메뉴 건너뛰기




Volumn 37, Issue 1, 2014, Pages 83-92

Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1

Author keywords

[No Author keywords available]

Indexed keywords

COMPLEMENT COMPONENT C3; INTERLEUKIN 10; INTERLEUKIN 12P40; INTERLEUKIN 13; INTERLEUKIN 3; INTERLEUKIN 4; MIGLUSTAT; RANTES;

EID: 84891809529     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-013-9610-6     Document Type: Article
Times cited : (68)

References (53)
  • 1
    • 79959654327 scopus 로고    scopus 로고
    • Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment
    • 1:CAS:528:DC%2BC3MXos1yis7w%3D 3134878 21697390 10.1523/JNEUROSCI.1317- 11.2011
    • Aqul A, Liu B, Ramirez CM et al (2011) Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment. J Neurosci 31(25):9404-9413
    • (2011) J Neurosci , vol.31 , Issue.25 , pp. 9404-9413
    • Aqul, A.1    Liu, B.2    Ramirez, C.M.3
  • 2
    • 0842320913 scopus 로고    scopus 로고
    • Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: Immunohistochemical observations of microglia and astroglia
    • 1:CAS:528:DC%2BD2cXhtVSisb8%3D 14769381 10.1016/S0014-4886(03)00345-5
    • Baudry M, Yao Y, Simmons D, Liu J, Bi X (2003) Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia. Exp Neurol 184(2):887-903
    • (2003) Exp Neurol , vol.184 , Issue.2 , pp. 887-903
    • Baudry, M.1    Yao, Y.2    Simmons, D.3    Liu, J.4    Bi, X.5
  • 3
    • 0030863352 scopus 로고    scopus 로고
    • Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
    • 1:CAS:528:DyaK2sXksFClu7Y%3D 9211849 10.1126/science.277.5323.228
    • Carstea ED, Morris JA, Coleman KG et al (1997) Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science 277(5323):228-231
    • (1997) Science , vol.277 , Issue.5323 , pp. 228-231
    • Carstea, E.D.1    Morris, J.A.2    Coleman, K.G.3
  • 4
    • 0033152034 scopus 로고    scopus 로고
    • Mechanisms of IL-10 production in human microglia-T cell interaction
    • 1:CAS:528:DyaK1MXjtlKiur8%3D 10352303
    • Chabot S, Williams G, Hamilton M, Sutherland G, Yong VW (1999) Mechanisms of IL-10 production in human microglia-T cell interaction. J Immunol 162(11):6819-6828
    • (1999) J Immunol , vol.162 , Issue.11 , pp. 6819-6828
    • Chabot, S.1    Williams, G.2    Hamilton, M.3    Sutherland, G.4    Yong, V.W.5
  • 5
    • 70349190528 scopus 로고    scopus 로고
    • Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression
    • 2736622 19750228 10.1371/journal.pone.0006951
    • Davidson CD, Ali NF, Micsenyi MC et al (2009) Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. PLoS One 4(9):e6951
    • (2009) PLoS One , vol.4 , Issue.9 , pp. 6951
    • Davidson, C.D.1    Ali, N.F.2    Micsenyi, M.C.3
  • 6
    • 0034305621 scopus 로고    scopus 로고
    • Glial fibrillary acidic protein: GFAP-thirty-one years (1969-2000)
    • 1:CAS:528:DC%2BD3cXns1eiuro%3D 11059815 10.1023/A:1007677003387
    • Eng LF, Ghirnikar RS, Lee YL (2000) Glial fibrillary acidic protein: GFAP-thirty-one years (1969-2000). Neurochem Res 25(9-10):1439-1451
    • (2000) Neurochem Res , vol.25 , Issue.9-10 , pp. 1439-1451
    • Eng, L.F.1    Ghirnikar, R.S.2    Lee, Y.L.3
  • 7
    • 77957226432 scopus 로고    scopus 로고
    • Oxidative stress in Niemann-Pick disease, type C
    • 1:CAS:528:DC%2BC3cXht1Cmtb%2FJ 2950258 20667755 10.1016/j.ymgme.2010.06. 018
    • Fu R, Yanjanin NM, Bianconi S, Pavan WJ, Porter FD (2010) Oxidative stress in Niemann-Pick disease, type C. Mol Genet Metab 101(2-3):214-218
    • (2010) Mol Genet Metab , vol.101 , Issue.2-3 , pp. 214-218
    • Fu, R.1    Yanjanin, N.M.2    Bianconi, S.3    Pavan, W.J.4    Porter, F.D.5
  • 8
    • 77952312366 scopus 로고    scopus 로고
    • An accurate substitution method for analyzing censored data
    • 20169489 10.1080/15459621003609713
    • Ganser GH, Hewett P (2010) An accurate substitution method for analyzing censored data. J Occup Environ Hyg 7(4):233-244
    • (2010) J Occup Environ Hyg , vol.7 , Issue.4 , pp. 233-244
    • Ganser, G.H.1    Hewett, P.2
  • 9
    • 81755173570 scopus 로고    scopus 로고
    • Role of microglia in CNS inflammation
    • 1:CAS:528:DC%2BC3MXhsFSis73M 21889505 10.1016/j.febslet.2011.08.033
    • Graeber MB, Li W, Rodriguez ML (2011) Role of microglia in CNS inflammation. FEBS Lett 585(23):3798-3805
    • (2011) FEBS Lett , vol.585 , Issue.23 , pp. 3798-3805
    • Graeber, M.B.1    Li, W.2    Rodriguez, M.L.3
  • 10
    • 61349193143 scopus 로고    scopus 로고
    • Peripheral lipopolysaccharide (LPS) challenge promotes microglial hyperactivity in aged mice that is associated with exaggerated induction of both pro-inflammatory IL-1beta and anti-inflammatory IL-10 cytokines
    • 1:CAS:528:DC%2BD1MXisFSquro%3D 2692986 18814846 10.1016/j.bbi.2008.09.002
    • Henry CJ, Huang Y, Wynne AM, Godbout JP (2009) Peripheral lipopolysaccharide (LPS) challenge promotes microglial hyperactivity in aged mice that is associated with exaggerated induction of both pro-inflammatory IL-1beta and anti-inflammatory IL-10 cytokines. Brain Behav Immun 23(3):309-317
    • (2009) Brain Behav Immun , vol.23 , Issue.3 , pp. 309-317
    • Henry, C.J.1    Huang, Y.2    Wynne, A.M.3    Godbout, J.P.4
  • 11
    • 78349306067 scopus 로고    scopus 로고
    • Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons
    • 1:CAS:528:DC%2BC3cXhsVWqsLbO 20883783 10.1016/j.nbd.2010.09.008
    • Klein A, Maldonado C, Vargas LM et al (2011) Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons. Neurobiol Dis 41(1):209-218
    • (2011) Neurobiol Dis , vol.41 , Issue.1 , pp. 209-218
    • Klein, A.1    Maldonado, C.2    Vargas, L.M.3
  • 12
    • 3042701254 scopus 로고    scopus 로고
    • Macrophage specificity of three anti-CD68 monoclonal antibodies (KP1, EBM11, and PGM1) widely used for immunohistochemistry and flow cytometry
    • 1:CAS:528:DC%2BD2cXmsVKqur8%3D 15194571 10.1136/ard.2003.013029
    • Kunisch E, Fuhrmann R, Roth A, Winter R, Lungershausen W, Kinne RW (2004) Macrophage specificity of three anti-CD68 monoclonal antibodies (KP1, EBM11, and PGM1) widely used for immunohistochemistry and flow cytometry. Ann Rheum Dis 63(7):774-784
    • (2004) Ann Rheum Dis , vol.63 , Issue.7 , pp. 774-784
    • Kunisch, E.1    Fuhrmann, R.2    Roth, A.3    Winter, R.4    Lungershausen, W.5    Kinne, R.W.6
  • 13
    • 33748793667 scopus 로고    scopus 로고
    • Pregnane X receptor (PXR) activation: A mechanism for neuroprotection in a mouse model of Niemann-Pick C disease
    • 1:CAS:528:DC%2BD28XpvFSlur8%3D 16940355 10.1073/pnas.0606218103
    • Langmade SJ, Gale SE, Frolov A et al (2006) Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease. Proc Natl Acad Sci USA 103(37):13807-13812
    • (2006) Proc Natl Acad Sci USA , vol.103 , Issue.37 , pp. 13807-13812
    • Langmade, S.J.1    Gale, S.E.2    Frolov, A.3
  • 14
    • 16844373434 scopus 로고    scopus 로고
    • Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease
    • 1:CAS:528:DC%2BD2MXjvVWmsL0%3D 15835268
    • Li H, Repa JJ, Valasek MA et al (2005) Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease. J Neuropathol Exp Neurol 64(4):323-333
    • (2005) J Neuropathol Exp Neurol , vol.64 , Issue.4 , pp. 323-333
    • Li, H.1    Repa, J.J.2    Valasek, M.A.3
  • 15
    • 77949912882 scopus 로고    scopus 로고
    • Abnormal gene expression in cerebellum of Npc1-/- mice during postnatal development
    • 1:CAS:528:DC%2BC3cXktlGnsbo%3D 20153740 10.1016/j.brainres.2010.02.019
    • Liao G, Wen Z, Irizarry K et al (2010) Abnormal gene expression in cerebellum of Npc1-/- mice during postnatal development. Brain Res 1325:128-140
    • (2010) Brain Res , vol.1325 , pp. 128-140
    • Liao, G.1    Wen, Z.2    Irizarry, K.3
  • 16
    • 77951073185 scopus 로고    scopus 로고
    • Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid
    • 1:CAS:528:DC%2BC3cXltlWktr0%3D 19965601 10.1194/jlr.M000257
    • Liu B, Ramirez CM, Miller AM, Repa JJ, Turley SD, Dietschy JM (2010) Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid. J Lipid Res 51(5):933-944
    • (2010) J Lipid Res , vol.51 , Issue.5 , pp. 933-944
    • Liu, B.1    Ramirez, C.M.2    Miller, A.M.3    Repa, J.J.4    Turley, S.D.5    Dietschy, J.M.6
  • 17
    • 60549084168 scopus 로고    scopus 로고
    • Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse
    • 1:CAS:528:DC%2BD1MXit12ju74%3D 19171898 10.1073/pnas.0810895106
    • Liu B, Turley SD, Burns DK, Miller AM, Repa JJ, Dietschy JM (2009) Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse. Proc Natl Acad Sci USA 106(7):2377-2382
    • (2009) Proc Natl Acad Sci USA , vol.106 , Issue.7 , pp. 2377-2382
    • Liu, B.1    Turley, S.D.2    Burns, D.K.3    Miller, A.M.4    Repa, J.J.5    Dietschy, J.M.6
  • 18
    • 0035710746 scopus 로고    scopus 로고
    • Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method
    • 1:CAS:528:DC%2BD38XhtFelt7s%3D 11846609 10.1006/meth.2001.1262
    • Livak KJ, Schmittgen TD (2001) Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method. Methods 25(4):402-408
    • (2001) Methods , vol.25 , Issue.4 , pp. 402-408
    • Livak, K.J.1    Schmittgen, T.D.2
  • 19
    • 0030768084 scopus 로고    scopus 로고
    • Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeostasis gene
    • 1:CAS:528:DyaK2sXksFCksr0%3D 9211850 10.1126/science.277.5323.232
    • Loftus SK, Morris JA, Carstea ED et al (1997) Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. Science 277(5323):232-235
    • (1997) Science , vol.277 , Issue.5323 , pp. 232-235
    • Loftus, S.K.1    Morris, J.A.2    Carstea, E.D.3
  • 20
    • 84863521004 scopus 로고    scopus 로고
    • Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C
    • 1:CAS:528:DC%2BC38Xos1yltLo%3D 22493001 10.1093/hmg/dds126
    • Lopez ME, Klein AD, Hong J, Dimbil UJ, Scott MP (2012a) Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C. Hum Mol Genet 21(13):2946-2960
    • (2012) Hum Mol Genet , vol.21 , Issue.13 , pp. 2946-2960
    • Lopez, M.E.1    Klein, A.D.2    Hong, J.3    Dimbil, U.J.4    Scott, M.P.5
  • 21
    • 84866149223 scopus 로고    scopus 로고
    • Complement is dispensable for neurodegeneration in Niemann-Pick disease type C
    • 1:CAS:528:DC%2BC3sXlsVCitA%3D%3D 10.1186/1742-2094-9-216
    • Lopez ME, Klein AD, Scott MP (2012b) Complement is dispensable for neurodegeneration in Niemann-Pick disease type C. J Neuroinflamm 9(1):216
    • (2012) J Neuroinflamm , vol.9 , Issue.1 , pp. 216
    • Lopez, M.E.1    Klein, A.D.2    Scott, M.P.3
  • 22
    • 0028929548 scopus 로고
    • Neurofibrillary tangles in Niemann-Pick disease type C
    • 7894998 10.1093/brain/118.1.119
    • Love S, Bridges LR, Case CP (1995) Neurofibrillary tangles in Niemann-Pick disease type C. Brain 118(Pt 1):119-129
    • (1995) Brain , vol.118 , Issue.PART 1 , pp. 119-129
    • Love, S.1    Bridges, L.R.2    Case, C.P.3
  • 24
    • 0034750568 scopus 로고    scopus 로고
    • Neurodegeneration in Niemann-Pick type C disease mice
    • 1:STN:280:DC%2BD3MnmslGgtg%3D%3D 11713633 10.1007/s002210100870
    • Ong WY, Kumar U, Switzer RC et al (2001) Neurodegeneration in Niemann-Pick type C disease mice. Exp Brain Res 141(2):218-231
    • (2001) Exp Brain Res , vol.141 , Issue.2 , pp. 218-231
    • Ong, W.Y.1    Kumar, U.2    Switzer, R.C.3
  • 25
    • 25444453444 scopus 로고    scopus 로고
    • Neuroprotective role of microglia expressing interleukin-4
    • 1:CAS:528:DC%2BD2MXnsVeltLg%3D 15948189 10.1002/jnr.20483
    • Park KW, Lee DY, Joe EH, Kim SU, Jin BK (2005) Neuroprotective role of microglia expressing interleukin-4. J Neurosci Res 81(3):397-402
    • (2005) J Neurosci Res , vol.81 , Issue.3 , pp. 397-402
    • Park, K.W.1    Lee, D.Y.2    Joe, E.H.3    Kim, S.U.4    Jin, B.K.5
  • 26
    • 80053299096 scopus 로고    scopus 로고
    • Npc1 deficiency in the C57BL/6J genetic background enhances Niemann-Pick disease type C spleen pathology
    • 1:CAS:528:DC%2BC3MXht1Gkur%2FI 21910975 10.1016/j.bbrc.2011.08.096
    • Parra J, Klein AD, Castro J et al (2011) Npc1 deficiency in the C57BL/6J genetic background enhances Niemann-Pick disease type C spleen pathology. Biochem Biophys Res Commun 413(3):400-406
    • (2011) Biochem Biophys Res Commun , vol.413 , Issue.3 , pp. 400-406
    • Parra, J.1    Klein, A.D.2    Castro, J.3
  • 27
    • 77649249576 scopus 로고    scopus 로고
    • Long-term miglustat therapy in children with Niemann-Pick disease type C
    • 19822772 10.1177/0883073809344222
    • Patterson MC, Vecchio D, Jacklin E et al (2010) Long-term miglustat therapy in children with Niemann-Pick disease type C. J Child Neurol 25(3):300-305
    • (2010) J Child Neurol , vol.25 , Issue.3 , pp. 300-305
    • Patterson, M.C.1    Vecchio, D.2    Jacklin, E.3
  • 28
    • 34547753513 scopus 로고    scopus 로고
    • Miglustat for treatment of Niemann-Pick C disease: A randomised controlled study
    • 1:CAS:528:DC%2BD2sXhtVyrsr3F 17689147 10.1016/S1474-4422(07)70194-1
    • Patterson MC, Vecchio D, Prady H, Abel L, Wraith JE (2007) Miglustat for treatment of Niemann-Pick C disease: a randomised controlled study. Lancet Neurol 6(9):765-772
    • (2007) Lancet Neurol , vol.6 , Issue.9 , pp. 765-772
    • Patterson, M.C.1    Vecchio, D.2    Prady, H.3    Abel, L.4    Wraith, J.E.5
  • 29
    • 0023588096 scopus 로고
    • Group C Niemann-Pick disease: Faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts
    • 1:CAS:528:DyaL2sXltlajtbw%3D 3609608
    • Pentchev PG, Comly ME, Kruth HS et al (1987) Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts. FASEB J 1(1):40-45
    • (1987) FASEB J , vol.1 , Issue.1 , pp. 40-45
    • Pentchev, P.G.1    Comly, M.E.2    Kruth, H.S.3
  • 30
    • 70349748397 scopus 로고    scopus 로고
    • Miglustat in patients with Niemann-Pick disease Type C (NP-C): A multicenter observational retrospective cohort study
    • 1:CAS:528:DC%2BD1MXht1GmtLrE 19656703 10.1016/j.ymgme.2009.07.003
    • Pineda M, Wraith JE, Mengel E et al (2009) Miglustat in patients with Niemann-Pick disease Type C (NP-C): a multicenter observational retrospective cohort study. Mol Genet Metab 98(3):243-249
    • (2009) Mol Genet Metab , vol.98 , Issue.3 , pp. 243-249
    • Pineda, M.1    Wraith, J.E.2    Mengel, E.3
  • 31
    • 78149342830 scopus 로고    scopus 로고
    • Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease
    • 10.1126/scitranslmed.3001417
    • Porter FD, Scherrer DE, Lanier MH et al (2010) Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease. Sci Trans Med 2(56):56ra81
    • (2010) Sci Trans Med , vol.2 , Issue.56
    • Porter, F.D.1    Scherrer, D.E.2    Lanier, M.H.3
  • 32
    • 84856583715 scopus 로고    scopus 로고
    • Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice
    • 1:CAS:528:DC%2BC38XitlGksbk%3D 3657200 22198570 10.1016/j.nbd.2011.12.027
    • Pressey SN, Smith DA, Wong AM, Platt FM, Cooper JD (2012) Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice. Neurobiol Dis 45(3):1086-1100
    • (2012) Neurobiol Dis , vol.45 , Issue.3 , pp. 1086-1100
    • Pressey, S.N.1    Smith, D.A.2    Wong, A.M.3    Platt, F.M.4    Cooper, J.D.5
  • 33
    • 77957254517 scopus 로고    scopus 로고
    • Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life
    • 1:CAS:528:DC%2BC3cXhtFGntr3N 3065173 20581737 10.1203/PDR. 0b013e3181ee4dd2
    • Ramirez CM, Liu B, Taylor AM et al (2010) Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life. Pediatr Res 68(4):309-315
    • (2010) Pediatr Res , vol.68 , Issue.4 , pp. 309-315
    • Ramirez, C.M.1    Liu, B.2    Taylor, A.M.3
  • 34
    • 84859717206 scopus 로고    scopus 로고
    • Innate immunity in the central nervous system
    • 1:CAS:528:DC%2BC38Xlt1egt7c%3D 3314450 22466658 10.1172/JCI58644
    • Ransohoff RM, Brown MA (2012) Innate immunity in the central nervous system. J Clin Invest 122(4):1164-1171
    • (2012) J Clin Invest , vol.122 , Issue.4 , pp. 1164-1171
    • Ransohoff, R.M.1    Brown, M.A.2
  • 35
    • 34250324909 scopus 로고    scopus 로고
    • Clues to neuro-degeneration in Niemann-Pick type C disease from global gene expression profiling
    • 1762405 17183645 10.1371/journal.pone.0000019
    • Reddy JV, Ganley IG, Pfeffer SR (2006) Clues to neuro-degeneration in Niemann-Pick type C disease from global gene expression profiling. PLoS One 1:e19
    • (2006) PLoS One , vol.1 , pp. 19
    • Reddy, J.V.1    Ganley, I.G.2    Pfeffer, S.R.3
  • 36
    • 37549037886 scopus 로고    scopus 로고
    • Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse
    • 1:CAS:528:DC%2BD1cXktVGhsg%3D%3D 18160655 10.1523/JNEUROSCI.4823-07.2007
    • Repa JJ, Li H, Frank-Cannon TC et al (2007) Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse. J Neurosci 27(52):14470-14480
    • (2007) J Neurosci , vol.27 , Issue.52 , pp. 14470-14480
    • Repa, J.J.1    Li, H.2    Frank-Cannon, T.C.3
  • 37
    • 84861078831 scopus 로고    scopus 로고
    • A review: Inflammatory process in Alzheimer's disease, role of cytokines
    • Rubio-Perez JM, Morillas-Ruiz JM (2012) A review: inflammatory process in Alzheimer's disease, role of cytokines. SciWorld J 2012:756357
    • (2012) SciWorld J , vol.2012 , pp. 756357
    • Rubio-Perez, J.M.1    Morillas-Ruiz, J.M.2
  • 38
    • 0037428937 scopus 로고    scopus 로고
    • Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease
    • 12528192 10.1002/cne.10522
    • Sarna JR, Larouche M, Marzban H, Sillitoe RV, Rancourt DE, Hawkes R (2003) Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease. J Comp Neurol 456(3):279-291
    • (2003) J Comp Neurol , vol.456 , Issue.3 , pp. 279-291
    • Sarna, J.R.1    Larouche, M.2    Marzban, H.3    Sillitoe, R.V.4    Rancourt, D.E.5    Hawkes, R.6
  • 39
    • 70349767908 scopus 로고    scopus 로고
    • Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1
    • 1:CAS:528:DC%2BD1MXht1CqtL3M 19632328 10.1016/j.nbd.2009.07.010
    • Smith D, Wallom KL, Williams IM, Jeyakumar M, Platt FM (2009) Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1. Neurobiol Dis 36(2):242-251
    • (2009) Neurobiol Dis , vol.36 , Issue.2 , pp. 242-251
    • Smith, D.1    Wallom, K.L.2    Williams, I.M.3    Jeyakumar, M.4    Platt, F.M.5
  • 40
    • 84860224053 scopus 로고    scopus 로고
    • Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C
    • 1:CAS:528:DC%2BC38XlvFeitbs%3D 3352323 22487861 10.1097/NEN. 0b013e31825414a6
    • Stein VM, Crooks A, Ding W et al (2012) Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C. J Neuropathol Exp Neurol 71(5):434-448
    • (2012) J Neuropathol Exp Neurol , vol.71 , Issue.5 , pp. 434-448
    • Stein, V.M.1    Crooks, A.2    Ding, W.3
  • 41
    • 77950847889 scopus 로고    scopus 로고
    • Combined analysis of the glia secretome and the CSF proteome: Neuroinflammation and novel biomarkers
    • 1:CAS:528:DC%2BC3cXksVCku70%3D 20377392 10.1586/epr.10.6
    • Suk K (2010) Combined analysis of the glia secretome and the CSF proteome: neuroinflammation and novel biomarkers. Expert Rev proteomics 7(2):263-274
    • (2010) Expert Rev Proteomics , vol.7 , Issue.2 , pp. 263-274
    • Suk, K.1
  • 42
    • 0028922151 scopus 로고
    • Neurofibrillary tangles in Niemann-Pick disease type C
    • 1:STN:280:DyaK2M3nsVemuw%3D%3D 7754743 10.1007/BF00309338
    • Suzuki K, Parker CC, Pentchev PG et al (1995) Neurofibrillary tangles in Niemann-Pick disease type C. Acta Neuropathol 89(3):227-238
    • (1995) Acta Neuropathol , vol.89 , Issue.3 , pp. 227-238
    • Suzuki, K.1    Parker, C.C.2    Pentchev, P.G.3
  • 43
    • 77953019480 scopus 로고    scopus 로고
    • Niemann-Pick disease type C
    • 2902432 20525256 10.1186/1750-1172-5-16
    • Vanier MT (2010) Niemann-Pick disease type C. Orphanet J Rare Dis 5:16
    • (2010) Orphanet J Rare Dis , vol.5 , pp. 16
    • Vanier, M.T.1
  • 44
    • 0141886877 scopus 로고    scopus 로고
    • Niemann-Pick disease type C
    • 1:STN:280:DC%2BD3svos1ektw%3D%3D 12974729 10.1034/j.1399-0004.2003.00147. x
    • Vanier MT, Millat G (2003) Niemann-Pick disease type C. Clin Genet 64(4):269-281
    • (2003) Clin Genet , vol.64 , Issue.4 , pp. 269-281
    • Vanier, M.T.1    Millat, G.2
  • 45
    • 84055172758 scopus 로고    scopus 로고
    • Alteration of gene expression profile in niemann-pick type C mice correlates with tissue damage and oxidative stress
    • 1:CAS:528:DC%2BC38XltFentA%3D%3D 3245218 22216111 10.1371/journal.pone. 0028777
    • Vazquez MC, Del Pozo T, Robledo FA et al (2011) Alteration of gene expression profile in niemann-pick type C mice correlates with tissue damage and oxidative stress. PLoS One 6(12):e28777
    • (2011) PLoS One , vol.6 , Issue.12 , pp. 28777
    • Vazquez, M.C.1    Del Pozo, T.2    Robledo, F.A.3
  • 46
    • 79960447858 scopus 로고    scopus 로고
    • Complement in the brain
    • 1:CAS:528:DC%2BC3MXpt1ahur8%3D 3142281 21546088 10.1016/j.molimm.2011.04. 003
    • Veerhuis R, Nielsen HM, Tenner AJ (2011) Complement in the brain. Mol Immunol 48(14):1592-1603
    • (2011) Mol Immunol , vol.48 , Issue.14 , pp. 1592-1603
    • Veerhuis, R.1    Nielsen, H.M.2    Tenner, A.J.3
  • 47
    • 77953811394 scopus 로고    scopus 로고
    • 2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease
    • 1:CAS:528:DC%2BC3cXns1yntbc%3D 2913583 20357695 10.1203/PDR. 0b013e3181df4623
    • Ward S, O'Donnell P, Fernandez S, Vite CH (2010) 2-hydroxypropyl-beta- cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease. Pediatr Res 68(1):52-56
    • (2010) Pediatr Res , vol.68 , Issue.1 , pp. 52-56
    • Ward, S.1    O'Donnell, P.2    Fernandez, S.3    Vite, C.H.4
  • 48
    • 77649338413 scopus 로고    scopus 로고
    • Miglustat in adult and juvenile patients with Niemann-Pick disease type C: Long-term data from a clinical trial
    • 1:CAS:528:DC%2BC3cXjt1elsLs%3D 20045366 10.1016/j.ymgme.2009.12.006
    • Wraith JE, Vecchio D, Jacklin E et al (2010) Miglustat in adult and juvenile patients with Niemann-Pick disease type C: long-term data from a clinical trial. Mol Genet Metab 99(4):351-357
    • (2010) Mol Genet Metab , vol.99 , Issue.4 , pp. 351-357
    • Wraith, J.E.1    Vecchio, D.2    Jacklin, E.3
  • 49
    • 11444254013 scopus 로고    scopus 로고
    • Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease
    • 1:CAS:528:DC%2BD2MXis1Wjug%3D%3D 15639190 10.1016/j.ymgme.2004.08.017
    • Wu YP, Mizukami H, Matsuda J, Saito Y, Proia RL, Suzuki K (2005) Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease. Mol Genet Metab 84(1):9-17
    • (2005) Mol Genet Metab , vol.84 , Issue.1 , pp. 9-17
    • Wu, Y.P.1    Mizukami, H.2    Matsuda, J.3    Saito, Y.4    Proia, R.L.5    Suzuki, K.6
  • 50
    • 2942563944 scopus 로고    scopus 로고
    • Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice
    • 1:CAS:528:DC%2BD2cXkvFOmsbc%3D 15155903 10.1073/pnas.0400625101
    • Wu YP, Proia RL (2004) Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice. Proc Natl Acad Sci USA 101(22):8425-8430
    • (2004) Proc Natl Acad Sci USA , vol.101 , Issue.22 , pp. 8425-8430
    • Wu, Y.P.1    Proia, R.L.2
  • 51
    • 73949159934 scopus 로고    scopus 로고
    • Linear clinical progression, independent of age of onset, in Niemann-Pick disease, type C
    • 2798912 19415691
    • Yanjanin NM, Velez JI, Gropman A et al (2010) Linear clinical progression, independent of age of onset, in Niemann-Pick disease, type C. Am J Med Genet B Neuropsychiatr Genet 153B(1):132-140
    • (2010) Am J Med Genet B Neuropsychiatr Genet , vol.153 , Issue.1 , pp. 132-140
    • Yanjanin, N.M.1    Velez, J.I.2    Gropman, A.3
  • 52
    • 71549134227 scopus 로고    scopus 로고
    • Oxidative stress in NPC1 deficient cells: Protective effect of allopregnanolone
    • 2832077 18774957 10.1111/j.1582-4934.2008.00493.x
    • Zampieri S, Mellon SH, Butters TD et al (2009) Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone. J Cell Mol Med 13(9B):3786-3796
    • (2009) J Cell Mol Med , vol.13 B , Issue.9 , pp. 3786-3796
    • Zampieri, S.1    Mellon, S.H.2    Butters, T.D.3
  • 53
    • 0035142802 scopus 로고    scopus 로고
    • Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations
    • 1:CAS:528:DC%2BD3MXptVKmuw%3D%3D 11202175
    • Zervas M, Dobrenis K, Walkley SU (2001) Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. J Neuropathol Exp Neurol 60(1):49-64
    • (2001) J Neuropathol Exp Neurol , vol.60 , Issue.1 , pp. 49-64
    • Zervas, M.1    Dobrenis, K.2    Walkley, S.U.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.