-
2
-
-
34948886661
-
Progressive oxidative damage in the central nervous system of a murine model for juvenile Batten disease
-
Benedict J.W., Sommers C.A., Pearce D.A. Progressive oxidative damage in the central nervous system of a murine model for juvenile Batten disease. Journal of Neuroscience Research 2007, 85:2882-2891.
-
(2007)
Journal of Neuroscience Research
, vol.85
, pp. 2882-2891
-
-
Benedict, J.W.1
Sommers, C.A.2
Pearce, D.A.3
-
3
-
-
77957347060
-
Alpha-synuclein promotes SNARE-complex assembly in vivo and in vitro
-
Burré J., Sharma M., Tsetsenis T., Buchman V., Etherton M.R., Südhof T.C. Alpha-synuclein promotes SNARE-complex assembly in vivo and in vitro. Science 2010, 329:1663-1667.
-
(2010)
Science
, vol.329
, pp. 1663-1667
-
-
Burré, J.1
Sharma, M.2
Tsetsenis, T.3
Buchman, V.4
Etherton, M.R.5
Südhof, T.C.6
-
4
-
-
79958052481
-
Drug targeting of sphingolipid metabolism: sphingomyelinases and ceramidases
-
Canals D., Perry D.M., Jenkins R.W., Hannun Y.A. Drug targeting of sphingolipid metabolism: sphingomyelinases and ceramidases. British Journal of Pharmacology 2011, 163:694-712.
-
(2011)
British Journal of Pharmacology
, vol.163
, pp. 694-712
-
-
Canals, D.1
Perry, D.M.2
Jenkins, R.W.3
Hannun, Y.A.4
-
5
-
-
33745976466
-
Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis
-
Cao Y. Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis. Journal of Biological Chemistry 2006, 281:20483-20493.
-
(2006)
Journal of Biological Chemistry
, vol.281
, pp. 20483-20493
-
-
Cao, Y.1
-
6
-
-
27544507306
-
Alpha-synuclein cooperates with CSPalpha in preventing neurodegeneration
-
Chandra S., Gallardo G., Fernández-Chacón R., Schlüter O.M., Südhof T.C. Alpha-synuclein cooperates with CSPalpha in preventing neurodegeneration. Cell 2005, 123:383-396.
-
(2005)
Cell
, vol.123
, pp. 383-396
-
-
Chandra, S.1
Gallardo, G.2
Fernández-Chacón, R.3
Schlüter, O.M.4
Südhof, T.C.5
-
8
-
-
33746533924
-
Synuclein blocks ER-Golgi traffic and Rab1 rescues neuron loss in Parkinson's models
-
Cooper A.A. Synuclein blocks ER-Golgi traffic and Rab1 rescues neuron loss in Parkinson's models. Science 2006, 313:324-328.
-
(2006)
Science
, vol.313
, pp. 324-328
-
-
Cooper, A.A.1
-
9
-
-
79954534373
-
Molecular basis for the glycosphingolipid-binding specificity of α-synuclein: key role of tyrosine 39 in membrane insertion
-
Fantini J., Yahi N. Molecular basis for the glycosphingolipid-binding specificity of α-synuclein: key role of tyrosine 39 in membrane insertion. Journal of Molecular Biology 2011, 408:654-669.
-
(2011)
Journal of Molecular Biology
, vol.408
, pp. 654-669
-
-
Fantini, J.1
Yahi, N.2
-
10
-
-
84875619492
-
Cell cycle arrest in Batten disease lymphoblast cells
-
Kang S., Kim J.-B., Heo T.H., Kim S.-J. Cell cycle arrest in Batten disease lymphoblast cells. Gene 2013, 519:245-250.
-
(2013)
Gene
, vol.519
, pp. 245-250
-
-
Kang, S.1
Kim, J.-B.2
Heo, T.H.3
Kim, S.-J.4
-
11
-
-
84875930662
-
Batten disease is linked to altered expression of mitochondria-related metabolic molecules
-
Kang S., Seo J.H., Heo T.-H., Kim S.-J. Batten disease is linked to altered expression of mitochondria-related metabolic molecules. Neurochemistry International 2013, 62:931-935.
-
(2013)
Neurochemistry International
, vol.62
, pp. 931-935
-
-
Kang, S.1
Seo, J.H.2
Heo, T.-H.3
Kim, S.-J.4
-
12
-
-
84870654471
-
N-acetylcysteine normalizes the urea cycle and DNA repair in cells from patients with Batten disease
-
Kim J.-B., Lim N., Kim S.-J., Heo T.-H. N-acetylcysteine normalizes the urea cycle and DNA repair in cells from patients with Batten disease. Cell Biochemistry and Function 2012, 30:677-682.
-
(2012)
Cell Biochemistry and Function
, vol.30
, pp. 677-682
-
-
Kim, J.-B.1
Lim, N.2
Kim, S.-J.3
Heo, T.-H.4
-
13
-
-
84862598619
-
Alpha-synuclein aggregation involves a bafilomycin A1-sensitive autophagy pathway
-
Klucken J., et al. Alpha-synuclein aggregation involves a bafilomycin A1-sensitive autophagy pathway. Autophagy 2012, 8:754-766.
-
(2012)
Autophagy
, vol.8
, pp. 754-766
-
-
Klucken, J.1
-
14
-
-
28244493702
-
Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease)
-
Koike M., et al. Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease). The American Journal of Pathology 2005, 167:1713-1728.
-
(2005)
The American Journal of Pathology
, vol.167
, pp. 1713-1728
-
-
Koike, M.1
-
15
-
-
33646831810
-
Regulation of autophagy by sphingosine kinase 1 and its role in cell survival during nutrient starvation
-
Lavieu G., et al. Regulation of autophagy by sphingosine kinase 1 and its role in cell survival during nutrient starvation. The Journal of Biological Chemistry 2006, 281:8518-8527.
-
(2006)
The Journal of Biological Chemistry
, vol.281
, pp. 8518-8527
-
-
Lavieu, G.1
-
16
-
-
84862602473
-
Autophagy in lysosomal storage disorders
-
Lieberman A.P., Puertollano R., Raben N., Slaugenhaupt S., Walkley S.U., Ballabio A. Autophagy in lysosomal storage disorders. Autophagy 2012, 8:719-730.
-
(2012)
Autophagy
, vol.8
, pp. 719-730
-
-
Lieberman, A.P.1
Puertollano, R.2
Raben, N.3
Slaugenhaupt, S.4
Walkley, S.U.5
Ballabio, A.6
-
17
-
-
33847022701
-
GM1 specifically interacts with α-synuclein and inhibits fibrillation †
-
Martinez Z., Zhu M., Han S., Fink A.L. GM1 specifically interacts with α-synuclein and inhibits fibrillation †. Biochemistry 2007, 46:1868-1877.
-
(2007)
Biochemistry
, vol.46
, pp. 1868-1877
-
-
Martinez, Z.1
Zhu, M.2
Han, S.3
Fink, A.L.4
-
18
-
-
79960009804
-
Gaucher disease glucocerebrosidase and α-synuclein form a bidirectional pathogenic loop in synucleinopathies
-
Mazzulli J.R., et al. Gaucher disease glucocerebrosidase and α-synuclein form a bidirectional pathogenic loop in synucleinopathies. Cell 2011, 146:37-52.
-
(2011)
Cell
, vol.146
, pp. 37-52
-
-
Mazzulli, J.R.1
-
19
-
-
34347398122
-
Nitric oxide signaling is disrupted in the yeast model for Batten disease
-
Osório N.S., et al. Nitric oxide signaling is disrupted in the yeast model for Batten disease. Molecular Biology of the Cell 2007, 18:2755-2767.
-
(2007)
Molecular Biology of the Cell
, vol.18
, pp. 2755-2767
-
-
Osório, N.S.1
-
20
-
-
24644471460
-
Cell death pathways in juvenile Batten disease
-
Persaud-Sawin D.A., Boustany R.-M.N. Cell death pathways in juvenile Batten disease. Apoptosis 2005, 10:973-985.
-
(2005)
Apoptosis
, vol.10
, pp. 973-985
-
-
Persaud-Sawin, D.A.1
Boustany, R.-M.N.2
-
22
-
-
0030833658
-
Upregulation of Bcl-2 and elevation of ceramide in batten disease
-
Puranam K., et al. Upregulation of Bcl-2 and elevation of ceramide in batten disease. Neuropediatrics 2007, 28:37-41.
-
(2007)
Neuropediatrics
, vol.28
, pp. 37-41
-
-
Puranam, K.1
-
23
-
-
0033810454
-
GD3 ganglioside directly targets mitochondria in a bcl-2-controlled fashion
-
Rippo M.R., et al. GD3 ganglioside directly targets mitochondria in a bcl-2-controlled fashion. FASEB Journal 2000, 14:2047-2054.
-
(2000)
FASEB Journal
, vol.14
, pp. 2047-2054
-
-
Rippo, M.R.1
-
24
-
-
45849149313
-
CLN3p impacts galactosylceramide transport, raft morphology, and lipid content
-
Rusyn E., Mousallem T., Persaud-Sawin D.-A., Miller S., Boustany R.-M.N. CLN3p impacts galactosylceramide transport, raft morphology, and lipid content. Pediatric Research 2008, 63:625-631.
-
(2008)
Pediatric Research
, vol.63
, pp. 625-631
-
-
Rusyn, E.1
Mousallem, T.2
Persaud-Sawin, D.-A.3
Miller, S.4
Boustany, R.-M.N.5
-
25
-
-
0033821703
-
Induction of neuronal death by alpha-synuclein
-
Saha A.R., Ninkina N.N., Hanger D.P., Anderton B.H., Davies A.M., Buchman V.L. Induction of neuronal death by alpha-synuclein. The European Journal of Neuroscience 2000, 12:3073-3077.
-
(2000)
The European Journal of Neuroscience
, vol.12
, pp. 3073-3077
-
-
Saha, A.R.1
Ninkina, N.N.2
Hanger, D.P.3
Anderton, B.H.4
Davies, A.M.5
Buchman, V.L.6
-
26
-
-
0345714776
-
Aberrant sphingomyelin/ceramide metabolic-induced neuronal endosomal/lysosomal dysfunction: potential pathological consequences in age-related neurodegeneration
-
Soreghan B., Thomas S.N., Yang A.J. Aberrant sphingomyelin/ceramide metabolic-induced neuronal endosomal/lysosomal dysfunction: potential pathological consequences in age-related neurodegeneration. Advanced Drug Delivery Reviews 2003, 55:1515-1524.
-
(2003)
Advanced Drug Delivery Reviews
, vol.55
, pp. 1515-1524
-
-
Soreghan, B.1
Thomas, S.N.2
Yang, A.J.3
-
28
-
-
0038298898
-
Neuronal accumulation of alpha- and beta-synucleins in the brain of a GM2 gangliosidosis mouse model
-
Suzuki K., et al. Neuronal accumulation of alpha- and beta-synucleins in the brain of a GM2 gangliosidosis mouse model. Neuroreport 2003, 14:551-554.
-
(2003)
Neuroreport
, vol.14
, pp. 551-554
-
-
Suzuki, K.1
-
29
-
-
79955381027
-
The Batten disease gene CLN3 is required for the response to oxidative stress
-
Tuxworth R.I., Chen H., Vivancos V., Carvajal N., Huang X., Tear G. The Batten disease gene CLN3 is required for the response to oxidative stress. Human Molecular Genetics 2011, 20:2037-2047.
-
(2011)
Human Molecular Genetics
, vol.20
, pp. 2037-2047
-
-
Tuxworth, R.I.1
Chen, H.2
Vivancos, V.3
Carvajal, N.4
Huang, X.5
Tear, G.6
-
30
-
-
34548620297
-
Neuropathology, biochemistry, and biophysics of alpha-synuclein aggregation
-
Uversky V.N. Neuropathology, biochemistry, and biophysics of alpha-synuclein aggregation. Journal of Neurochemistry 2007, 103:17-37.
-
(2007)
Journal of Neurochemistry
, vol.103
, pp. 17-37
-
-
Uversky, V.N.1
-
31
-
-
38849146956
-
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 S.J., Zhang Z., Tsai P.C., Wisniewski K.E., Mukherjee A.B. ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones. Human Molecular Genetics 2007, 17:469-477.
-
(2007)
Human Molecular Genetics
, 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
-
32
-
-
77953499076
-
Multi-system disorders of glycosphingolipid and ganglioside metabolism
-
Xu Y.H., Barnes S., Sun Y., Grabowski G.A. Multi-system disorders of glycosphingolipid and ganglioside metabolism. The Journal of Lipid Research 2010, 51:1643-1675.
-
(2010)
The Journal of Lipid Research
, vol.51
, pp. 1643-1675
-
-
Xu, Y.H.1
Barnes, S.2
Sun, Y.3
Grabowski, G.A.4
-
33
-
-
84871250128
-
Sphingolipids: regulators of crosstalk between apoptosis and autophagy
-
Young M.M., Kester M., Wang H.-G. Sphingolipids: regulators of crosstalk between apoptosis and autophagy. The Journal of Lipid Research 2013, 54:5-19.
-
(2013)
The Journal of Lipid Research
, vol.54
, pp. 5-19
-
-
Young, M.M.1
Kester, M.2
Wang, H.-G.3
|