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1)Wegorzewska I, Bell S, Cairns NJ, et al. TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A. 2009; 106: 18809-14
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2)Wils H, Kleinberger G, Janssens J, et al. TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A. 2010; 107: 3858-63
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3)Tsai KJ, Yang CH, Fang YH, et al. Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U. J Exp Med. 2010; 207: 1661-73
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4)Stallings NR, Puttaparthi K, Luther CM, et al. Progressive motor weakness in transgenic mice expressing human TDP-43. Neurobiol Dis. 2010; 40: 404-14
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5)Xu YF, Gendron TF, Zhang YJ, et al. Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci. 2010; 30: 10851-9
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6)Shan X, Chiang PM, Price DL, et al. Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. Proc Natl Acad Sci U S A. 2010; 107: 16325-30
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7)Igaz LM, Kwong LK, Lee EB, et al. Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice. J Clin Invest. 2011; 121: 726-38
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8)Swarup V, Phaneuf D, Bareil C, et al. Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. Brain. 2011; 134: 2610-26
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9)Xu YF, Zhang YJ, Lin WL, et al. Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice. Mol Neurodegener. 2011; 6: 73
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10)Caccamo A, Majumder S, Oddo S. Cognitive decline typical of frontotemporal lobar degene-ration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43. Am J Pathol. 2012; 180: 293-302
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11)Cannon A, Yang B, Knight J, et al. Neuronal sensitivity to TDP-43 overexpression is depend-ent on timing of induction. Acta Neuropathol. 2012; 123: 807-23
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12)Tsuji H, Arai T, Kametani F, et al. Molecular analysis and biochemical classification of TDP-43 proteinopathy. Brain. 2012. In press
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13)Kraemer BC, Schuck T, Wheeler JM, et al. Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis. Acta Neuropathol. 2010; 119: 409-19
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14)Chiang PM, Ling J, Jeong YH, et al. Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism. Proc Natl Acad Sci U S A. 2010; 107: 16320-4
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15)Ahmed Z, Sheng H, Xu YF, et al. Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging. Am J Pathol. 2010; 177: 311-24
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16)Wils H, Kleinberger G, Pereson S, et al. Cellular ageing, increased mortality and FTLD-TDP-associated neuropathology in progranulin knock-out mice. J Pathol. 2012; 228: 67-76
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