The Role of Nucleoporins and Nuclear Pore Complexes in Neurodegeneration

Recent Publications

  • Rappold PM, Cui M, Chesser AS, Tibbett J, Grima JC, Duan L, Sen N, Javitch JA& Tieu K. Paraquat neurotoxicity is mediated by the dopamine transporter and organic cation transporter-3. Proceedings of the National Academy of Sciences of the United States of Amierica, 2011; 108(51):20766-71. doi: 10.1073/pnas.1115141108.
  • Lim S, Chesser AS, Grima JC, Rappold PM, Blum D, Przedborski S, Tieu K. D-b-hydroxybutyrate is protective in mouse models of Huntington’s disease. PLoS One, 2011; 6(9):e24620. doi: 10.1371/journal.pone.0024620.
  • Rappold PM, Cui M, Grima JC, Fan RZ, de Mesy-Bentley KL, Chen L, Zhuang X, Bowers WJ & Tieu K. Drp1 inhibtion attenuates neurotoxicity and dopamine release deficits in vivo. Nature Communications, 2014; 5:5244. doi: 10.1038/ncomms6244.
  • Donnelly CJ, Grima JC, Sattler R. Aberrant RNA homeostasis in amyotrophic lateral sclerosis: potential for new therapeutic targets? Future Medicine 2014; 4(6):417-37. doi: 10.2217/nmt.14.36.
  • Ke Zhang, Christopher J. Donnelly, Aaron R. Haeusler, Jonathan C. Grima, James B. Machamer, Elizabeth L. Daley, Svetlana Vidensky, Michael A. Thomas, Kathleen M. Cunningham, Peter Steinwald, Michael J. Matunis, Jiou Wang, Rita Sattler, Thomas E. Lloyd, Jeffrey D. Rothsein. The C90RF72 repeat expansion disrupts nucleocytoplasmic transport. Under review at Nature, 2015.
  • Ahmed I, Sbodio JI, Harraz MM, Tyagi R, Albacarys LK, Hubbi ME, Grima JC, Xu R, Kim S, Paul BD, Snyder SH. Huntington’s disease: Neural dysfunction linked to inositol polyphosphate multikinase. Submitted to Neuron, 2015.

Recent Presentations

  • Yulan Xiong, Xiaobo Mao, Jeannette N. Stankowski, Byoung Dae Lee, Han Seok Ko, Yunjong
    Lee, Stewart Neifert, Jonathan Grima, Debbie Swing, Lorraine Iacovitti, Lino Tessarollo, Ted
    M. Dawson and Valina L. Dawson. A novel mouse model of Parkinson’s disease with conditional
    expression of mutant LRRK2 in dopaminergic neurons causes progressive neurodegeneration.
    Society for Neuroscience 2014, November 15-19th 2014 [Oral Presentation].
  • Ahmed I, Sbodio JI, Harraz MM, Tyagi R, Albacarys LK, Hubbi ME, Grima JC, Xu R, Kim S,
    Paul BD, Snyder SH. Huntington’s disease: Neural dysfunction linked to inositol polyphosphate
    multikinase. Society for Neuroscience 2014, November 15-19th 2014 [Oral Presentation].
  • Donnelly CJ, Zhang K, Haeusler AR, Daley EL, Grima JC, Vidensky S, Wang J, Lloyd TE,
    Sattler R, Rothstein JD. Nuclear transport defect underlies C9ORF72 ALS/FTD neuronal injury in
    human neurons and is rescued by antisense and small molecules that target GGGGCC RNA.
    Society for Neuroscience 2014, November 15-19th 2014 [Poster Presentation].
  • Successfully defended senior thesis titled Therapeutic potential of blocking dynamin-related protein-1
    (Drp1) function in the R6/2 mouse model of Huntington’s disease on May 3, 2012.
  • Jonathan Grima, Soyeon Lim, Phillip Rappold, William Bowers, Kim Tieu. Therapeutic
    potential of blocking dynamin-related protein-1 (Drp1) function in the R6/2 mouse model of
    Huntington’s disease. University of Rochester Undergraduate Research Exposition, April 20th
    2012 [Oral Presentation – Awarded Deans’ Prize].
  • Jonathan Grima, Soyeon Lim, Phillip Rappold, William Bowers, Kim Tieu. Therapeutic
    potential of blocking dynamin-related protein-1 (Drp1) function in the R6/2 mouse model of
    Huntington’s disease. National Conference on Undergraduate Research, March 29-31st 2012 [Oral
    Presentation].
  • Jonathan Grima, Soyeon Lim, Phillip Rappold, William Bowers, Kim Tieu. Therapeutic
    potential of blocking dynamin-related protein-1 (Drp1) function in the R6/2 mouse model of
    Huntington’s disease. University of Rochester Undergraduate Program in Biology and Medicine
    Poster Session, October 21st 2011 [Poster Presentation].