The Pro-Apoptotic JNK Scaffold POSH/SH3RF1 Mediates CHMP2BIntron5-Associated Toxicity in Animal Models Frontotemporal Dementia

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West, Ryan John Hatcher; Ugbode, Christopher; Gao, Fen-Biao; Sweeney, Sean;
(2018)
  • Subject:
    mesheuropmc: mental disorders

Frontotemporal Dementia (FTD) is one of the most prevalent forms of early-onset dementia. However, the pathological mechanisms driving neuronal atrophy in FTD remain poorly understood. Here we identify a conserved role for the novel pro-apoptotic protein POSH/SH3RF1 in ... View more
  • References (57)
    57 references, page 1 of 6

    1. Broe, M., Kril, J. and Halliday, G.M. (2004) Astrocytic degeneration relates to the severity of disease in frontotemporal dementia. Brain, 127, 2214-2220.

    2. Ferrari, R., Forabosco, P., Vandrovcova, J., Botı´a, J.A., Guelfi, S., Warren, J.D., Momeni, P., Weale, M.E., Ryten, M. and Hardy, J. (2016) Frontotemporal dementia: insights into the biological underpinnings of disease through gene co-expression network analysis. Mol. Neurodegener., 11, 21.

    3. Ferrari, R., Hernandez, D.G., Nalls, M.A., Rohrer, J.D., Ramasamy, A., Kwok, J.B., Dobson-Stone, C., Brooks, W.S., Schofield, P.R., Halliday, G.M. et al. (2014) Frontotemporal dementia and its subtypes: a genome-wide association study. Lancet Neurol., 13, 686-699.

    4. Lui, H., Zhang, J., Makinson, S.R., Cahill, M.K., Kelley, K.W., Huang, H.Y., Shang, Y., Oldham, M.C., Martens, L.H., Gao, F. et al. (2016) Progranulin deficiency promotes circuit-specific synaptic pruning by microglia via complement activation. Cell, 165, 921-935.

    5. Sjogren, M., Folkesson, S., Blennow, K. and Tarkowski, E. (2004) Increased intrathecal inflammatory activity in frontotemporal dementia: pathophysiological implications. J. Neurol. Neurosurg. Psychiatry, 75, 1107-1111.

    6. Su, J.H., Nichol, K.E., Sitch, T., Sheu, P., Chubb, C., Miller, B.L., Tomaselli, K.J., Kim, R.C. and Cotman, C.W. (2000) DNA damage and activated caspase-3 expression in neurons and astrocytes: evidence for apoptosis in frontotemporal dementia. Exp. Neurol., 163, 9-19.

    7. West, R.J., Lu, Y., Marie, B., Gao, F.B. and Sweeney, S.T. (2015) Rab8, POSH, and TAK1 regulate synaptic growth in a Drosophila model of frontotemporal dementia. J. Cell Biol., 208, 931-947.

    8. Li, M., Liu, Y., Xia, F., Wu, Z., Deng, L., Jiang, R. and Guo, F.J. (2014) Progranulin is required for proper ER stress response and inhibits ER stress-mediated apoptosis through TNFR2. Cell. Signal, 26, 1539-1548.

    9. Vandermoere, F., El Yazidi-Belkoura, I., Slomianny, C., Demont, Y., Bidaux, G., Adriaenssens, E., Lemoine, J. and Hondermarck, H. (2006) The valosin-containing protein (VCP) is a target of Akt signaling required for cell survival. J. Biol. Chem., 281, 14307-14313.

    10. Ahmad, S.T., Sweeney, S.T., Lee, J.A., Sweeney, N.T. and Gao, F.B. (2009) Genetic screen identifies serpin5 as a regulator of the toll pathway and CHMP2B toxicity associated with frontotemporal dementia. Proc. Natl. Acad. Sci. USA, 106, 12168-12173.

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