-
1
-
-
34547753513
-
Miglustat for treatment of niemann-pick c disease: A randomised controlled study
-
Patterson, M. C.; Vecchio, D.; Prady, H.; Abel, L.; Wraith, J. E. Miglustat for treatment of niemann-pick c disease: A randomised controlled study. Lancet Neurol. 2007, 6, 765-772.
-
(2007)
Lancet Neurol
, vol.6
, pp. 765-772
-
-
Patterson, M.C.1
Vecchio, D.2
Prady, H.3
Abel, L.4
Wraith, J.E.5
-
2
-
-
84862532953
-
Recommendations for the diagnosis and management of niemann-pick disease type c: An update
-
Patterson, M. C.; Hendriksz, C. J.; Walterfang, M.; Sedel, F.; Vanier, M. T.; Wijburg, F. Recommendations for the diagnosis and management of niemann-pick disease type c: An update. Mol. Genet. Metab. 2012, 106, 330-344.
-
(2012)
Mol. Genet. Metab
, vol.106
, pp. 330-344
-
-
Patterson, M.C.1
Hendriksz, C.J.2
Walterfang, M.3
Sedel, F.4
Vanier, M.T.5
Wijburg, F.6
-
3
-
-
70349742377
-
Natural history of Niemann-Pick disease type C in a multicentre observational retrospective cohort study
-
Wraith, J. E.; Guffon, N.; Rohrbach, M.; Hwu, W. L.; Korenke, G. C.; Bembi, B.; Luzy, C.; Giorgino, R.; Sedel, F. Natural history of Niemann-Pick disease type C in a multicentre observational retrospective cohort study. Mol. Genet. Metab. 2009, 98, 250-254.
-
(2009)
Mol. Genet. Metab
, vol.98
, pp. 250-254
-
-
Wraith, J.E.1
Guffon, N.2
Rohrbach, M.3
Hwu, W.L.4
Korenke, G.C.5
Bembi, B.6
Luzy, C.7
Giorgino, R.8
Sedel, F.9
-
4
-
-
0030863352
-
Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
-
Carstea, E. D.; Morris, J. A.; Coleman, K. G.; Loftus, S. K.; Zhang, D.; Cummings, C.; Gu, J.; Rosenfeld, M. A.; Pavan, W. J.; Krizman, D. B.; et al. Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis. Science 1997, 277, 228-231.
-
(1997)
Science
, vol.277
, pp. 228-231
-
-
Carstea, E.D.1
Morris, J.A.2
Coleman, K.G.3
Loftus, S.K.4
Zhang, D.5
Cummings, C.6
Gu, J.7
Rosenfeld, M.A.8
Pavan, W.J.9
Krizman, D.B.10
-
5
-
-
0034704245
-
Identification of he1 as the second gene of niemann-pick c disease
-
Naureckiene, S.; Sleat, D. E.; Lackland, H.; Fensom, A.; Vanier, M. T.; Wattiaux, R.; Jadot, M.; Lobel, P. Identification of he1 as the second gene of niemann-pick c disease. Science 2000, 290, 2298-2301.
-
(2000)
Science
, vol.290
, pp. 2298-2301
-
-
Naureckiene, S.1
Sleat, D.E.2
Lackland, H.3
Fensom, A.4
Vanier, M.T.5
Wattiaux, R.6
Jadot, M.7
Lobel, P.8
-
6
-
-
67549105629
-
Structure of N-terminal domain of npc1 reveals distinct subdomains for binding and transfer of cholesterol
-
Kwon, H. J.; Abi-Mosleh, L.; Wang, M. L.; Deisenhofer, J.; Goldstein, J. L.; Brown, M. S.; Infante, R. E. Structure of N-terminal domain of npc1 reveals distinct subdomains for binding and transfer of cholesterol. Cell 2009, 137, 1213-1224.
-
(2009)
Cell
, vol.137
, pp. 1213-1224
-
-
Kwon, H.J.1
Abi-Mosleh, L.2
Wang, M.L.3
Deisenhofer, J.4
Goldstein, J.L.5
Brown, M.S.6
Infante, R.E.7
-
7
-
-
79851476221
-
Niemann-Pick type C disease: Molecular mechanisms and potential therapeutic approaches
-
Rosenbaum, A. I.; Maxfield, F. R. Niemann-Pick type C disease: Molecular mechanisms and potential therapeutic approaches. J. Neurochem. 2011, 116, 789-795.
-
(2011)
J. Neurochem
, vol.116
, pp. 789-795
-
-
Rosenbaum, A.I.1
Maxfield, F.R.2
-
8
-
-
80052010267
-
Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway
-
Dixit, S. S.; Jadot, M.; Sohar, I.; Sleat, D. E.; Stock, A. M.; Lobel, P. Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway. PLoS One 2011, 6, e23677.
-
(2011)
PLoS One
, vol.6
-
-
Dixit, S.S.1
Jadot, M.2
Sohar, I.3
Sleat, D.E.4
Stock, A.M.5
Lobel, P.6
-
9
-
-
11444254013
-
Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease
-
Wu, Y. P.; Mizukami, H.; Matsuda, J.; Saito, Y.; Proia, R. L.; Suzuki, K. Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease. Mol. Genet. Metab. 2005, 84, 9-17.
-
(2005)
Mol. Genet. Metab
, vol.84
, pp. 9-17
-
-
Wu, Y.P.1
Mizukami, H.2
Matsuda, J.3
Saito, Y.4
Proia, R.L.5
Suzuki, K.6
-
10
-
-
4744356190
-
Before the loss: Neuronal dysfunction in Niemann-Pick type C disease
-
Paul, C. A.; Boegle, A. K.; Maue, R. A. Before the loss: Neuronal dysfunction in Niemann-Pick type C disease. Biochim. Biophys. Acta 2004, 1685, 63-76.
-
(2004)
Biochim. Biophys. Acta
, vol.1685
, pp. 63-76
-
-
Paul, C.A.1
Boegle, A.K.2
Maue, R.A.3
-
11
-
-
4744365602
-
Consequences of NPC1 and NPC2 loss of function in mammalian neurons
-
Walkley, S. U.; Suzuki, K. Consequences of NPC1 and NPC2 loss of function in mammalian neurons. Biochim. Biophys. Acta 2004, 1685, 48-62.
-
(2004)
Biochim. Biophys. Acta
, vol.1685
, pp. 48-62
-
-
Walkley, S.U.1
Suzuki, K.2
-
12
-
-
77950544225
-
Conditional Niemann-Pick C mice demonstrate cell autonomous purkinje cell neurodegeneration
-
Elrick, M. J.; Pacheco, C. D.; Yu, T.; Dadgar, N.; Shakkottai, V. G.; Ware, C.; Paulson, H. L.; Lieberman, A. P. Conditional Niemann-Pick C mice demonstrate cell autonomous purkinje cell neurodegeneration. Hum. Mol. Genet. 2010, 19, 837-847.
-
(2010)
Hum. Mol. Genet
, vol.19
, pp. 837-847
-
-
Elrick, M.J.1
Pacheco, C.D.2
Yu, T.3
Dadgar, N.4
Shakkottai, V.G.5
Ware, C.6
Paulson, H.L.7
Lieberman, A.P.8
-
13
-
-
77953314838
-
Pre-synaptic defects of NPC1-deficient hippocampal neurons are not directly related to plasma membrane cholesterol
-
Hawes, C. M.; Wiemer, H.; Krueger, S. R.; Karten, B. Pre-synaptic defects of NPC1-deficient hippocampal neurons are not directly related to plasma membrane cholesterol. J. Neurochem. 2010, 114, 311-322.
-
(2010)
J. Neurochem
, vol.114
, pp. 311-322
-
-
Hawes, C.M.1
Wiemer, H.2
Krueger, S.R.3
Karten, B.4
-
14
-
-
54049085754
-
Imatinib therapy blocks cerebellar apoptosis and improves neurological symptoms in a mouse model of Niemann-Pick type C disease
-
Alvarez, A. R.; Klein, A.; Castro, J.; Cancino, G. I.; Amigo, J.; Mosqueira, M.; Vargas, L. M.; Yevenes, L. F.; Bronfman, F. C.; Zanlungo, S. Imatinib therapy blocks cerebellar apoptosis and improves neurological symptoms in a mouse model of Niemann-Pick type C disease. FASEB J. 2008, 22, 3617-3627.
-
(2008)
FASEB J
, vol.22
, pp. 3617-3627
-
-
Alvarez, A.R.1
Klein, A.2
Castro, J.3
Cancino, G.I.4
Amigo, J.5
Mosqueira, M.6
Vargas, L.M.7
Yevenes, L.F.8
Bronfman, F.C.9
Zanlungo, S.10
-
15
-
-
84055172758
-
Alteration of gene expression profile in Niemann-Pick type C mice correlates with tissue damage and oxidative stress
-
Vazquez, M. C.; del Pozo, T.; Robledo, F. A.; Carrasco, G.; Pavez, L.; Olivares, F.; Gonzalez, M.; Zanlungo, S. Alteration of gene expression profile in Niemann-Pick type C mice correlates with tissue damage and oxidative stress. PLoS One 2011, 6, e28777.
-
(2011)
PLoS One
, vol.6
-
-
Vazquez, M.C.1
del Pozo, T.2
Robledo, F.A.3
Carrasco, G.4
Pavez, L.5
Olivares, F.6
Gonzalez, M.7
Zanlungo, S.8
-
16
-
-
84891809529
-
Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1
-
Cologna, S. M.; Cluzeau, C. V.; Yanjanin, N. M.; Blank, P. S.; Dail, M. K.; Siebel, S.; Toth, C. L.; Wassif, C. A.; Lieberman, A. P.; Porter, F. D. Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1. J. Inherit. Metab. Dis. 2014, 37, 83-92.
-
(2014)
J. Inherit. Metab. Dis
, vol.37
, pp. 83-92
-
-
Cologna, S.M.1
Cluzeau, C.V.2
Yanjanin, N.M.3
Blank, P.S.4
Dail, M.K.5
Siebel, S.6
Toth, C.L.7
Wassif, C.A.8
Lieberman, A.P.9
Porter, F.D.10
-
17
-
-
84893192165
-
Pre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick type C1-deficient murine brain
-
Kennedy, B. E.; Leblanc, V. G.; Mailman, T. M.; Fice, D.; Burton, I.; Karakach, T. K.; Karten, B. Pre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick type C1-deficient murine brain. PLoS One 2013, 8, e82685.
-
(2013)
PLoS One
, vol.8
-
-
Kennedy, B.E.1
Leblanc, V.G.2
Mailman, T.M.3
Fice, D.4
Burton, I.5
Karakach, T.K.6
Karten, B.7
-
18
-
-
84865865703
-
Oxidative stress in Niemann-Pick type C patients: A protective role of n-butyl-deoxynojirimycin therapy
-
Ribas, G. S.; Pires, R.; Coelho, J. C.; Rodrigues, D.; Mescka, C. P.; Vanzin, C. S.; Biancini, G. B.; Negretto, G.; Wayhs, C. A.; Wajner, M.; et al. Oxidative stress in Niemann-Pick type C patients: A protective role of n-butyl-deoxynojirimycin therapy. Int. J. Dev. Neurosci. 2012, 30, 439-444.
-
(2012)
Int. J. Dev. Neurosci
, vol.30
, pp. 439-444
-
-
Ribas, G.S.1
Pires, R.2
Coelho, J.C.3
Rodrigues, D.4
Mescka, C.P.5
Vanzin, C.S.6
Biancini, G.B.7
Negretto, G.8
Wayhs, C.A.9
Wajner, M.10
-
19
-
-
77957226432
-
Oxidative stress in Niemann-Pick disease, type C
-
Fu, R.; Yanjanin, N. M.; Bianconi, S.; Pavan, W. J.; Porter, F. D. Oxidative stress in Niemann-Pick disease, type C. Mol. Genet. Metab. 2010, 101, 214-218.
-
(2010)
Mol. Genet. Metab
, vol.101
, pp. 214-218
-
-
Fu, R.1
Yanjanin, N.M.2
Bianconi, S.3
Pavan, W.J.4
Porter, F.D.5
-
20
-
-
84868124278
-
Quantitative proteomic analysis of Niemann-Pick disease, type C1 cerebellum identifies protein biomarkers and provides pathological insight
-
Cologna, S. M.; Jiang, X. S.; Backlund, P. S.; Cluzeau, C. V.; Dail, M. K.; Yanjanin, N. M.; Siebel, S.; Toth, C. L.; Jun, H. S.; Wassif, C. A.; et al. Quantitative proteomic analysis of Niemann-Pick disease, type C1 cerebellum identifies protein biomarkers and provides pathological insight. PLoS One 2012, 7, e47845.
-
(2012)
PLoS One
, vol.7
-
-
Cologna, S.M.1
Jiang, X.S.2
Backlund, P.S.3
Cluzeau, C.V.4
Dail, M.K.5
Yanjanin, N.M.6
Siebel, S.7
Toth, C.L.8
Jun, H.S.9
Wassif, C.A.10
-
21
-
-
78149342830
-
Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease
-
doi: 10. 1126/scitranslmed. 3001417
-
Porter, F. D.; Scherrer, D. E.; Lanier, M. H.; Langmade, S. J.; Molugu, V.; Gale, S. E.; Olzeski, D.; Sidhu, R.; Dietzen, D. J.; Fu, R.; et al. Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease. Sci. Transl. Med. 2010, 2, 56ra81, doi: 10. 1126/scitranslmed. 3001417.
-
(2010)
Sci. Transl. Med
, vol.2
-
-
Porter, F.D.1
Scherrer, D.E.2
Lanier, M.H.3
Langmade, S.J.4
Molugu, V.5
Gale, S.E.6
Olzeski, D.7
Sidhu, R.8
Dietzen, D.J.9
Fu, R.10
-
22
-
-
79959232858
-
A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma
-
Jiang, X.; Sidhu, R.; Porter, F. D.; Yanjanin, N. M.; Speak, A. O.; te Vruchte, D. T.; Platt, F. M.; Fujiwara, H.; Scherrer, D. E.; Zhang, J.; et al. A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma. J. Lipid Res. 2011, 52, 1435-1445.
-
(2011)
J. Lipid Res
, vol.52
, pp. 1435-1445
-
-
Jiang, X.1
Sidhu, R.2
Porter, F.D.3
Yanjanin, N.M.4
Speak, A.O.5
Te Vruchte, D.T.6
Platt, F.M.7
Fujiwara, H.8
Scherrer, D.E.9
Zhang, J.10
-
23
-
-
84881600379
-
Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1
-
Fu, R.; Wassif, C. A.; Yanjanin, N. M.; Watkins-Chow, D. E.; Baxter, L. L.; Incao, A.; Liscum, L.; Sidhu, R.; Firnkes, S.; Graham, M.; et al. Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1. Hum. Mol. Genet. 2013, 22, 3508-3523.
-
(2013)
Hum. Mol. Genet
, vol.22
, pp. 3508-3523
-
-
Fu, R.1
Wassif, C.A.2
Yanjanin, N.M.3
Watkins-Chow, D.E.4
Baxter, L.L.5
Incao, A.6
Liscum, L.7
Sidhu, R.8
Firnkes, S.9
Graham, M.10
-
24
-
-
78349306067
-
Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons
-
Klein, A.; Maldonado, C.; Vargas, L. M.; Gonzalez, M.; Robledo, F.; Perez de Arce, K.; Munoz, F. J.; Hetz, C.; Alvarez, A. R.; Zanlungo, S. Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons. Neurobiol. Dis. 2011, 41, 209-218.
-
(2011)
Neurobiol. Dis
, vol.41
, pp. 209-218
-
-
Klein, A.1
Maldonado, C.2
Vargas, L.M.3
Gonzalez, M.4
Robledo, F.5
Perez de Arce, K.6
Munoz, F.J.7
Hetz, C.8
Alvarez, A.R.9
Zanlungo, S.10
-
25
-
-
83155178547
-
Lysosomal vitamin E accumulation in Niemann-Pick type C disease
-
Yevenes, L. F.; Klein, A.; Castro, J. F.; Marin, T.; Leal, N.; Leighton, F.; Alvarez, A. R.; Zanlungo, S. Lysosomal vitamin E accumulation in Niemann-Pick type C disease. Biochim. Biophys. Acta 2012, 1822, 150-160.
-
(2012)
Biochim. Biophys. Acta
, vol.1822
, pp. 150-160
-
-
Yevenes, L.F.1
Klein, A.2
Castro, J.F.3
Marin, T.4
Leal, N.5
Leighton, F.6
Alvarez, A.R.7
Zanlungo, S.8
-
26
-
-
79959188265
-
Altered vitamin e status in Niemann-Pick type C disease
-
Ulatowski, L.; Parker, R.; Davidson, C.; Yanjanin, N.; Kelley, T. J.; Corey, D.; Atkinson, J.; Porter, F.; Arai, H.; Walkley, S. U.; et al. Altered vitamin e status in Niemann-Pick type C disease. J. Lipid Res. 2011, 52, 1400-1410.
-
(2011)
J. Lipid Res
, vol.52
, pp. 1400-1410
-
-
Ulatowski, L.1
Parker, R.2
Davidson, C.3
Yanjanin, N.4
Kelley, T.J.5
Corey, D.6
Atkinson, J.7
Porter, F.8
Arai, H.9
Walkley, S.U.10
-
27
-
-
84863648223
-
The role of vitamin E on intracellular signaling pathways in brain: Molecular basis for the treatment of neurodegenerative processes
-
In, Braunstein, M.H., Ed.; Nova Science: New York, NY, USA
-
Serra, S. A.; Ill-Raga, G.; Coma, M.; Guix, F. X.; Muñoz, F. J. The role of vitamin E on intracellular signaling pathways in brain: Molecular basis for the treatment of neurodegenerative processes. In Vitamin E: New research; Braunstein, M. H., Ed.; Nova Science: New York, NY, USA, 2006; pp. 151-176.
-
(2006)
Vitamin E: New research
, pp. 151-176
-
-
Serra, S.A.1
Ill-Raga, G.2
Coma, M.3
Guix, F.X.4
Muñoz, F.J.5
-
28
-
-
0031889483
-
Ataxia with isolated vitamin E deficiency: Heterogeneity of mutations and phenotypic variability in a large number of families
-
Cavalier, L.; Ouahchi, K.; Kayden, H. J.; Di Donato, S.; Reutenauer, L.; Mandel, J. L.; Koenig, M. Ataxia with isolated vitamin E deficiency: Heterogeneity of mutations and phenotypic variability in a large number of families. Am. J. Hum. Genet. 1998, 62, 301-310.
-
(1998)
Am. J. Hum. Genet
, vol.62
, pp. 301-310
-
-
Cavalier, L.1
Ouahchi, K.2
Kayden, H.J.3
Di Donato, S.4
Reutenauer, L.5
Mandel, J.L.6
Koenig, M.7
-
29
-
-
0034610293
-
Increased atherosclerosis in hyperlipidemic mice deficient in alpha-tocopherol transfer protein and vitamin e
-
Terasawa, Y.; Ladha, Z.; Leonard, S. W.; Morrow, J. D.; Newland, D.; Sanan, D.; Packer, L.; Traber, M. G.; Farese, R. V., Jr. Increased atherosclerosis in hyperlipidemic mice deficient in alpha-tocopherol transfer protein and vitamin e. Proc. Natl. Acad. Sci. USA 2000, 97, 13830-13834.
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 13830-13834
-
-
Terasawa, Y.1
Ladha, Z.2
Leonard, S.W.3
Morrow, J.D.4
Newland, D.5
Sanan, D.6
Packer, L.7
Traber, M.G.8
Farese Jr., R.V.9
-
30
-
-
0035910076
-
Delayed-onset ataxia in mice lacking alpha-tocopherol transfer protein: Model for neuronal degeneration caused by chronic oxidative stress
-
Yokota, T.; Igarashi, K.; Uchihara, T.; Jishage, K.; Tomita, H.; Inaba, A.; Li, Y.; Arita, M.; Suzuki, H.; Mizusawa, H.; et al. Delayed-onset ataxia in mice lacking alpha-tocopherol transfer protein: Model for neuronal degeneration caused by chronic oxidative stress. Proc. Natl. Acad. Sci. USA 2001, 98, 15185-15190.
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 15185-15190
-
-
Yokota, T.1
Igarashi, K.2
Uchihara, T.3
Jishage, K.4
Tomita, H.5
Inaba, A.6
Li, Y.7
Arita, M.8
Suzuki, H.9
Mizusawa, H.10
-
31
-
-
10444258232
-
Tamoxifen and vitamin E treatments delay symptoms in the mouse model of Niemann-Pick C
-
Bascunan-Castillo, E. C.; Erickson, R. P.; Howison, C. M.; Hunter, R. J.; Heidenreich, R. H.; Hicks, C.; Trouard, T. P.; Gillies, R. J. Tamoxifen and vitamin E treatments delay symptoms in the mouse model of Niemann-Pick C. J. Appl. Genet. 2004, 45, 461-467.
-
(2004)
J. Appl. Genet
, vol.45
, pp. 461-467
-
-
Bascunan-Castillo, E.C.1
Erickson, R.P.2
Howison, C.M.3
Hunter, R.J.4
Heidenreich, R.H.5
Hicks, C.6
Trouard, T.P.7
Gillies, R.J.8
-
32
-
-
0036789434
-
Relevance of Niemann-Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice
-
Amigo, L.; Mendoza, H.; Castro, J.; Quinones, V.; Miquel, J. F.; Zanlungo, S. Relevance of Niemann-Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice. Hepatology 2002, 36, 819-828.
-
(2002)
Hepatology
, vol.36
, pp. 819-828
-
-
Amigo, L.1
Mendoza, H.2
Castro, J.3
Quinones, V.4
Miquel, J.F.5
Zanlungo, S.6
-
33
-
-
0032740439
-
Cholesterol accumulation in tissues of the Niemann-Pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ
-
Xie, C.; Turley, S. D.; Dietschy, J. M. Cholesterol accumulation in tissues of the Niemann-Pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ. Proc. Natl. Acad. Sci. USA 1999, 96, 11992-11997.
-
(1999)
Proc. Natl. Acad. Sci. USA
, vol.96
, pp. 11992-11997
-
-
Xie, C.1
Turley, S.D.2
Dietschy, J.M.3
-
34
-
-
0037089805
-
Cognitive deficit and development of motor impairment in a mouse model of Niemann-Pick type C disease
-
Voikar, V.; Rauvala, H.; Ikonen, E. Cognitive deficit and development of motor impairment in a mouse model of Niemann-Pick type C disease. Behav. Brain Res. 2002, 132, 1-10.
-
(2002)
Behav. Brain Res
, vol.132
, pp. 1-10
-
-
Voikar, V.1
Rauvala, H.2
Ikonen, E.3
-
35
-
-
59649085329
-
HT22 hippocampal neuronal cell line possesses functional cholinergic properties
-
Liu, J.; Li, L.; Suo, W. Z. HT22 hippocampal neuronal cell line possesses functional cholinergic properties. Life Sci. 2009, 84, 267-271.
-
(2009)
Life Sci
, vol.84
, pp. 267-271
-
-
Liu, J.1
Li, L.2
Suo, W.Z.3
-
36
-
-
50849116386
-
Sti571 prevents apoptosis, tau phosphorylation and behavioural impairments induced by Alzheimer's beta-amyloid deposits
-
Cancino, G. I.; Toledo, E. M.; Leal, N. R.; Hernandez, D. E.; Yevenes, L. F.; Inestrosa, N. C.; Alvarez, A. R. Sti571 prevents apoptosis, tau phosphorylation and behavioural impairments induced by Alzheimer's beta-amyloid deposits. Brain 2008, 131, 2425-2442.
-
(2008)
Brain
, vol.131
, pp. 2425-2442
-
-
Cancino, G.I.1
Toledo, E.M.2
Leal, N.R.3
Hernandez, D.E.4
Yevenes, L.F.5
Inestrosa, N.C.6
Alvarez, A.R.7
-
37
-
-
0028054149
-
Measurement of antioxidants in human blood plasma
-
Motchnik, P. A.; Frei, B.; Ames, B. N. Measurement of antioxidants in human blood plasma. Methods Enzymol. 1994, 234, 269-279.
-
(1994)
Methods Enzymol
, vol.234
, pp. 269-279
-
-
Motchnik, P.A.1
Frei, B.2
Ames, B.N.3
-
38
-
-
0037428937
-
Patterned purkinje cell degeneration in mouse models of Niemann-Pick type C disease
-
Sarna, J. R.; Larouche, M.; Marzban, H.; Sillitoe, R. V.; Rancourt, D. E.; Hawkes, R. Patterned purkinje cell degeneration in mouse models of Niemann-Pick type C disease. J. Comp. Neurol. 2003, 456, 279-291.
-
(2003)
J. Comp. Neurol
, vol.456
, 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
-
-
0032935277
-
U18666a inhibits intracellular cholesterol transport and neurotransmitter release in human neuroblastoma cells
-
Sparrow, S. M.; Carter, J. M.; Ridgway, N. D.; Cook, H. W.; Byers, D. M. U18666a inhibits intracellular cholesterol transport and neurotransmitter release in human neuroblastoma cells. Neurochem. Res. 1999, 24, 69-77.
-
(1999)
Neurochem. Res
, vol.24
, pp. 69-77
-
-
Sparrow, S.M.1
Carter, J.M.2
Ridgway, N.D.3
Cook, H.W.4
Byers, D.M.5
-
40
-
-
0024312609
-
The intracellular transport of low density lipoprotein-derived cholesterol is inhibited in chinese hamster ovary cells cultured with 3-beta-[2-(diethylamino)ethoxy]androst-5-en-17-one
-
Liscum, L.; Faust, J. R. The intracellular transport of low density lipoprotein-derived cholesterol is inhibited in chinese hamster ovary cells cultured with 3-beta-[2-(diethylamino)ethoxy]androst-5-en-17-one. J. Biol. Chem. 1989, 264, 11796-11806.
-
(1989)
J. Biol. Chem
, vol.264
, pp. 11796-11806
-
-
Liscum, L.1
Faust, J.R.2
-
41
-
-
33749247584
-
Cellular mechanism of U18666a-mediated apoptosis in cultured murine cortical neurons: Bridging Niemann-Pick disease type C and Alzheimer's disease
-
Koh, C. H.; Cheung, N. S. Cellular mechanism of U18666a-mediated apoptosis in cultured murine cortical neurons: Bridging Niemann-Pick disease type C and Alzheimer's disease. Cell Signal. 2006, 18, 1844-1853.
-
(2006)
Cell Signal
, vol.18
, pp. 1844-1853
-
-
Koh, C.H.1
Cheung, N.S.2
-
42
-
-
0033586250
-
Localization of alpha-tocopherol transfer protein in the brains of patients with ataxia with vitamin E deficiency and other oxidative stress related neurodegenerative disorders
-
Copp, R. P.; Wisniewski, T.; Hentati, F.; Larnaout, A.; Ben Hamida, M.; Kayden, H. J. Localization of alpha-tocopherol transfer protein in the brains of patients with ataxia with vitamin E deficiency and other oxidative stress related neurodegenerative disorders. Brain Res. 1999, 822, 80-87.
-
(1999)
Brain Res
, vol.822
, pp. 80-87
-
-
Copp, R.P.1
Wisniewski, T.2
Hentati, F.3
Larnaout, A.4
Ben Hamida, M.5
Kayden, H.J.6
-
43
-
-
84892450811
-
Vitamin E is essential for purkinje neuron integrity
-
Ulatowski, L.; Parker, R.; Warrier, G.; Sultana, R.; Butterfield, D. A.; Manor, D. Vitamin E is essential for purkinje neuron integrity. Neuroscience 2014, 260, 120-129.
-
(2014)
Neuroscience
, vol.260
, pp. 120-129
-
-
Ulatowski, L.1
Parker, R.2
Warrier, G.3
Sultana, R.4
Butterfield, D.A.5
Manor, D.6
-
44
-
-
84862154360
-
Plasma and tissue concentrations of alpha-tocopherol and delta-tocopherol following high dose dietary supplementation in mice
-
Baxter, L. L.; Marugan, J. J.; Xiao, J.; Incao, A.; McKew, J. C.; Zheng, W.; Pavan, W. J. Plasma and tissue concentrations of alpha-tocopherol and delta-tocopherol following high dose dietary supplementation in mice. Nutrients 2012, 4, 467-490.
-
(2012)
Nutrients
, vol.4
, pp. 467-490
-
-
Baxter, L.L.1
Marugan, J.J.2
Xiao, J.3
Incao, A.4
McKew, J.C.5
Zheng, W.6
Pavan, W.J.7
-
45
-
-
33750923120
-
How much vitamin e? Just enough!
-
Traber, M. G. How much vitamin e? Just enough! Am. J. Clin. Nutr. 2006, 84, 959-960.
-
(2006)
Am. J. Clin. Nutr
, vol.84
, pp. 959-960
-
-
Traber, M.G.1
-
46
-
-
84887541408
-
Liver x receptor up-regulates alpha-tocopherol transfer protein expression and alpha-tocopherol status
-
Koh, M.; Takitani, K.; Miyazaki, H.; Yamaoka, S.; Tamai, H. Liver x receptor up-regulates alpha-tocopherol transfer protein expression and alpha-tocopherol status. J. Nutr. Biochem. 2013, 24, 2158-2167.
-
(2013)
J. Nutr. Biochem
, vol.24
, pp. 2158-2167
-
-
Koh, M.1
Takitani, K.2
Miyazaki, H.3
Yamaoka, S.4
Tamai, H.5
-
47
-
-
84874482064
-
Oxidative stress: A pathogenic mechanism for Niemann-Pick type C disease
-
Vazquez, M. C.; Balboa, E.; Alvarez, A. R.; Zanlungo, S.; Oxidative stress: A pathogenic mechanism for Niemann-Pick type C disease. Oxid. Med. Cell. Longev. 2012, 2012, 205713.
-
(2012)
Oxid Med. Cell. Longev
, vol.2012
, pp. 205713
-
-
Vazquez, M.C.1
Balboa, E.2
Alvarez, A.R.3
Zanlungo, S.4
-
48
-
-
15744378799
-
Altered cholesterol metabolism in Niemann-Pick type C1 mouse brains affects mitochondrial function
-
Yu, W.; Gong, J. S.; Ko, M.; Garver, W. S.; Yanagisawa, K.; Michikawa, M. Altered cholesterol metabolism in Niemann-Pick type C1 mouse brains affects mitochondrial function. J. Biol. Chem. 2005, 280, 11731-11739.
-
(2005)
J. Biol. Chem
, vol.280
, pp. 11731-11739
-
-
Yu, W.1
Gong, J.S.2
Ko, M.3
Garver, W.S.4
Yanagisawa, K.5
Michikawa, M.6
-
49
-
-
84897421659
-
Vitamin E-gene interactions in aging and inflammatory age-related diseases: Implications for treatment. A systematic review
-
Mocchegiani, E.; Costarelli, L.; Giacconi, R.; Malavolta, M.; Basso, A.; Piacenza, F.; Ostan, R.; Cevenini, E.; Gonos, E. S.; Franceschi, C.; et al. Vitamin E-gene interactions in aging and inflammatory age-related diseases: Implications for treatment. A systematic review. Ageing Res. Rev. 2014, 14, 81-101.
-
(2014)
Ageing Res. Rev
, vol.14
, pp. 81-101
-
-
Mocchegiani, E.1
Costarelli, L.2
Giacconi, R.3
Malavolta, M.4
Basso, A.5
Piacenza, F.6
Ostan, R.7
Cevenini, E.8
Gonos, E.S.9
Franceschi, C.10
-
50
-
-
0028824565
-
Vitamin E and aging immune response
-
Meydani, S. N.; Hayek, M. G. Vitamin E and aging immune response. Clin. Geriatr. Med. 1995, 11, 567-576.
-
(1995)
Clin. Geriatr. Med
, vol.11
, pp. 567-576
-
-
Meydani, S.N.1
Hayek, M.G.2
-
51
-
-
0035882482
-
Enhanced antioxidant and cytoprotective abilities of vitamin e succinate is associated with a rapid uptake advantage in rat hepatocytes and mitochondria
-
Fariss, M. W.; Nicholls-Grzemski, F. A.; Tirmenstein, M. A.; Zhang, J. G. Enhanced antioxidant and cytoprotective abilities of vitamin e succinate is associated with a rapid uptake advantage in rat hepatocytes and mitochondria. Free Radic. Biol. Med. 2001, 31, 530-541.
-
(2001)
Free Radic. Biol. Med
, vol.31
, pp. 530-541
-
-
Fariss, M.W.1
Nicholls-Grzemski, F.A.2
Tirmenstein, M.A.3
Zhang, J.G.4
-
52
-
-
0035930395
-
Vitamin E succinate protects hepatocytes against the toxic effect of reactive oxygen species generated at mitochondrial complexes I and III by alkylating agents
-
Zhang, J. G.; Nicholls-Grzemski, F. A.; Tirmenstein, M. A.; Fariss, M. W. Vitamin E succinate protects hepatocytes against the toxic effect of reactive oxygen species generated at mitochondrial complexes I and III by alkylating agents. Chem. Biol. Interact. 2001, 138, 267-284.
-
(2001)
Chem. Biol. Interact
, vol.138
, pp. 267-284
-
-
Zhang, J.G.1
Nicholls-Grzemski, F.A.2
Tirmenstein, M.A.3
Fariss, M.W.4
-
53
-
-
0032820358
-
Vitamin E regulates mitochondrial hydrogen peroxide generation
-
Chow, C. K.; Ibrahim, W.; Wei, Z.; Chan, A. C. Vitamin E regulates mitochondrial hydrogen peroxide generation. Free Radic. Biol. Med. 1999, 27, 580-587.
-
(1999)
Free Radic. Biol. Med
, vol.27
, pp. 580-587
-
-
Chow, C.K.1
Ibrahim, W.2
Wei, Z.3
Chan, A.C.4
-
54
-
-
33747626511
-
Mitochondrial free cholesterol loading sensitizes to TNF-and Fas-mediated steatohepatitis
-
Mari, M.; Caballero, F.; Colell, A.; Morales, A.; Caballeria, J.; Fernandez, A.; Enrich, C.; Fernandez-Checa, J. C.; Garcia-Ruiz, C. Mitochondrial free cholesterol loading sensitizes to TNF-and Fas-mediated steatohepatitis. Cell Metab. 2006, 4, 185-198.
-
(2006)
Cell Metab
, vol.4
, pp. 185-198
-
-
Mari, M.1
Caballero, F.2
Colell, A.3
Morales, A.4
Caballeria, J.5
Fernandez, A.6
Enrich, C.7
Fernandez-Checa, J.C.8
Garcia-Ruiz, C.9
-
55
-
-
84888799687
-
Age and heat exposure-dependent changes in antioxidant enzymes activities in rat's liver and brain mitochondria: Role of alpha-tocopherol
-
Stojkovski, V.; Hadzi-Petrushev, N.; Ilieski, V.; Sopi, R.; Gjorgoski, I.; Mitrov, D.; Jankulovski, N.; Mladenov, M. Age and heat exposure-dependent changes in antioxidant enzymes activities in rat's liver and brain mitochondria: Role of alpha-tocopherol. Physiol. Res. 2013, 62, 503-510.
-
(2013)
Physiol. Res
, vol.62
, pp. 503-510
-
-
Stojkovski, V.1
Hadzi-Petrushev, N.2
Ilieski, V.3
Sopi, R.4
Gjorgoski, I.5
Mitrov, D.6
Jankulovski, N.7
Mladenov, M.8
-
56
-
-
80051472674
-
Alpha-tocopherol impairs 7-ketocholesterol-induced caspase-3-dependent apoptosis involving GSK-3 activation and Mcl-1 degradation on 158N murine oligodendrocytes
-
Ragot, K.; Delmas, D.; Athias, A.; Nury, T.; Baarine, M.; Lizard, G. Alpha-tocopherol impairs 7-ketocholesterol-induced caspase-3-dependent apoptosis involving GSK-3 activation and Mcl-1 degradation on 158N murine oligodendrocytes. Chem. Phys. Lipids 2011, 164, 469-478.
-
(2011)
Chem. Phys. Lipids
, vol.164
, pp. 469-478
-
-
Ragot, K.1
Delmas, D.2
Athias, A.3
Nury, T.4
Baarine, M.5
Lizard, G.6
-
57
-
-
70349920999
-
Oxidation of cholesterol and beta-sitosterol and prevention by natural antioxidants
-
Xu, G.; Guan, L.; Sun, J.; Chen, Z. Y. Oxidation of cholesterol and beta-sitosterol and prevention by natural antioxidants. J. Agric. Food Chem. 2009, 57, 9284-9292.
-
(2009)
J. Agric. Food Chem
, vol.57
, pp. 9284-9292
-
-
Xu, G.1
Guan, L.2
Sun, J.3
Chen, Z.Y.4
-
58
-
-
84869223163
-
Delta-tocopherol reduces lipid accumulation in Niemann-Pick type C1 and wolman cholesterol storage disorders
-
Xu, M.; Liu, K.; Swaroop, M.; Porter, F. D.; Sidhu, R.; Firnkes, S.; Ory, D. S.; Marugan, J. J.; Xiao, J.; Southall, N.; et al. Delta-tocopherol reduces lipid accumulation in Niemann-Pick type C1 and wolman cholesterol storage disorders. J. Biol. Chem. 2012, 287, 39349-39360.
-
(2012)
J. Biol. Chem
, vol.287
, pp. 39349-39360
-
-
Xu, M.1
Liu, K.2
Swaroop, M.3
Porter, F.D.4
Sidhu, R.5
Firnkes, S.6
Ory, D.S.7
Marugan, J.J.8
Xiao, J.9
Southall, N.10
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