pellitero E, Wodzinski M, Manitt C, Krimpenfort P, Kolb B, Flores C. (2018) DCC Receptors Drive Prefrontal Cortex Maturation by Determining Dopamine Axon Targeting in Adolescence.  Biol Psychiatry. 15:181-192

Del Cid-Pellitero E, Plavski A, Mainville L, Jones BE. (2017) Homeostatic Changes in GABA and Glutamate Receptors on Excitatory Cortical Neurons during Sleep Deprivation and Recovery.  Front Syst Neurosci. 31;11-17

Toossi H, Del Cid-Pellitero E, Jones BE. (2017) Homeostatic regulation through GABA and acetylcholine muscarinic receptors of motor trigeminal neurons following sleep deprivation.  Brain Struct Funct. 222:3163-3178

Toossi H, Del Cid-Pellitero E, Jones BE. (2016) GABA Receptors on Orexin and Melanin-Concentrating Hormone Neurons Are Differentially Homeostatically Regulated Following Sleep Deprivation.  eNeuro. 9;3(3)

Del Cid-Pellitero E, Garzón M. (2014) Hypocretin1/orexinA-immunoreactive axons form few synaptic contacts on rat ventral tegmental area neurons that project to the medial prefrontal cortex.  BMC Neurosci. 15:10

Del Cid-Pellitero E, Jones BE. (2012) Immunohistochemical evidence for synaptic release of GABA from melanin- concentrating hormone containing varicosities in the locus coeruleus.  Neuroscience. 223:269-276

Toossi H, Del Cid-Pellitero E, Stroh T, Jones BE. (2012) Somatostatin varicosities contain the vesicular GABA transporter and contact orexin neurons in the hypothalamus.  Eur J Neurosci. 10: 3388-3395

Del Cid-Pellitero E, Garzón M. (2011) Hypocretin 1/orexin A axon targeting of laterodorsal tegmental nucleus neurons projecting to the rat medial prefrontal cortex.  Cerebral Cortex. 21:2762-2773

Del Cid-Pellitero E, Garzón M. (2011) Medial prefrontal cortex receives input from dorsal raphe nucleus neurons targeted by hypocretin1/orexinA-containing axons.  Neuroscience. 172:30-43

Del Cid-Pellitero E, Garzón M. (2011) Hypocretin 1/orexin A-containing axons innervate locus coeruleus neurons that project to the rat medial prefrontal cortex. Implication in the sleep-wakefulness cycle and cortical activation.  Synapse. 65:843-857

Del Cid-Pellitero E, Garzón M. (2007) Modulation by the hypocretinergic/orexinergic neurotransmission system in sleep-wakefulness cycle states.   Rev. Neurol. 45:482-49

 

Photo of Cornelia ZorcaCornelia Zorca, PhD

cornelia.zorca [at] mcgill.ca

CRISPR/Cas9 screening of PD-associated genes

 

 

 

 

 

List of publications

Lessard, F., Ingelmann, S., Trahan, C., Huot, G., Saint-Germain, E., Mignacca, L., DelToro, N., Lopes-Paciencia, S., Le Calve, B., Montero, M., Deschenes-Simard, X., Bury, M., Moiseeva, O., Rowell, M-C., Zorca, C.E., Zenklusen, D., Brakier-Gingras, L., Boudreau, V., Oeffinger, M., Ferbeyre, G. Ribosome biogenesis defects in senescence reveal a novel checkpoint pathway to control CDK4: in press Nat. Cell Biol.

Zorca.C.E.*, Rahman, S.*, Noutahi, E., Krause, M.R., Zenklusen, D. Single-cell profiling reveals that eRNAs accumulation at enhancer-promoter loops is not required for bursting transcription: Nucleic Acid Res., 45(6): 3017-3030, 2017.

*equal contribution

Zorca, C.E., Kim, L.K., Kim, Y.J., Krause,M.R., Zenklusen, D., Spilianakis, C.G., Flavell, R.A. Myosin VI regulates gene pairing and transcriptional pause release in T cells: PNAS, 112(13) E1587-E1593, 2015.

Kim, L.K., Esplugues, E., Zorca, C.E., Parisi, F., Kluger, Y., Kim, T.H., Galjart, N.J., Flavell, R.A., Oct-1 regulates IL-17 expression by directing interchromosomal associations in conjunction with CTCF in T cells: Mol. Cell., 54(1): 56-66, 2014.

Zorca, S.M., Zorca, C.E., The Legacy of a Founding Father of Modern Cell Biology: George Emil Palade (1910-2008): YJBM., 84(2): 113-116, 2011.

Granneman, S., Lin, C.Y., Champion, E.A., Nandineni, M.R., Zorca, C., Baserga, S.J., The nucleolar protein Esf2 interacts directly with the DexD/H box RNA helicase, Dbp8, to stimulate ATP hydrolysis: Nucleic Acid Res., 34(10): 3189-3199, 2006.

 


UNDERGRADUATE STUDENTS

Xue Ding

xue.ding [at] mail.mcgill.ca

Andrea Soumbassis

andrea.soumbasis [at] mail.mcgill.ca

Sophie Luo

sophie.luo [at] mail.mcgill.ca

Jiarui Ao

jiarui.ao [at] mail.mcgill.ca

 

Our lab is part of the Neuro (Montreal Neurological Institute and Hospital), a McGill research and teaching institute, delivering high quality patient care, as part of the Neuroscience Mission of the McGill University Health Centre.

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