Feil Family Brain & Mind Research Institute

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Magrane, Jordi - Manfredi Laboratory

Jordi Magrane, Ph.D.
Assistant Professor of Neuroscience



My research aims to understand how changes in the dynamics and distribution of mitochondria contribute to synaptic dysfunction and axonal neurodegeneration in neurodegenerative diseases. We are currently investigating the involvement of mitochondrial dynamics in Friedreich’s Ataxia, using cultured neurons and in vivo mouse models. We are also generating novel transgenic mouse models where normal mitochondrial distribution in neurons is altered.

For the past few years, our research has focused on studying abnormalities in axonal dynamics of mitochondria (transport, fusion, and fission), an emerging topic in the field of neurodegenerative diseases. We have dedicated efforts toward developing novel microscopy-based, live imaging assays to assess the role of mitochondrial dynamics to disease pathogenesis both in vitro and in vivo. In the future, we plan to combine these live imaging assays with molecular approaches to identify the mechanisms that cause axonal transport abnormalities in certain neurodegenerative diseases and to investigate the spatial and temporal relationship between mitochondria and synaptic plasticity.


Achievements & Publications

  • Kim HJ, Magrané J, Starkov AA, Manfredi G. (2012) The mitochondrial calcium regulator cyclophilin D is an essential component of estrogen mediated neuroprotection in amyotrophic lateral sclerosis. Brain, 135:2865-74. PMID: 22961554
  • Magrané J*, Sahawneh MA, Przedborski S, Estevez AG, Manfredi G. (2012) Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. J Neurosci, 32:229-42. PMID: 22219285 *Corresponding author
  • Igoudjil* A, Magrané* J, Fischer LR, Kim HJ, Hervias I, Dumont M, Cortez C, Glass JD, Starkov AA, Manfredi G (2011) In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space. J Neurosci, 31:15826-37. PMID: 22049426 *These authors contributed equally to the work
  • Fonte V, Dostal V, Roberts CM, Gonzales P, Lacor P, Magrané J, Dingwell N, Fan NY, Silverman MA, Stein G, Link CD (2011)  A glycine zipper motif mediates the formation of toxic beta-amyloid oligomers in vitro and in vivo. Mol Neurodegener Aug 23;6(1):61. PMID: 21861874
  • Kawamata H, Tiranti V, Magrané J , Chinopoulos C, Manfredi G (2011) adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria. Hum Molec Genet. 20(15):2964-74. Epub 2011 May 17. PMID: 21586654
  • Wilk S, Scheibenbogen C, Bauer S, Jenke A, Rother M, Guerreiro M, Kudernatsch R1, Goerner N, Poller W, X Elligsen-Merkel D, Utku N, Magrané J, Volk HD, Skurk C (2011) Adiponectin is a negative regulator of antigen-activated T cells. Eur J Immunol, May 2. doi: 10.1002/eji.201041349. [Epub ahead of print]. PMID: 21538348
  • Fischer LR, Igoudjil A, Magrané J, Li Y, Hansen JM, Manfredi G, Glass JD (2011) SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the SOD1 knockout mouse. Brain 134 (Pt 1):196-209. Epub 2010 Nov 14. PMID: 21078595
  • Vives-Bauza C, Zhou C, Huang Y, Cui M, de Vries RLA, Kim J, May J, Tocilescu MA, Liu W, Ko HS, Magrané J, Moore DJ, Dawson VL, Grailhe R, Dawson TM, Li C, Tieu K, Przedborski S (2010) PINK1-dependent recruitment of Parkin to mitochondria in mitophagy. Proc Natl Acad Sci USA. 107(1):378-83. Epub 2009 Dec 4. PMID: 19966284
  • Vives-Bauza C, Magrané J, Andreu A, Manfredi G (2010) Novel role of ATPase subunit c targeting peptides beyond mitochondrial protein import. Molec Biol Cell. 21(1):131-9. Epub 2009 Nov 4. PMID: 19889836
  • Magrané J, Hervias I, Henning MS, Damiano M, Kawamata H, Manfredi G (2009) Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities. Hum Mol Genet. 18(23):4552-64. Epub 2009 Sep 24. PMID: 19779023

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