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Volumn 136, Issue 4, 2013, Pages 1083-1101

Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis

Author keywords

axonal damage; neurodegeneration; neuroinflammation; neuronal ceroid lipofuscinosis; T lymphocytes

Indexed keywords

CD8 ANTIGEN; PALMITOYL PROTEIN THIOESTERASE; PALMITOYL PROTEIN THIOESTERASE 1; UNCLASSIFIED DRUG;

EID: 84876059193     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awt020     Document Type: Article
Times cited : (52)

References (44)
  • 1
    • 34548843340 scopus 로고    scopus 로고
    • Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction
    • Ahtiainen L, Kolikova J, Mutka AL, Luiro K, Gentile M, Ikonen E, et al. Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction. Neurobiol Dis 2007; 28: 52-64.
    • (2007) Neurobiol Dis , vol.28 , pp. 52-64
    • Ahtiainen, L.1    Kolikova, J.2    Mutka, A.L.3    Luiro, K.4    Gentile, M.5    Ikonen, E.6
  • 2
    • 62549095466 scopus 로고    scopus 로고
    • Distinct roles of transcription factors brn3a and brn3b in controlling the development, morphology, and function of retinal ganglion cells
    • Badea TC, Cahill H, Ecker J, Hattar S, Nathans J. Distinct roles of transcription factors brn3a and brn3b in controlling the development, morphology, and function of retinal ganglion cells. Neuron 2009; 61: 852-64.
    • (2009) Neuron , vol.61 , pp. 852-864
    • Badea, T.C.1    Cahill, H.2    Ecker, J.3    Hattar, S.4    Nathans, J.5
  • 3
    • 40849088250 scopus 로고    scopus 로고
    • Progressive ganglion cell degeneration precedes neuronal loss in a mouse model of glaucoma
    • Buckingham BP, Inman DM, Lambert W, Oglesby E, Calkins DJ, Steele MR, et al. Progressive ganglion cell degeneration precedes neuronal loss in a mouse model of glaucoma. J Neurosci 2008; 28: 2735-44.
    • (2008) J Neurosci , vol.28 , pp. 2735-2744
    • Buckingham, B.P.1    Inman, D.M.2    Lambert, W.3    Oglesby, E.4    Calkins, D.J.5    Steele, M.R.6
  • 4
    • 43649091527 scopus 로고    scopus 로고
    • Moving towards therapies for juvenile Batten disease?
    • Cooper JD. Moving towards therapies for juvenile Batten disease? Exp Neurol 2008; 211: 329-31.
    • (2008) Exp Neurol , vol.211 , pp. 329-331
    • Cooper, J.D.1
  • 5
    • 78649747041 scopus 로고    scopus 로고
    • The neuronal ceroid lipofuscinoses: The same, but different?
    • Cooper JD. The neuronal ceroid lipofuscinoses: the same, but different? Biochem Soc Trans 2010; 38: 1448-52.
    • (2010) Biochem Soc Trans , vol.38 , pp. 1448-1452
    • Cooper, J.D.1
  • 6
    • 0019493368 scopus 로고
    • Ganglion cell distribution in the retina of the mouse
    • Drager UC, Olsen JF. Ganglion cell distribution in the retina of the mouse. Invest Ophthalmol Vis Sci 1981; 20: 285-93.
    • (1981) Invest Ophthalmol Vis Sci , vol.20 , pp. 285-293
    • Drager, U.C.1    Olsen, J.F.2
  • 7
    • 77950188666 scopus 로고    scopus 로고
    • Microglial Cx3cr1 knockout prevents neuron loss in a mouse model of Alzheimers disease
    • Fuhrmann M, Bittner T, Jung CK, Burgold S, Page RM, Mitteregger G, et al. Microglial Cx3cr1 knockout prevents neuron loss in a mouse model of Alzheimers disease. Nat Neurosci 2010; 13: 411-3.
    • (2010) Nat Neurosci , vol.13 , pp. 411-413
    • Fuhrmann, M.1    Bittner, T.2    Jung, C.K.3    Burgold, S.4    Page, R.M.5    Mitteregger, G.6
  • 10
  • 11
    • 32944454332 scopus 로고    scopus 로고
    • CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis
    • Griffey MA, Wozniak D, Wong M, Bible E, Johnson K, Rothman SM, et al. CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis. Mol Ther 2006; 13: 538-47.
    • (2006) Mol Ther , vol.13 , pp. 538-547
    • Griffey, M.A.1    Wozniak, D.2    Wong, M.3    Bible, E.4    Johnson, K.5    Rothman, S.M.6
  • 12
    • 84856746699 scopus 로고    scopus 로고
    • Colonystimulating factor-1 mediates macrophage-related neural damage in a model for Charcot-Marie-Tooth disease type 1X
    • Groh J, Weis J, Zieger H, Stanley ER, Heuer H, Martini R. Colonystimulating factor-1 mediates macrophage-related neural damage in a model for Charcot-Marie-Tooth disease type 1X. Brain 2012; 135: 88-104.
    • (2012) Brain , vol.135 , pp. 88-104
    • Groh, J.1    Weis, J.2    Zieger, H.3    Stanley, E.R.4    Heuer, H.5    Martini, R.6
  • 14
    • 33750986182 scopus 로고    scopus 로고
    • The neuronal ceroid-lipofuscinoses: From past to present
    • Haltia M. The neuronal ceroid-lipofuscinoses: from past to present. Biochim Biophys Acta 2006; 1762: 850-6.
    • (2006) Biochim Biophys Acta , vol.1762 , pp. 850-856
    • Haltia, M.1
  • 15
    • 47749106032 scopus 로고    scopus 로고
    • Origin of CD11b + macrophage-like cells in the CNS of PLP-overexpressing mice: Low influx of haematogenous macrophages and unchanged blood-brain-barrier in the optic nerve
    • Ip CW, Kohl B, Kleinschnitz C, Reuss B, Nave KA, Kroner A, et al. Origin of CD11b + macrophage-like cells in the CNS of PLP-overexpressing mice: low influx of haematogenous macrophages and unchanged blood-brain-barrier in the optic nerve. Mol Cell Neurosci 2008; 38: 489-94.
    • (2008) Mol Cell Neurosci , vol.38 , pp. 489-494
    • Ip, C.W.1    Kohl, B.2    Kleinschnitz, C.3    Reuss, B.4    Nave, K.A.5    Kroner, A.6
  • 16
    • 33748138567 scopus 로고    scopus 로고
    • Immune cells contribute to myelin degeneration and axonopathic changes in mice overexpressing proteolipid protein in oligodendrocytes
    • Ip CW, Kroner A, Bendszus M, Leder C, Kobsar I, Fischer S, et al. Immune cells contribute to myelin degeneration and axonopathic changes in mice overexpressing proteolipid protein in oligodendrocytes. J Neurosci 2006; 26: 8206-16.
    • (2006) J Neurosci , vol.26 , pp. 8206-8216
    • Ip, C.W.1    Kroner, A.2    Bendszus, M.3    Leder, C.4    Kobsar, I.5    Fischer, S.6
  • 17
    • 84864705710 scopus 로고    scopus 로고
    • Neuroinflammation by cytotoxic T-lymphocytes impairs retrograde axonal transport in an oligodendrocyte mutant mouse
    • Ip CW, Kroner A, Groh J, Huber M, Klein D, Spahn I, et al. Neuroinflammation by cytotoxic T-lymphocytes impairs retrograde axonal transport in an oligodendrocyte mutant mouse. PLoS One 2012; 7: e42554.
    • (2012) PLoS One , vol.7
    • Ip, C.W.1    Kroner, A.2    Groh, J.3    Huber, M.4    Klein, D.5    Spahn, I.6
  • 18
    • 62949195147 scopus 로고    scopus 로고
    • Neuronal ceroid lipofuscinoses
    • Jalanko A, Braulke T. Neuronal ceroid lipofuscinoses. Biochim Biophys Acta 2009; 1793: 697-709.
    • (2009) Biochim Biophys Acta , vol.1793 , pp. 697-709
    • Jalanko, A.1    Braulke, T.2
  • 19
    • 33751349970 scopus 로고    scopus 로고
    • Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis
    • Kielar C, Maddox L, Bible E, Pontikis CC, Macauley SL, Griffey MA, et al. Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis 2007; 25: 150-62.
    • (2007) Neurobiol Dis , vol.25 , pp. 150-162
    • Kielar, C.1    Maddox, L.2    Bible, E.3    Pontikis, C.C.4    Macauley, S.L.5    Griffey, M.A.6
  • 20
    • 70349997680 scopus 로고    scopus 로고
    • Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease
    • Kielar C, Wishart TM, Palmer A, Dihanich S, Wong AM, Macauley SL, et al. Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease. Hum Mol Genet 2009; 18: 4066-80.
    • (2009) Hum Mol Genet , vol.18 , pp. 4066-4080
    • Kielar, C.1    Wishart, T.M.2    Palmer, A.3    Dihanich, S.4    Wong, A.M.5    Macauley, S.L.6
  • 21
    • 51349089035 scopus 로고    scopus 로고
    • Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice
    • Kim SJ, Zhang Z, Sarkar C, Tsai PC, Lee YC, Dye L, et al. Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice. J Clin Invest 2008; 118: 3075-86.
    • (2008) J Clin Invest , vol.118 , pp. 3075-3086
    • Kim, S.J.1    Zhang, Z.2    Sarkar, C.3    Tsai, P.C.4    Lee, Y.C.5    Dye, L.6
  • 22
    • 77749264210 scopus 로고    scopus 로고
    • MCP-1/CCL2 modifies axon properties in a PMP22-overexpressing mouse model for Charcot-Marie-Tooth 1A neuropathy
    • Kohl B, Fischer S, Groh J, Wessig C, Martini R. MCP-1/CCL2 modifies axon properties in a PMP22-overexpressing mouse model for Charcot-Marie-Tooth 1A neuropathy. Am J Pathol 2010; 176: 1390-9.
    • (2010) Am J Pathol , vol.176 , pp. 1390-1399
    • Kohl, B.1    Fischer, S.2    Groh, J.3    Wessig, C.4    Martini, R.5
  • 23
    • 0027225696 scopus 로고
    • Human forms of neuronal ceroid-lipofuscinosis (Batten disease): Consensus on diagnostic criteria, Hamburg
    • Kohlschutter A, Gardiner RM, Goebel HH. Human forms of neuronal ceroid-lipofuscinosis (Batten disease): consensus on diagnostic criteria, Hamburg 1992. J Inherit Metab Dis 1993; 16: 241-4.
    • (1992) J Inherit Metab Dis , vol.16 , pp. 241-244
    • Kohlschutter, A.1    Gardiner, R.M.2    Goebel, H.H.3
  • 24
    • 76149091902 scopus 로고    scopus 로고
    • Ectopic T-cell specificity and absence of perforin and granzyme B alleviate neural damage in oligodendrocyte mutant mice
    • Kroner A, Ip CW, Thalhammer J, Nave KA, Martini R. Ectopic T-cell specificity and absence of perforin and granzyme B alleviate neural damage in oligodendrocyte mutant mice. Am J Pathol 2010; 176: 549-55.
    • (2010) Am J Pathol , vol.176 , pp. 549-555
    • Kroner, A.1    Ip, C.W.2    Thalhammer, J.3    Nave, K.A.4    Martini, R.5
  • 25
    • 84868535759 scopus 로고    scopus 로고
    • Progressive multiple sclerosis: Pathology and pathogenesis
    • Lassmann H, van Horssen J, Mahad D. Progressive multiple sclerosis: pathology and pathogenesis. Nat Rev Neurol 2012; 8: 647-56.
    • (2012) Nat Rev Neurol , vol.8 , pp. 647-656
    • Lassmann, H.1    Van Horssen, J.2    Mahad, D.3
  • 26
    • 35449001021 scopus 로고    scopus 로고
    • Clonal expansions of pathogenic CD8 + effector cells in the CNS of myelin mutant mice
    • Leder C, Schwab N, Ip CW, Kroner A, Nave KA, Dornmair K, et al. Clonal expansions of pathogenic CD8 + effector cells in the CNS of myelin mutant mice. Mol Cell Neurosci 2007; 36: 416-24.
    • (2007) Mol Cell Neurosci , vol.36 , pp. 416-424
    • Leder, C.1    Schwab, N.2    Ip, C.W.3    Kroner, A.4    Nave, K.A.5    Dornmair, K.6
  • 27
    • 80054910077 scopus 로고    scopus 로고
    • The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis
    • Macauley SL, Pekny M, Sands MS. The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis. J Neurosci 2011; 31: 15575-85.
    • (2011) J Neurosci , vol.31 , pp. 15575-15585
    • Macauley, S.L.1    Pekny, M.2    Sands, M.S.3
  • 29
    • 37549057968 scopus 로고    scopus 로고
    • Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: A model of human congenital neuronal ceroidlipofuscinosis
    • Partanen S, Haapanen A, Kielar C, Pontikis C, Alexander N, Inkinen T, et al. Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: a model of human congenital neuronal ceroidlipofuscinosis. J Neuropathol Exp Neurol 2008; 67: 16-29.
    • (2008) J Neuropathol Exp Neurol , vol.67 , pp. 16-29
    • Partanen, S.1    Haapanen, A.2    Kielar, C.3    Pontikis, C.4    Alexander, N.5    Inkinen, T.6
  • 30
    • 0034237482 scopus 로고    scopus 로고
    • Behavioral assessment of visual acuity in mice and rats
    • Prusky GT, West PW, Douglas RM. Behavioral assessment of visual acuity in mice and rats. Vision Res 2000; 40: 2201-9.
    • (2000) Vision Res , vol.40 , pp. 2201-2209
    • Prusky, G.T.1    West, P.W.2    Douglas, R.M.3
  • 31
    • 0025817991 scopus 로고
    • Normal development and function of CD8 + cells but markedly decreased helper cell activity in mice lacking CD4
    • Rahemtulla A, Fung-Leung WP, Schilham MW, Kundig TM, Sambhara SR, Narendran A, et al. Normal development and function of CD8 + cells but markedly decreased helper cell activity in mice lacking CD4. Nature 1991; 353: 180-4.
    • (1991) Nature , vol.353 , pp. 180-184
    • Rahemtulla, A.1    Fung-Leung, W.P.2    Schilham, M.W.3    Kundig, T.M.4    Sambhara, S.R.5    Narendran, A.6
  • 32
    • 54849432622 scopus 로고    scopus 로고
    • RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosis
    • Saha A, Kim SJ, Zhang Z, Lee YC, Sarkar C, Tsai PC, et al. RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosis. FEBS Lett 2008; 582: 3823-31.
    • (2008) FEBS Lett , vol.582 , pp. 3823-3831
    • Saha, A.1    Kim, S.J.2    Zhang, Z.3    Lee, Y.C.4    Sarkar, C.5    Tsai, P.C.6
  • 33
    • 84860437194 scopus 로고    scopus 로고
    • The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: Amelioration by resveratrol
    • Saha A, Sarkar C, Singh SP, Zhang Z, Munasinghe J, Peng S, et al. The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol. Hum Mol Genet 2012; 21: 2233-44.
    • (2012) Hum Mol Genet , vol.21 , pp. 2233-2244
    • Saha, A.1    Sarkar, C.2    Singh, S.P.3    Zhang, Z.4    Munasinghe, J.5    Peng, S.6
  • 34
    • 0015595210 scopus 로고
    • Infantile type of so-called neuronal ceroid-lipofuscinosis. rfvn1 1. A clinical study of 15 patients
    • Santavuori P, Haltia M, Rapola J, Raitta C. Infantile type of so-called neuronal ceroid-lipofuscinosis. 1. A clinical study of 15 patients. J Neurol Sci 1973; 18: 257-67.
    • (1973) J Neurol Sci , vol.18 , pp. 257-267
    • Santavuori, P.1    Haltia, M.2    Rapola, J.3    Raitta, C.4
  • 35
    • 0016174506 scopus 로고
    • Infantile type of so-called neuronal ceroid-lipofuscinosis
    • Santavuori P, Haltia M, Rapola J. Infantile type of so-called neuronal ceroid-lipofuscinosis. Dev Med Child Neurol 1974; 16: 644-53.
    • (1974) Dev Med Child Neurol , vol.16 , pp. 644-653
    • Santavuori, P.1    Haltia, M.2    Rapola, J.3
  • 36
    • 33344469599 scopus 로고    scopus 로고
    • You say lipofuscin, we say ceroid: Defining autofluorescent storage material
    • Seehafer SS, Pearce DA. You say lipofuscin, we say ceroid: defining autofluorescent storage material. Neurobiol Aging 2006; 27: 576-88.
    • (2006) Neurobiol Aging , vol.27 , pp. 576-588
    • Seehafer, S.S.1    Pearce, D.A.2
  • 38
    • 0037174618 scopus 로고    scopus 로고
    • Alzheimers disease is a synaptic failure
    • Selkoe DJ. Alzheimers disease is a synaptic failure. Science 2002; 298: 789-91.
    • (2002) Science , vol.298 , pp. 789-791
    • Selkoe, D.J.1
  • 40
    • 38849146956 scopus 로고    scopus 로고
    • ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones
    • Wei H, Kim SJ, Zhang Z, Tsai PC, Wisniewski KE, Mukherjee AB. ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones. Hum Mol Genet 2008; 17: 469-77.
    • (2008) Hum Mol Genet , vol.17 , pp. 469-477
    • Wei, H.1    Kim, S.J.2    Zhang, Z.3    Tsai, P.C.4    Wisniewski, K.E.5    Mukherjee, A.B.6
  • 41
    • 33646486569 scopus 로고    scopus 로고
    • Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons
    • Weimer JM, Custer AW, Benedict JW, Alexander NA, Kingsley E, Federoff HJ, et al. Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons. Neurobiol Dis 2006; 22: 284-93.
    • (2006) Neurobiol Dis , vol.22 , pp. 284-293
    • Weimer, J.M.1    Custer, A.W.2    Benedict, J.W.3    Alexander, N.A.4    Kingsley, E.5    Federoff, H.J.6
  • 42
    • 78649738728 scopus 로고    scopus 로고
    • Current therapies for the soluble lysosomal forms of neuronal ceroid lipofuscinosis
    • Wong AM, Rahim AA, Waddington SN, Cooper JD. Current therapies for the soluble lysosomal forms of neuronal ceroid lipofuscinosis. Biochem Soc Trans 2010; 38: 1484-8.
    • (2010) Biochem Soc Trans , vol.38 , pp. 1484-1488
    • Wong, A.M.1    Rahim, A.A.2    Waddington, S.N.3    Cooper, J.D.4
  • 43
    • 2942563944 scopus 로고    scopus 로고
    • Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice
    • Wu YP, Proia RL. Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice. Proc Natl Acad Sci USA 2004; 101: 8425-30.
    • (2004) Proc Natl Acad Sci USA , vol.101 , pp. 8425-8430
    • Wu, Y.P.1    Proia, R.L.2
  • 44
    • 31144462635 scopus 로고    scopus 로고
    • Palmitoylprotein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL
    • Zhang Z, Lee YC, Kim SJ, Choi MS, Tsai PC, Xu Y, et al. Palmitoylprotein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL. Hum Mol Genet 2006; 15: 337-46.
    • (2006) Hum Mol Genet , vol.15 , pp. 337-346
    • Zhang, Z.1    Lee, Y.C.2    Kim, S.J.3    Choi, M.S.4    Tsai, P.C.5    Xu, Y.6


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