메뉴 건너뛰기




Volumn 140, Issue 5, 2017, Pages 1447-1465

Restoring neuronal progranulin reverses deficits in a mouse model of frontotemporal dementia

Author keywords

dementia; experimental models; FTD; gene therapy; progranulin

Indexed keywords

CALCIUM CALMODULIN DEPENDENT PROTEIN KINASE II; NESTIN; PROGRANULIN; GRN PROTEIN, MOUSE; SIGNAL PEPTIDE;

EID: 85019611196     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awx060     Document Type: Article
Times cited : (61)

References (75)
  • 1
    • 77954578417 scopus 로고    scopus 로고
    • Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging
    • Ahmed Z, Sheng H, Xu YF, Lin WL, Innes AE, Gass J, 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.
    • (2010) Am J Pathol , vol.177 , pp. 311-324
    • Ahmed, Z.1    Sheng, H.2    Xu, Y.F.3    Lin, W.L.4    Innes, A.E.5    Gass, J.6
  • 2
    • 84979058578 scopus 로고    scopus 로고
    • Progranulin haploinsufficiency causes biphasic social dominance abnormalities in the tube test
    • Arrant AE, Filiano AJ, Warmus BA, Hall AM, Roberson ED. Progranulin haploinsufficiency causes biphasic social dominance abnormalities in the tube test. Genes Brain Behav 2016; 15: 588-603.
    • (2016) Genes Brain Behav , vol.15 , pp. 588-603
    • Arrant, A.E.1    Filiano, A.J.2    Warmus, B.A.3    Hall, A.M.4    Roberson, E.D.5
  • 3
    • 85019597752 scopus 로고    scopus 로고
    • Effects of exercise on progranulin levels and gliosis in progranulin-insufficient mice
    • Arrant AE, Patel AR, Roberson ED. Effects of exercise on progranulin levels and gliosis in progranulin-insufficient mice. eNeuro 2015; 2: e0061.
    • (2015) ENeuro , vol.2 , pp. e0061
    • Arrant, A.E.1    Patel, A.R.2    Roberson, E.D.3
  • 6
    • 0031854765 scopus 로고    scopus 로고
    • Granulins: The structure and function of an emerging family of growth factors
    • Bateman A, Bennett HP. Granulins: the structure and function of an emerging family of growth factors. J Endocrinol 1998; 158: 145-51.
    • (1998) J Endocrinol , vol.158 , pp. 145-151
    • Bateman, A.1    Bennett, H.P.2
  • 7
    • 75149132914 scopus 로고    scopus 로고
    • Is frontotemporal lobar degeneration a rare disorder? Evidence from a preliminary study in Brescia county, Italy
    • Borroni B, Alberici A, Grassi M, Turla M, Zanetti O, Bianchetti A, et al. Is frontotemporal lobar degeneration a rare disorder? Evidence from a preliminary study in Brescia county, Italy. J Alzheimers Dis 2010; 19: 111-16.
    • (2010) J Alzheimers Dis , vol.19 , pp. 111-116
    • Borroni, B.1    Alberici, A.2    Grassi, M.3    Turla, M.4    Zanetti, O.5    Bianchetti, A.6
  • 9
    • 4344566457 scopus 로고    scopus 로고
    • Recombinant AAV viral vectors pseudotyped with viral capsids from serotypes 1, 2, and 5 display differential efficiency and cell tropism after delivery to different regions of the central nervous system
    • Burger C, Gorbatyuk OS, Velardo MJ, Peden CS, Williams P, Zolotukhin S, et al. Recombinant AAV viral vectors pseudotyped with viral capsids from serotypes 1, 2, and 5 display differential efficiency and cell tropism after delivery to different regions of the central nervous system. Mol Ther 2004; 10: 302-17.
    • (2004) Mol Ther , vol.10 , pp. 302-317
    • Burger, C.1    Gorbatyuk, O.S.2    Velardo, M.J.3    Peden, C.S.4    Williams, P.5    Zolotukhin, S.6
  • 10
    • 79551543554 scopus 로고    scopus 로고
    • Rescue of progranulin deficiency associated with frontotemporal lobar degeneration by alkalizing reagents and inhibition of vacuolar ATPase
    • Capell A, Liebscher S, Fellerer K, Brouwers N, Willem M, Lammich S, et al. Rescue of progranulin deficiency associated with frontotemporal lobar degeneration by alkalizing reagents and inhibition of vacuolar ATPase. J Neurosci 2011; 31: 1885-94.
    • (2011) J Neurosci , vol.31 , pp. 1885-1894
    • Capell, A.1    Liebscher, S.2    Fellerer, K.3    Brouwers, N.4    Willem, M.5    Lammich, S.6
  • 11
    • 79955534069 scopus 로고    scopus 로고
    • Suberoylanilide hydroxamic acid (vorinostat) up-regulates progranulin transcription: Rational therapeutic approach to FTD
    • Cenik B, Sephton CF, Dewey CM, Xian X, Wei S, Yu K, et al. Suberoylanilide hydroxamic acid (vorinostat) up-regulates progranulin transcription: rational therapeutic approach to FTD. J Biol Chem 2011; 286: 16101-8.
    • (2011) J Biol Chem , vol.286 , pp. 16101-16108
    • Cenik, B.1    Sephton, C.F.2    Dewey, C.M.3    Xian, X.4    Wei, S.5    Yu, K.6
  • 12
    • 84866536050 scopus 로고    scopus 로고
    • Progranulin: A proteolytically processed protein at the crossroads of inflammation and neurodegeneration
    • Cenik B, Sephton CF, Kutluk Cenik B, Herz J, Yu G. Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration. J Biol Chem 2012; 287: 32298-306.
    • (2012) J Biol Chem , vol.287 , pp. 32298-32306
    • Cenik, B.1    Sephton, C.F.2    Kutluk Cenik, B.3    Herz, J.4    Yu, G.5
  • 14
    • 77957818067 scopus 로고    scopus 로고
    • Progranulin modulates zebrafish motoneuron development in vivo and rescues truncation defects associated with knockdown of Survival motor neuron 1
    • Chitramuthu BP, Baranowski DC, Kay DG, Bateman A, Bennett HP. Progranulin modulates zebrafish motoneuron development in vivo and rescues truncation defects associated with knockdown of Survival motor neuron 1. Mol Neurodegener 2010; 5: 41.
    • (2010) Mol Neurodegener , vol.5 , pp. 41
    • Chitramuthu, B.P.1    Baranowski, D.C.2    Kay, D.G.3    Bateman, A.4    Bennett, H.P.5
  • 15
    • 33746910649 scopus 로고    scopus 로고
    • Null mutations in progranulin cause ubiquitin-positive FTD linked to chromosome 17q21
    • Cruts M, Gijselinck I, van der Zee J, Engelborghs S, Wils H, Pirici D, et al. Null mutations in progranulin cause ubiquitin-positive FTD linked to chromosome 17q21. Nature 2006; 442: 920-4.
    • (2006) Nature , vol.442 , pp. 920-924
    • Cruts, M.1    Gijselinck, I.2    Van Der Zee, J.3    Engelborghs, S.4    Wils, H.5    Pirici, D.6
  • 17
    • 84881553329 scopus 로고    scopus 로고
    • The neurotrophic properties of progranulin depend on the granulin e domain but do not require sortilin binding
    • De Muynck L, Herdewyn S, Beel S, Scheveneels W, Van Den Bosch L, Robberecht W, et al. The neurotrophic properties of progranulin depend on the granulin E domain but do not require sortilin binding. Neurobiol Aging 2013; 34: 2541-7.
    • (2013) Neurobiol Aging , vol.34 , pp. 2541-2547
    • De Muynck, L.1    Herdewyn, S.2    Beel, S.3    Scheveneels, W.4    Van Den Bosch, L.5    Robberecht, W.6
  • 18
    • 84857010356 scopus 로고    scopus 로고
    • Assessing burrowing, nest construction, and hoarding in mice
    • Deacon R. Assessing burrowing, nest construction, and hoarding in mice. J Vis Exp 2012; 59: e2607.
    • (2012) J Vis Exp , vol.59 , pp. e2607
    • Deacon, R.1
  • 19
    • 61449189852 scopus 로고    scopus 로고
    • Cognitive and non-cognitive behaviors in an APPswe/PS1 bigenic model of Alzheimer's disease
    • Filali M, Lalonde R, Rivest S. Cognitive and non-cognitive behaviors in an APPswe/PS1 bigenic model of Alzheimer's disease. Genes Brain Behav 2009; 8: 143-8.
    • (2009) Genes Brain Behav , vol.8 , pp. 143-148
    • Filali, M.1    Lalonde, R.2    Rivest, S.3
  • 20
    • 84877099960 scopus 로고    scopus 로고
    • Dissociation of FTD-related deficits and neuroinflammation in progranulin haploinsufficient mice
    • Filiano AJ, Martens LH, Young AH, Warmus BA, Zhou P, Diaz-Ramirez G, et al. Dissociation of FTD-related deficits and neuroinflammation in progranulin haploinsufficient mice. J Neurosci 2013; 33: 5352-61.
    • (2013) J Neurosci , vol.33 , pp. 5352-5361
    • Filiano, A.J.1    Martens, L.H.2    Young, A.H.3    Warmus, B.A.4    Zhou, P.5    Diaz-Ramirez, G.6
  • 21
    • 64849103956 scopus 로고    scopus 로고
    • Plasma progranulin levels predict progranulin mutation status in FTD patients and asymptomatic family members
    • Finch N, Baker M, Crook R, Swanson K, Kuntz K, Surtees R, et al. Plasma progranulin levels predict progranulin mutation status in FTD patients and asymptomatic family members. Brain 2009; 132 (Pt 3): 583-91.
    • (2009) Brain , vol.132 , pp. 583-591
    • Finch, N.1    Baker, M.2    Crook, R.3    Swanson, K.4    Kuntz, K.5    Surtees, R.6
  • 22
    • 84879114551 scopus 로고    scopus 로고
    • Progranulin gene (GRN) promoter methylation is increased in patients with sporadic frontotemporal lobar degeneration
    • Galimberti D, D'Addario C, Dell'osso B, Fenoglio C, Marcone A, Cerami C, et al. Progranulin gene (GRN) promoter methylation is increased in patients with sporadic frontotemporal lobar degeneration. Neurol Sci 2013; 34: 899-903.
    • (2013) Neurol Sci , vol.34 , pp. 899-903
    • Galimberti, D.1    D'Addario, C.2    Dell'Osso, B.3    Fenoglio, C.4    Marcone, A.5    Cerami, C.6
  • 23
    • 33749568019 scopus 로고    scopus 로고
    • Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration
    • Gass J, Cannon A, Mackenzie IR, Boeve B, Baker M, Adamson J, et al. Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet 2006; 15: 2988-3001.
    • (2006) Hum Mol Genet , vol.15 , pp. 2988-3001
    • Gass, J.1    Cannon, A.2    Mackenzie, I.R.3    Boeve, B.4    Baker, M.5    Adamson, J.6
  • 25
    • 81955160693 scopus 로고    scopus 로고
    • Core features of FTD recapitulated in progranulin knockout mice
    • Ghoshal N, Dearborn JT, Wozniak DF, Cairns NJ. Core features of FTD recapitulated in progranulin knockout mice. Neurobiol Dis 2012; 45: 395-408.
    • (2012) Neurobiol Dis , vol.45 , pp. 395-408
    • Ghoshal, N.1    Dearborn, J.T.2    Wozniak, D.F.3    Cairns, N.J.4
  • 26
    • 84947739144 scopus 로고    scopus 로고
    • The role of the medial prefrontal cortex in the conditioning and extinction of fear
    • Giustino TF, Maren S. The role of the medial prefrontal cortex in the conditioning and extinction of fear. Front Behav Neurosci 2015; 9: 298.
    • (2015) Front Behav Neurosci , vol.9 , pp. 298
    • Giustino, T.F.1    Maren, S.2
  • 27
    • 84901653647 scopus 로고    scopus 로고
    • Common pathobiochemical hallmarks of progranulin - Associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis
    • Gotzl JK, Mori K, Damme M, Fellerer K, Tahirovic S, Kleinberger G, et al. Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis. Acta Neuropathol 2014; 127: 845-60.
    • (2014) Acta Neuropathol , vol.127 , pp. 845-860
    • Gotzl, J.K.1    Mori, K.2    Damme, M.3    Fellerer, K.4    Tahirovic, S.5    Kleinberger, G.6
  • 30
    • 44949087532 scopus 로고    scopus 로고
    • Fear conditioning in frontotemporal lobar degeneration and Alzheimer's disease
    • Hoefer M, Allison SC, Schauer GF, Neuhaus JM, Hall J, Dang JN, et al. Fear conditioning in frontotemporal lobar degeneration and Alzheimer's disease. Brain 2008; 131 (Pt 6): 1646-57.
    • (2008) Brain , vol.131 , pp. 1646-1657
    • Hoefer, M.1    Allison, S.C.2    Schauer, G.F.3    Neuhaus, J.M.4    Hall, J.5    Dang, J.N.6
  • 31
    • 84975894236 scopus 로고    scopus 로고
    • Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: A novel therapeutic lead to treat FTD
    • Holler CJ, Taylor G, McEachin ZT, Deng Q, Watkins WJ, Hudson K, et al. Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: a novel therapeutic lead to treat FTD. Mol Neurodegener 2016; 11: 46.
    • (2016) Mol Neurodegener , vol.11 , pp. 46
    • Holler, C.J.1    Taylor, G.2    McEachin, Z.T.3    Deng, Q.4    Watkins, W.J.5    Hudson, K.6
  • 32
    • 78449286213 scopus 로고    scopus 로고
    • Sortilin-mediated endocytosis determines levels of the FTD protein, progranulin
    • Hu F, Padukkavidana T, Vægter CB, Brady OA, Zheng Y, Mackenzie IR, et al. Sortilin-mediated endocytosis determines levels of the FTD protein, progranulin. Neuron 2010; 68: 654-67.
    • (2010) Neuron , vol.68 , pp. 654-667
    • Hu, F.1    Padukkavidana, T.2    Vægter, C.B.3    Brady, O.A.4    Zheng, Y.5    Mackenzie, I.R.6
  • 33
    • 33744991083 scopus 로고    scopus 로고
    • Brief uncontrollable stress causes dendritic retraction in infralimbic cortex and resistance to fear extinction in mice
    • Izquierdo A, Wellman CL, Holmes A. Brief uncontrollable stress causes dendritic retraction in infralimbic cortex and resistance to fear extinction in mice. J Neurosci 2006; 26: 5733-8.
    • (2006) J Neurosci , vol.26 , pp. 5733-5738
    • Izquierdo, A.1    Wellman, C.L.2    Holmes, A.3
  • 34
    • 84889794517 scopus 로고    scopus 로고
    • Progranulin deficiency promotes post-ischemic blood-brain barrier disruption
    • Jackman K, Kahles T, Lane D, Garcia-Bonilla L, Abe T, Capone C, et al. Progranulin deficiency promotes post-ischemic blood-brain barrier disruption. J Neurosci 2013; 33: 19579-89.
    • (2013) J Neurosci , vol.33 , pp. 19579-19589
    • Jackman, K.1    Kahles, T.2    Lane, D.3    Garcia-Bonilla, L.4    Abe, T.5    Capone, C.6
  • 35
    • 84903275882 scopus 로고    scopus 로고
    • Frontotemporal lobar degeneration: A clinical approach
    • Karageorgiou E, Miller BL. Frontotemporal lobar degeneration: a clinical approach. Semin Neurol 2014; 34: 189-201.
    • (2014) Semin Neurol , vol.34 , pp. 189-201
    • Karageorgiou, E.1    Miller, B.L.2
  • 36
    • 35448929818 scopus 로고    scopus 로고
    • Alteration of behavioural phenotype in mice by targeted disruption of the progranulin gene
    • Kayasuga Y, Chiba S, Suzuki M, Kikusui T, Matsuwaki T, Yamanouchi K, et al. Alteration of behavioural phenotype in mice by targeted disruption of the progranulin gene. Behav Brain Res 2007; 185: 110-18.
    • (2007) Behav Brain Res , vol.185 , pp. 110-118
    • Kayasuga, Y.1    Chiba, S.2    Suzuki, M.3    Kikusui, T.4    Matsuwaki, T.5    Yamanouchi, K.6
  • 37
    • 84872497448 scopus 로고    scopus 로고
    • Mechanisms of granulin deficiency: Lessons from cellular and animal models
    • Kleinberger G, Capell A, Haass C, Van Broeckhoven C. Mechanisms of granulin deficiency: lessons from cellular and animal models. Mol Neurobiol 2013; 47: 337-60.
    • (2013) Mol Neurobiol , vol.47 , pp. 337-360
    • Kleinberger, G.1    Capell, A.2    Haass, C.3    Van Broeckhoven, C.4
  • 38
    • 79955830791 scopus 로고    scopus 로고
    • Rs5848 variant of progranulin gene is a risk of Alzheimer's disease in the Taiwanese population
    • Lee MJ, Chen TF, Cheng TW, Chiu MJ. rs5848 variant of progranulin gene is a risk of Alzheimer's disease in the Taiwanese population. Neurodegener Dis 2011; 8: 216-20.
    • (2011) Neurodegener Dis , vol.8 , pp. 216-220
    • Lee, M.J.1    Chen, T.F.2    Cheng, T.W.3    Chiu, M.J.4
  • 39
    • 84894298786 scopus 로고    scopus 로고
    • Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency
    • Lee WC, Almeida S, Prudencio M, Caulfield TR, Zhang YJ, Tay WM, et al. Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency. Hum Mol Genet 2014; 23: 1467-78.
    • (2014) Hum Mol Genet , vol.23 , pp. 1467-1478
    • Lee, W.C.1    Almeida, S.2    Prudencio, M.3    Caulfield, T.R.4    Zhang, Y.J.5    Tay, W.M.6
  • 41
    • 84922887918 scopus 로고    scopus 로고
    • Seizure resistance without parkinsonism in aged mice after tau reduction
    • Li Z, Hall AM, Kelinske M, Roberson ED. Seizure resistance without parkinsonism in aged mice after tau reduction. Neurobiol Aging 2014; 35: 2617-24.
    • (2014) Neurobiol Aging , vol.35 , pp. 2617-2624
    • Li, Z.1    Hall, A.M.2    Kelinske, M.3    Roberson, E.D.4
  • 42
    • 84963969178 scopus 로고    scopus 로고
    • Progranulin deficiency promotes circuit-specific synaptic pruning by microglia via complement activation
    • Lui H, Zhang J, Makinson SR, Cahill MK, Kelley KW, Huang HY, et al. Progranulin deficiency promotes circuit-specific synaptic pruning by microglia via complement activation. Cell 2016; 165: 921-35.
    • (2016) Cell , vol.165 , pp. 921-935
    • Lui, H.1    Zhang, J.2    Makinson, S.R.3    Cahill, M.K.4    Kelley, K.W.5    Huang, H.Y.6
  • 43
    • 77649187519 scopus 로고    scopus 로고
    • Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: An update
    • Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol 2010; 119: 1-4.
    • (2010) Acta Neuropathol , vol.119 , pp. 1-4
    • Mackenzie, I.R.1    Neumann, M.2    Bigio, E.H.3    Cairns, N.J.4    Alafuzoff, I.5    Kril, J.6
  • 44
    • 84868623124 scopus 로고    scopus 로고
    • Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury
    • Martens LH, Zhang J, Barmada SJ, Zhou P, Kamiya S, Sun B, et al. Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury. J Clin Invest 2012; 122: 3955-9.
    • (2012) J Clin Invest , vol.122 , pp. 3955-3959
    • Martens, L.H.1    Zhang, J.2    Barmada, S.J.3    Zhou, P.4    Kamiya, S.5    Sun, B.6
  • 45
    • 84855430348 scopus 로고    scopus 로고
    • PGRN is a key adipokine mediating high fat diet-induced insulin resistance and obesity through IL-6 in adipose tissue
    • Matsubara T, Mita A, Minami K, Hosooka T, Kitazawa S, Takahashi K, et al. PGRN is a key adipokine mediating high fat diet-induced insulin resistance and obesity through IL-6 in adipose tissue. Cell Metab 2012; 15: 38-50.
    • (2012) Cell Metab , vol.15 , pp. 38-50
    • Matsubara, T.1    Mita, A.2    Minami, K.3    Hosooka, T.4    Kitazawa, S.5    Takahashi, K.6
  • 46
    • 84921968828 scopus 로고    scopus 로고
    • Progranulin protects against amyloid b deposition and toxicity in Alzheimer's disease mouse models
    • Minami SS, Min SW, Krabbe G, Wang C, Zhou Y, Asgarov R, et al. Progranulin protects against amyloid b deposition and toxicity in Alzheimer's disease mouse models. Nat Med 2014; 20: 1157-64.
    • (2014) Nat Med , vol.20 , pp. 1157-1164
    • Minami, S.S.1    Min, S.W.2    Krabbe, G.3    Wang, C.4    Zhou, Y.5    Asgarov, R.6
  • 47
    • 58049221032 scopus 로고    scopus 로고
    • Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury
    • Moisse K, Volkening K, Leystra-Lantz C, Welch I, Hill T, Strong MJ. Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury. Brain Res 2009; 1249: 202-11.
    • (2009) Brain Res , vol.1249 , pp. 202-211
    • Moisse, K.1    Volkening, K.2    Leystra-Lantz, C.3    Welch, I.4    Hill, T.5    Strong, M.J.6
  • 50
    • 84934441665 scopus 로고    scopus 로고
    • Quantifying biomarkers of cognitive dysfunction and neuronal network hyperexcitability in mouse models of Alzheimer's disease: Depletion of calcium-dependent proteins and inhibitory hippocampal remodeling
    • Palop JJ, Mucke L, Roberson ED. Quantifying biomarkers of cognitive dysfunction and neuronal network hyperexcitability in mouse models of Alzheimer's disease: depletion of calcium-dependent proteins and inhibitory hippocampal remodeling. Methods Mol Biol 2011; 670: 245-62.
    • (2011) Methods Mol Biol , vol.670 , pp. 245-262
    • Palop, J.J.1    Mucke, L.2    Roberson, E.D.3
  • 54
    • 80052938441 scopus 로고    scopus 로고
    • Sensitivity of revised diagnostic criteria for the behavioural variant of FTD
    • Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of FTD. Brain 2011; 134: 2456-77.
    • (2011) Brain , vol.134 , pp. 2456-2477
    • Rascovsky, K.1    Hodges, J.R.2    Knopman, D.3    Mendez, M.F.4    Kramer, J.H.5    Neuhaus, J.6
  • 56
    • 0041320789 scopus 로고    scopus 로고
    • FTD in the Netherlands: Patient characteristics and prevalence estimates from a population-based study
    • Rosso SM, Donker Kaat L, Baks T, Joosse M, de Koning I, Pijnenburg Y, et al. FTD in the Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain 2003; 126 (Pt 9): 2016-22.
    • (2003) Brain , vol.126 , pp. 2016-2022
    • Rosso, S.M.1    Donker Kaat, L.2    Baks, T.3    Joosse, M.4    De Koning, I.5    Pijnenburg, Y.6
  • 57
  • 59
    • 38349173569 scopus 로고    scopus 로고
    • Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion
    • Shankaran SS, Capell A, Hruscha AT, Fellerer K, Neumann M, Schmid B, et al. Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion. J Biol Chem 2008; 283: 1744-53.
    • (2008) J Biol Chem , vol.283 , pp. 1744-1753
    • Shankaran, S.S.1    Capell, A.2    Hruscha, A.T.3    Fellerer, K.4    Neumann, M.5    Schmid, B.6
  • 61
    • 33847699408 scopus 로고    scopus 로고
    • Dopaminergic neuron loss and up-regulation of chaperone protein mRNA induced by targeted over-expression of alpha-synuclein in mouse substantia nigra
    • St Martin JL, Klucken J, Outeiro TF, Nguyen P, Keller-McGandy C, Cantuti-Castelvetri I, et al. Dopaminergic neuron loss and up-regulation of chaperone protein mRNA induced by targeted over-expression of alpha-synuclein in mouse substantia nigra. J Neurochem 2007; 100: 1449-57.
    • (2007) J Neurochem , vol.100 , pp. 1449-1457
    • St Martin, J.L.1    Klucken, J.2    Outeiro, T.F.3    Nguyen, P.4    Keller-McGandy, C.5    Cantuti-Castelvetri, I.6
  • 62
    • 84925935972 scopus 로고    scopus 로고
    • Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice
    • Tanaka Y, Chambers JK, Matsuwaki T, Yamanouchi K, Nishihara M. Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice. Acta Neuropathol Commun 2014; 2: 78.
    • (2014) Acta Neuropathol Commun , vol.2 , pp. 78
    • Tanaka, Y.1    Chambers, J.K.2    Matsuwaki, T.3    Yamanouchi, K.4    Nishihara, M.5
  • 63
    • 84881220459 scopus 로고    scopus 로고
    • Increased lysosomal biogenesis in activated microglia and exacerbated neuronal damage after traumatic brain injury in progranulin-deficient mice
    • Tanaka Y, Matsuwaki T, Yamanouchi K, Nishihara M. Increased lysosomal biogenesis in activated microglia and exacerbated neuronal damage after traumatic brain injury in progranulin-deficient mice. Neuroscience 2013; 250: 8-19.
    • (2013) Neuroscience , vol.250 , pp. 8-19
    • Tanaka, Y.1    Matsuwaki, T.2    Yamanouchi, K.3    Nishihara, M.4
  • 64
    • 0032842478 scopus 로고    scopus 로고
    • Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety
    • Tronche F, Kellendonk C, Kretz O, Gass P, Anlag K, Orban PC, et al. Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety. Nat Genet 1999; 23: 99-103.
    • (1999) Nat Genet , vol.23 , pp. 99-103
    • Tronche, F.1    Kellendonk, C.2    Kretz, O.3    Gass, P.4    Anlag, K.5    Orban, P.C.6
  • 66
    • 42049087853 scopus 로고    scopus 로고
    • Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival
    • Van Damme P, Van Hoecke A, Lambrechts D, Vanacker P, Bogaert E, van Swieten J, et al. Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival. J Cell Biol 2008; 181: 37-41.
    • (2008) J Cell Biol , vol.181 , pp. 37-41
    • Van Damme, P.1    Van Hoecke, A.2    Lambrechts, D.3    Vanacker, P.4    Bogaert, E.5    Van Swieten, J.6
  • 67
    • 84900547297 scopus 로고    scopus 로고
    • Progranulin gene delivery protects dopaminergic neurons in a mouse model of Parkinson's disease
    • Van Kampen JM, Baranowski D, Kay DG. Progranulin gene delivery protects dopaminergic neurons in a mouse model of Parkinson's disease. PLoS One 2014; 9: e97032.
    • (2014) PLoS One , vol.9 , pp. e97032
    • Van Kampen, J.M.1    Baranowski, D.2    Kay, D.G.3
  • 68
    • 80555150639 scopus 로고    scopus 로고
    • Bidirectional control of social hierarchy by synaptic efficacy in medial prefrontal cortex
    • Wang F, Zhu J, Zhu H, Zhang Q, Lin Z, Hu H. Bidirectional control of social hierarchy by synaptic efficacy in medial prefrontal cortex. Science 2011; 334: 693-7.
    • (2011) Science , vol.334 , pp. 693-697
    • Wang, F.1    Zhu, J.2    Zhu, H.3    Zhang, Q.4    Lin, Z.5    Hu, H.6
  • 69
    • 75949130374 scopus 로고    scopus 로고
    • Pathogenic cysteine mutations affect progranulin function and production of mature granulins
    • Wang J, Van Damme P, Cruchaga C, Gitcho MA, Vidal JM, Seijo-Martinez M, et al. Pathogenic cysteine mutations affect progranulin function and production of mature granulins. J Neurochem 2010; 112: 1305-15.
    • (2010) J Neurochem , vol.112 , pp. 1305-1315
    • Wang, J.1    Van Damme, P.2    Cruchaga, C.3    Gitcho, M.A.4    Vidal, J.M.5    Seijo-Martinez, M.6
  • 70
    • 84864744790 scopus 로고    scopus 로고
    • Cellular ageing, increased mortality and FTLD-TDP - Associated neuropathology in progranulin knockout mice
    • Wils H, Kleinberger G, Pereson S, Janssens J, Capell A, Van Dam D, et al. Cellular ageing, increased mortality and FTLD-TDP-associated neuropathology in progranulin knockout mice. J Pathol 2012; 228: 67-76.
    • (2012) J Pathol , vol.228 , pp. 67-76
    • Wils, H.1    Kleinberger, G.2    Pereson, S.3    Janssens, J.4    Capell, A.5    Van Dam, D.6
  • 71
    • 84906932165 scopus 로고    scopus 로고
    • Neuronal representation of working memory in the medial prefrontal cortex of rats
    • Yang ST, Shi Y, Wang Q, Peng JY, Li BM. Neuronal representation of working memory in the medial prefrontal cortex of rats. Mol Brain 2014; 7: 61.
    • (2014) Mol Brain , vol.7 , pp. 61
    • Yang, S.T.1    Shi, Y.2    Wang, Q.3    Peng, J.Y.4    Li, B.M.5
  • 72
    • 76149118401 scopus 로고    scopus 로고
    • Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice
    • Yin F, Banerjee R, Thomas B, Zhou P, Qian L, Jia T, et al. Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice. J Exp Med 2010a; 207: 117-28.
    • (2010) J Exp Med , vol.207 , pp. 117-128
    • Yin, F.1    Banerjee, R.2    Thomas, B.3    Zhou, P.4    Qian, L.5    Jia, T.6
  • 73
    • 78149296002 scopus 로고    scopus 로고
    • Behavioral deficits and progressive neuropathology in progranulin-deficient mice: A mouse model of FTD
    • Yin F, Dumont M, Banerjee R, Ma Y, Li H, Lin MT, et al. Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of FTD. FASEB J 2010b; 24: 4639-47.
    • (2010) FASEB J , vol.24 , pp. 4639-4647
    • Yin, F.1    Dumont, M.2    Banerjee, R.3    Ma, Y.4    Li, H.5    Lin, M.T.6
  • 74
    • 84906901689 scopus 로고    scopus 로고
    • An RNA-sequencing transcriptome and splicing database of glia, neurons, and vascular cells of the cerebral cortex
    • Zhang Y, Chen K, Sloan SA, Bennett ML, Scholze AR, O'Keeffe S, et al. An RNA-sequencing transcriptome and splicing database of glia, neurons, and vascular cells of the cerebral cortex. J Neurosci 2014; 34: 11929-47.
    • (2014) J Neurosci , vol.34 , pp. 11929-11947
    • Zhang, Y.1    Chen, K.2    Sloan, S.A.3    Bennett, M.L.4    Scholze, A.R.5    O'Keeffe, S.6
  • 75
    • 0028057681 scopus 로고
    • Independent regulatory elements in the nestin gene direct transgene expression to neural stem cells or muscle precursors
    • Zimmerman L, Lendahl U, Cunningham M, McKay R, Parr B, Gavin B, et al. Independent regulatory elements in the nestin gene direct transgene expression to neural stem cells or muscle precursors. Neuron 1994; 12: 11-24.
    • (1994) Neuron , vol.12 , pp. 11-24
    • Zimmerman, L.1    Lendahl, U.2    Cunningham, M.3    McKay, R.4    Parr, B.5    Gavin, B.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.