Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains

Arai, 2006, TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis, Biochem. Biophys. Res. Commun., 351, 602, 10.1016/j.bbrc.2006.10.093

Arai, 2010, Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy, Neuropathology, 30, 170, 10.1111/j.1440-1789.2009.01089.x

Bence, 2001, Impairment of the ubiquitin-proteasome system by protein aggregation, Science, 292, 1552, 10.1126/science.292.5521.1552

Bigio, 2013, Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid, Acta Neuropathol., 125, 463, 10.1007/s00401-013-1089-6

Braak, 1991, Neuropathological stageing of Alzheimer-related changes, Acta Neuropathol., 82, 239, 10.1007/BF00308809

Braak, 2003, Staging of brain pathology related to sporadic Parkinson’s disease, Neurobiol. Aging, 24, 197, 10.1016/S0197-4580(02)00065-9

Brooks, 1994, J. Neurol. Sci., 124, 96, 10.1016/0022-510X(94)90191-0

Budini, 2012, Cellular model of TAR DNA binding protein 43 (TDP-43) aggregation based on its C-terminal Q/N rich region, J. Biol. Chem., 287, 7512, 10.1074/jbc.M111.288720

Buratti, 2009, The molecular links between TDP-43 dysfunction and neurodegeneration, Adv. Genet., 66, 1, 10.1016/S0065-2660(09)66001-6

Buratti, 2001, Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping, EMBO J., 20, 1774, 10.1093/emboj/20.7.1774

Clavaguera, 2009, Transmission and spreading of tauopathy in transgenic mouse brain, Nat. Cell Biol., 11, 909, 10.1038/ncb1901

de Calignon, 2012, Propagation of tau pathology in a model of early Alzheimer’s disease, Neuron, 73, 685, 10.1016/j.neuron.2011.11.033

Desplats, 2009, Inclusion formation and neuronal cell death through neuron-to-neuron transmission of alpha-synuclein, Proc. Natl. Acad. Sci. USA, 106, 13010, 10.1073/pnas.0903691106

Fevrier, 2004, Cells release prions in association with exosomes, Proc. Natl. Acad. Sci. USA, 101, 9683, 10.1073/pnas.0308413101

Frost, 2009, Propagation of tau misfolding from the outside to the inside of a cell, J. Biol. Chem., 284, 12845, 10.1074/jbc.M808759200

Furukawa, 2011, A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions, J. Biol. Chem., 286, 18664, 10.1074/jbc.M111.231209

Goedert, 2010, The propagation of prion-like protein inclusions in neurodegenerative diseases, Trends Neurosci., 33, 317, 10.1016/j.tins.2010.04.003

Gousset, 2009, Prions hijack tunnelling nanotubes for intercellular spread, Nat. Cell Biol., 11, 328, 10.1038/ncb1841

Guo, 2011, An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity, Nat. Struct. Mol. Biol., 18, 822, 10.1038/nsmb.2053

Hasegawa, 2008, Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis, Ann. Neurol., 64, 60, 10.1002/ana.21425

Hasegawa, 2011, Molecular dissection of TDP-43 proteinopathies, J. Mol. Neurosci., 45, 480, 10.1007/s12031-011-9571-x

Igaz, 2009, Expression of TDP-43 C-terminal fragments in vitro recapitulates pathological features of TDP-43 proteinopathies, J. Biol. Chem., 284, 8516, 10.1074/jbc.M809462200

Kordower, 2008, Lewy body-like pathology in long-term embryonic nigral transplants in Parkinson’s disease, Nat. Med., 14, 504, 10.1038/nm1747

Li, 2008, Lewy bodies in grafted neurons in subjects with Parkinson’s disease suggest host-to-graft disease propagation, Nat. Med., 14, 501, 10.1038/nm1746

Liu, 2012, Trans-synaptic spread of tau pathology in vivo, PLoS ONE, 7, e31302, 10.1371/journal.pone.0031302

Luk, 2009, Exogenous alpha-synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cells, Proc. Natl. Acad. Sci. USA, 106, 20051, 10.1073/pnas.0908005106

Luk, 2012, Pathological α-synuclein transmission initiates Parkinson-like neurodegeneration in nontransgenic mice, Science, 338, 949, 10.1126/science.1227157

Luk, 2012, Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice, J. Exp. Med., 209, 975, 10.1084/jem.20112457

Mackenzie, 2011, A harmonized classification system for FTLD-TDP pathology, Acta Neuropathol., 122, 111, 10.1007/s00401-011-0845-8

Masuda-Suzukake, 2013, Prion-like spreading of pathological α-synuclein in brain, Brain, 136, 1128, 10.1093/brain/awt037

Neary, 1998, Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria, Neurology, 51, 1546, 10.1212/WNL.51.6.1546

Neumann, 2006, Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis, Science, 314, 130, 10.1126/science.1134108

Nonaka, 2009, A cellular model to monitor proteasome dysfunction by alpha-synuclein, Biochemistry, 48, 8014, 10.1021/bi900619j

Nonaka, 2009, Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43, Hum. Mol. Genet., 18, 3353, 10.1093/hmg/ddp275

Nonaka, 2010, Seeded aggregation and toxicity of alpha-synuclein and tau: cellular models of neurodegenerative diseases, J. Biol. Chem., 285, 34885, 10.1074/jbc.M110.148460

Pesiridis, 2009, Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis, Hum. Mol. Genet., 18, R156, 10.1093/hmg/ddp303

Polymenidou, 2011, The seeds of neurodegeneration: prion-like spreading in ALS, Cell, 147, 498, 10.1016/j.cell.2011.10.011

Ren, 2009, Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates, Nat. Cell Biol., 11, 219, 10.1038/ncb1830

Sephton, 2010, TDP-43 is a developmentally regulated protein essential for early embryonic development, J. Biol. Chem., 285, 6826, 10.1074/jbc.M109.061846

Snowden, 2002, Frontotemporal dementia, Br. J. Psychiatry, 180, 140, 10.1192/bjp.180.2.140

Tsuji, 2012, Molecular analysis and biochemical classification of TDP-43 proteinopathy, Brain, 135, 3380, 10.1093/brain/aws230

Wu, 2010, TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis, Genesis, 48, 56, 10.1002/dvg.20584

Zhang, 2009, Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity, Proc. Natl. Acad. Sci. USA, 106, 7607, 10.1073/pnas.0900688106

Fujiwara, 2002, α-Synuclein is phosphorylated in synucleinopathy lesions, Nat. Cell Biol., 4, 160, 10.1038/ncb748

Inukai, 2008, Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS, FEBS Lett., 582, 2899, 10.1016/j.febslet.2008.07.027