-
1
-
-
0037042224
-
Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis
-
Aleksandrov, A. A., Aleksandrov, L. and Riordan, J. R. (2002). Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis. FEBS Lett. 518, 183-188.
-
(2002)
FEBS Lett.
, vol.518
, pp. 183-188
-
-
Aleksandrov, A.A.1
Aleksandrov, L.2
Riordan, J.R.3
-
2
-
-
33847390511
-
Influence of a thiazolidine inhibitor on CFTR single channel activity
-
Aleksandrov, A. A., Fauq, A., Gentzsch, M., Aleksandrov, L. and Riordan, J. R. (2003). Influence of a thiazolidine inhibitor on CFTR single channel activity. Pediatr Pulmonol. Suppl. 25, 202.
-
(2003)
Pediatr Pulmonol. Suppl.
, vol.25
, pp. 202
-
-
Aleksandrov, A.A.1
Fauq, A.2
Gentzsch, M.3
Aleksandrov, L.4
Riordan, J.R.5
-
3
-
-
33745472965
-
MLN64 and MENTHO, two mediators of endosomal cholesterol transport
-
Alpy, F. and Tomasetto, C. (2006). MLN64 and MENTHO, two mediators of endosomal cholesterol transport. Biochem. Soc. Trans. 34, 343-345.
-
(2006)
Biochem. Soc. Trans.
, vol.34
, pp. 343-345
-
-
Alpy, F.1
Tomasetto, C.2
-
4
-
-
0034353227
-
The deltaF508 mutation in the cystic fibrosis transmembrane conductance regulator alters control of essential fatty acid utilization in epithelial cells
-
Bhura-Bandali, F. N., Suh, M., Man, S. F. and Clandinin, M. T. (2000). The deltaF508 mutation in the cystic fibrosis transmembrane conductance regulator alters control of essential fatty acid utilization in epithelial cells. J. Nutr 130, 2870-2875.
-
(2000)
J. Nutr
, vol.130
, pp. 2870-2875
-
-
Bhura-Bandali, F.N.1
Suh, M.2
Man, S.F.3
Clandinin, M.T.4
-
5
-
-
0032900934
-
Characterization of the internalization pathways for the cystic fibrosis transmembrane conductance regulator
-
Bradbury, N. A., Clark, J. A., Watkins, S. C., Widnell, C. C., Smith, H. S., 4th and Bridges, R. J. (1999). Characterization of the internalization pathways for the cystic fibrosis transmembrane conductance regulator. Am. J. Physiol. 276, L659-L668.
-
(1999)
Am. J. Physiol.
, vol.276
-
-
Bradbury, N.A.1
Clark, J.A.2
Watkins, S.C.3
Widnell, C.C.4
Smith IV, H.S.5
Bridges, R.J.6
-
6
-
-
9644259423
-
Mistargeted MRPdeltaF728 mutant is rescued by intracellular GSH
-
Buyse, F., Vandenbranden, M. and Ruysschaert, J. M. (2004). Mistargeted MRPdeltaF728 mutant is rescued by intracellular GSH. FEBS Lett. 578, 145-151.
-
(2004)
FEBS Lett.
, vol.578
, pp. 145-151
-
-
Buyse, F.1
Vandenbranden, M.2
Ruysschaert, J.M.3
-
7
-
-
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. (1997). Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science 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
-
8
-
-
33749073795
-
Cholesterol sensing, trafficking, and esterification
-
Chang, T. Y., Chang, C. C., Ohgami, N. and Yamauchi, Y. (2006). Cholesterol sensing, trafficking, and esterification. Annu. Rev. Cell Dev. Biol. 22, 129-157.
-
(2006)
Annu. Rev. Cell Dev. Biol.
, vol.22
, pp. 129-157
-
-
Chang, T.Y.1
Chang, C.C.2
Ohgami, N.3
Yamauchi, Y.4
-
9
-
-
0027311276
-
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites
-
Chang, X. B., Tabeharani, J. A., Hou, Y. X., Jensen, T. J., Kartner, N., Alon, N., Hanrahan, J. W. and Riordan, J. R. (1993). Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J. Biol. Chem. 268, 11304-11311.
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 11304-11311
-
-
Chang, X.B.1
Tabeharani, J.A.2
Hou, Y.X.3
Jensen, T.J.4
Kartner, N.5
Alon, N.6
Hanrahan, J.W.7
Riordan, J.R.8
-
10
-
-
0030689692
-
ATPase activity of purified multidrug resistance-associated protein
-
Chang, X. B., Hou, Y. X. and Riordan, J. R. (1997). ATPase activity of purified multidrug resistance-associated protein. J. Biol. Chem. 272, 30962-30968.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 30962-30968
-
-
Chang, X.B.1
Hou, Y.X.2
Riordan, J.R.3
-
11
-
-
33750318425
-
Mechanism of cholesterol transfer from the Niemann-Pick type C2 protein to model membranes supports a role in lysosomal cholesterol transport
-
Cheruku, S. R., Xu, Z., Dutia, R., Lobel, P. and Storch, J. (2006). Mechanism of cholesterol transfer from the Niemann-Pick type C2 protein to model membranes supports a role in lysosomal cholesterol transport. J. Biol. Chem. 281, 31594-31604.
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 31594-31604
-
-
Cheruku, S.R.1
Xu, Z.2
Dutia, R.3
Lobel, P.4
Storch, J.5
-
12
-
-
0036083829
-
Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells
-
Choudhury, A., Dominguez, M., Puri, V., Sharma, D. K., Narita, K., Wheatley, C. L., Marks, D. L. and Pagano, R. E. (2002). Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. J. Clin. Invest. 109, 1541-1550.
-
(2002)
J. Clin. Invest.
, vol.109
, pp. 1541-1550
-
-
Choudhury, A.1
Dominguez, M.2
Puri, V.3
Sharma, D.K.4
Narita, K.5
Wheatley, C.L.6
Marks, D.L.7
Pagano, R.E.8
-
13
-
-
4644263342
-
Elevated endosomal cholesterol levels in Niemann-Pick cells inhibit rab4 and perturb membrane recycling
-
Choudhury, A., Sharma, D. K., Marks, D. L. and Pagano, R. E. (2004). Elevated endosomal cholesterol levels in Niemann-Pick cells inhibit rab4 and perturb membrane recycling. Mol. Biol. Cell 15, 4500-4511.
-
(2004)
Mol. Biol. Cell
, vol.15
, pp. 4500-4511
-
-
Choudhury, A.1
Sharma, D.K.2
Marks, D.L.3
Pagano, R.E.4
-
14
-
-
0025310336
-
A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein
-
Cutting, G. R., Kasch, L. M., Rosenstein, B. J., Zielenski, J., Tsui, L. C., Antonarakis, S. E. and Kazazian, H. H., Jr (1990). A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein. Nature 346, 366-369.
-
(1990)
Nature
, vol.346
, pp. 366-369
-
-
Cutting, G.R.1
Kasch, L.M.2
Rosenstein, B.J.3
Zielenski, J.4
Tsui, L.C.5
Antonarakis, S.E.6
Kazazian Jr., H.H.7
-
15
-
-
30144435485
-
The genetics of ATP-binding cassette transporters
-
Dean, M. (2005). The genetics of ATP-binding cassette transporters. Meth. Enzymol. 400, 409-429.
-
(2005)
Meth. Enzymol.
, vol.400
, pp. 409-429
-
-
Dean, M.1
-
16
-
-
84891539797
-
Multidrug resistance protein 1 (ABCC1)
-
In (ed. I. B. Holland, S. P. C. Cole, K. Kuchler and C. F. Higgins), New York: Academic Press
-
Deeley, R. G. and Cole, S. P. C. (2003). Multidrug resistance protein 1 (ABCC1). In ABC Proteins (ed. I. B. Holland, S. P. C. Cole, K. Kuchler and C. F. Higgins), pp. 393-422. New York: Academic Press.
-
(2003)
ABC Proteins
, pp. 393-422
-
-
Deeley, R.G.1
Cole, S.P.C.2
-
17
-
-
3042760943
-
Disturted cholesterol traffic but normal proteolytic function in LAMP-1 /LAMP-2 double-deficient fibroblasts
-
Eskelinen, E. L., Schmidt, C. K., Neu, S., Willenborg, M., Fuertes, G., Salvador, N., Tanaka, Y., Lullmann-Rauch, R., Hartmann, D., Heeren, J. et al. (2004). Disturted cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts. Mol. Biol. Cell 15, 3132-3145.
-
(2004)
Mol. Biol. Cell
, vol.15
, pp. 3132-3145
-
-
Eskelinen, E.L.1
Schmidt, C.K.2
Neu, S.3
Willenborg, M.4
Fuertes, G.5
Salvador, N.6
Tanaka, Y.7
Lullmann-Rauch, R.8
Hartmann, D.9
Heeren, J.10
-
18
-
-
10744223051
-
Association of cystic fibrosis with abnormalities in fatty acid metabolism
-
Freedman, S. D., Blanco, F. G., Zaman, M. M., Shea, J. C., Ollero, M., Hopper, I. K., Weed, D. A., Gelrud, A., Regan, M. M., Laposata, M. et al. (2004). Association of cystic fibrosis with abnormalities in fatty acid metabolism. N. Engl. J. Med. 350, 560-569.
-
(2004)
N. Engl. J. Med.
, vol.350
, pp. 560-569
-
-
Freedman, S.D.1
Blanco, F.G.2
Zaman, M.M.3
Shea, J.C.4
Ollero, M.5
Hopper, I.K.6
Weed, D.A.7
Gelrud, A.8
Regan, M.M.9
Laposata, M.10
-
19
-
-
0035847015
-
Localization of sequences within the C-terminal domain of the cystic fibrosis transmembrane conductance regulator which impact maturation and stability
-
Gentzsch, M. and Riordan, J. R. (2001). Localization of sequences within the C-terminal domain of the cystic fibrosis transmembrane conductance regulator which impact maturation and stability. J. Biol. Chem. 276, 1291-1298.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 1291-1298
-
-
Gentzsch, M.1
Riordan, J.R.2
-
20
-
-
0036714504
-
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications
-
Gentzsch, M., Aleksandrov, A., Aleksandrov, L. and Riordan, J. R. (2002). Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications. Biochem. J. 366, 541-548.
-
(2002)
Biochem. J.
, vol.366
, pp. 541-548
-
-
Gentzsch, M.1
Aleksandrov, A.2
Aleksandrov, L.3
Riordan, J.R.4
-
21
-
-
2542480784
-
Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator
-
Gentzsch, M., Chang, X. B., Cui, L., Wu, Y., Ozols, V. V., Chourthury, A., Pagano, R. E. and Riordan, J. R. (2004). Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator. Mol. Biol. Cell 15, 2684-2696.
-
(2004)
Mol. Biol. Cell
, vol.15
, pp. 2684-2696
-
-
Gentzsch, M.1
Chang, X.B.2
Cui, L.3
Wu, Y.4
Ozols, V.V.5
Chourthury, A.6
Pagano, R.E.7
Riordan, J.R.8
-
22
-
-
0025912486
-
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2
-
Gregory, R. J., Rich, D. F., Cheng, S. H., Souza, D. W., Paul, S., Manavalan, P., Anderson, M. F., Welsh, M. J. and Smith, A. E. (1991). Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2. Mol. Cell. Biol. 11, 3886-3893.
-
(1991)
Mol. Cell. Biol.
, vol.11
, pp. 3886-3893
-
-
Gregory, R.J.1
Rich, D.F.2
Cheng, S.H.3
Souza, D.W.4
Paul, S.5
Manavalan, P.6
Anderson, M.F.7
Welsh, M.J.8
Smith, A.E.9
-
23
-
-
0033618404
-
C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation
-
Haardt, M., Benharouga, M., Lechardeur, D., Kartner, N. and Lukacs, G. L. (1999). C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation, J. Biol. Chem. 274, 21873-21877.
-
(1999)
J. Biol. Chem.
, vol.274
, pp. 21873-21877
-
-
Haardt, M.1
Benharouga, M.2
Lechardeur, D.3
Kartner, N.4
Lukacs, G.L.5
-
24
-
-
0346636602
-
The cystic fibrosis transmembrane conductance regulator (ABCC7)
-
In (ed. I. B. Hollantl, S. P. C. Cole, K. Kuchler and C. F Higgins), New York: Academic Press
-
Hanraham, J. W., Gentzsch, M. and Riordan, J. R. (2W3). The cystic fibrosis transmembrane conductance regulator (ABCC7). In ABC Proteins (ed. I. B. Hollantl, S. P. C. Cole, K. Kuchler and C. F Higgins), pp. 589-618. New York: Academic Press.
-
(2003)
ABC Proteins
, pp. 589-618
-
-
Hanraham, J.W.1
Gentzsch, M.2
Riordan, J.R.3
-
25
-
-
0034617097
-
Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1
-
Hou, Y., Cui, L., Riordan, J. R. and Chang, X. (2000). Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1. J. Biol. Chem. 275, 20280-20287.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 20280-20287
-
-
Hou, Y.1
Cui, L.2
Riordan, J.R.3
Chang, X.4
-
26
-
-
1442284337
-
Caveolins and membrane cholesterol
-
Ikonen, E., Heino, S. and Lusa, S. (2004). Caveolins and membrane cholesterol. Biochem. Soc. Trans. 32, 121-123.
-
(2004)
Biochem. Soc. Trans.
, vol.32
, pp. 121-123
-
-
Ikonen, E.1
Heino, S.2
Lusa, S.3
-
27
-
-
0346103721
-
Localization of cystic fibrosis transmembrane conductance regulator to lipid rafts of epithelial cells is required for Pseudomonas aeruginosa-induced cellular activation
-
Kowalski, M. P. and Pier, G. B. (2004). Localization of cystic fibrosis transmembrane conductance regulator to lipid rafts of epithelial cells is required for Pseudomonas aeruginosa-induced cellular activation. J. Immunol. 172, 418-425.
-
(2004)
J. Immunol.
, vol.172
, pp. 418-425
-
-
Kowalski, M.P.1
Pier, G.B.2
-
28
-
-
0022869016
-
Type C Niemann-Pick disease. Abnormal metabolism of low density lipoprotein in homozygous and heterozygous fibroblasts
-
Kruth, H. S., Comly, M. E., Butler, J. D., Vanier, M. T., Fink, J. K., Wenger, D. A. Patel, S. and Pentchev, F. G. (1986). Type C Niemann-Pick disease. Abnormal metabolism of low density lipoprotein in homozygous and heterozygous fibroblasts. J. Biol. Chem. 261, 16769-16774.
-
(1986)
J. Biol. Chem.
, vol.261
, pp. 16769-16774
-
-
Kruth, H.S.1
Comly, M.E.2
Butler, J.D.3
Vanier, M.T.4
Fink, J.K.5
Wenger, D.A.6
Patel, S.7
Pentchev, F.G.8
-
29
-
-
0027478252
-
Lipoprotein abnormalities associated with cholesteryl ester transfer activity in cystic fibrosis patients: The role of essential fatty acid deficiency
-
Levy, E., Roy, C., Lacaille, F., Lambert, M., Messier, M., Gavino, V., Lepage, G. and Thibault, L. (1993). Lipoprotein abnormalities associated with cholesteryl ester transfer activity in cystic fibrosis patients: the role of essential fatty acid deficiency. Am. J. Clin. Nutr. 57, 573-579.
-
(1993)
Am. J. Clin. Nutr.
, vol.57
, pp. 573-579
-
-
Levy, E.1
Roy, C.2
Lacaille, F.3
Lambert, M.4
Messier, M.5
Gavino, V.6
Lepage, G.7
Thibault, L.8
-
30
-
-
0027244942
-
Giantin, a novel conserved Golgi membrane protein containing a cytoplasmic domain of at least 350 kDa
-
Linstedt, A. D. and Hauri, H. P. (1993). Giantin, a novel conserved Golgi membrane protein containing a cytoplasmic domain of at least 350 kDa. Mol. Biol. Cell 4, 679-693.
-
(1993)
Mol. Biol. Cell
, vol.4
, pp. 679-693
-
-
Linstedt, A.D.1
Hauri, H.P.2
-
31
-
-
0032401771
-
Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome
-
Loo, M. A., Jensen, T. J., Cui, L., Hou, Y., Chang, X. B. and Riordan, J. R. (1998). Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome. EMBO J. 17, 6879-6887.
-
(1998)
EMBO J.
, vol.17
, pp. 6879-6887
-
-
Loo, M.A.1
Jensen, T.J.2
Cui, L.3
Hou, Y.4
Chang, X.B.5
Riordan, J.R.6
-
32
-
-
0027380236
-
The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells
-
Lukacs, G. L., Chang, X. B., Bear, C., Kartner, N., Mohamed, A., Riordan, J. R. and Grinstein, S. (1993). The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells. J. Biol. Chem. 268, 21592-21598.
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 21592-21598
-
-
Lukacs, G.L.1
Chang, X.B.2
Bear, C.3
Kartner, N.4
Mohamed, A.5
Riordan, J.R.6
Grinstein, S.7
-
33
-
-
0030712030
-
Constitutive internalization of cystic fibrosis transmembrane conductance regulator occurs via clathrin-dependent endocytosis and is regulated by protein phosphorylation
-
Lukaes, G. L., Segal, G., Kartner, N., Grinstein, S. and Zhang, F. (1997). Constitutive internalization of cystic fibrosis transmembrane conductance regulator occurs via clathrin-dependent endocytosis and is regulated by protein phosphorylation. Biochem. J. 328, 353-361.
-
(1997)
Biochem. J.
, vol.328
, pp. 353-361
-
-
Lukaes, G.L.1
Segal, G.2
Kartner, N.3
Grinstein, S.4
Zhang, F.5
-
34
-
-
0036896008
-
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion
-
Ma, T., Thiagarajah, J. R., Yang, H., Sonawane, N. D., Folli, C., Galietta, L. J. and Verkman, A. S. (2002). Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion. J. Clin. Invest. 110, 1651-1658.
-
(2002)
J. Clin. Invest.
, vol.110
, pp. 1651-1658
-
-
Ma, T.1
Thiagarajah, J.R.2
Yang, H.3
Sonawane, N.D.4
Folli, C.5
Galietta, L.J.6
Verkman, A.S.7
-
35
-
-
19444384638
-
Use of fluorescent sphingolipid analogs to study lipid transport along the endocytic pathway
-
Marks, D. L., Singh, R. D., Choudhury, A., Wheatley, C. L. and Pagano, R. E. (2005). Use of fluorescent sphingolipid analogs to study lipid transport along the endocytic pathway. Methods 36, 186-195.
-
(2005)
Methods
, vol.36
, pp. 186-195
-
-
Marks, D.L.1
Singh, R.D.2
Choudhury, A.3
Wheatley, C.L.4
Pagano, R.E.5
-
36
-
-
0028335260
-
Internalization and sorting of a fluorescent analogue of glucosylceramide to the Golgi apparatus of human skin fibroblasts: Utilization of endocytic and nonendocytic transport mechanisms
-
Martin, O. C. and Pagano, R. E. (1994). Internalization and sorting of a fluorescent analogue of glucosylceramide to the Golgi apparatus of human skin fibroblasts: utilization of endocytic and nonendocytic transport mechanisms. J. Cell Biol. 125, 769-781.
-
(1994)
J. Cell Biol.
, vol.125
, pp. 769-781
-
-
Martin, O.C.1
Pagano, R.E.2
-
37
-
-
28444479505
-
Role of cholesterol and lipid organization in disease
-
Maxfield, F. R. and Tabas, I. (2005). Role of cholesterol and lipid organization in disease. Nature 438, 612-621.
-
(2005)
Nature
, vol.438
, pp. 612-621
-
-
Maxfield, F.R.1
Tabas, I.2
-
38
-
-
4744376060
-
Lipid and cholesterol trafficking in NPC
-
Mukherjee, S. and Maxfield, F. R. (2004). Lipid and cholesterol trafficking in NPC. Biochim. Biophys. Acta 1685, 28-37.
-
(2004)
Biochim. Biophys. Acta
, vol.1685
, pp. 28-37
-
-
Mukherjee, S.1
Maxfield, F.R.2
-
39
-
-
0029595302
-
Characterization of a cis-Golgi matrix protein, GM130
-
Nakamura, N., Rabouille, C., Watson, R., Nilsson, T., Hui, N., Slusarewicz, F., Kreis, T. E. and Warren, G. (1995). Characterization of a cis-Golgi matrix protein, GM130. J. Cell Biol. 131, 1715-1726.
-
(1995)
J. Cell Biol.
, vol.131
, pp. 1715-1726
-
-
Nakamura, N.1
Rabouille, C.2
Watson, R.3
Nilsson, T.4
Hui, N.5
Slusarewicz, F.6
Kreis, T.E.7
Warren, G.8
-
40
-
-
24644494640
-
Protein transduction of Rab9 in Niemann-Pick C cells reduces cholesterol storage
-
Narita, K., Choudbury, A., Dobrenis, K., Sharma, D. K., Holicky, E. L., Marks, D. L., Walkley, S. U. and Pagano, R. E. (2005). Protein transduction of Rab9 in Niemann-Pick C cells reduces cholesterol storage. FASEB J. 19, 1558-1560.
-
(2005)
FASEB J.
, vol.19
, pp. 1558-1560
-
-
Narita, K.1
Choudbury, A.2
Dobrenis, K.3
Sharma, D.K.4
Holicky, E.L.5
Marks, D.L.6
Walkley, S.U.7
Pagano, R.E.8
-
41
-
-
0034704245
-
Identification of HE1 as the second gene of Niemann-Pick C disease
-
Naureckiene, S., Sleat, A E., Lackland, H., Fensom, A., Vanier, M. T., Wattiaux, R., Jadot, M. and Lobel, P. (2000). Identification of HE1 as the second gene of Niemann-Pick C disease. Science 290, 2298-2301.
-
(2000)
Science
, vol.290
, pp. 2298-2301
-
-
Naureckiene, S.1
Sleat, A.E.2
Lackland, H.3
Fensom, A.4
Vanier, M.T.5
Wattiaux, R.6
Jadot, M.7
Lobel, P.8
-
42
-
-
33746911531
-
Cholesterol depletion facilitates ubiquitylation of NPCI and its association with SKD1/Vps4
-
Ohsaki, Y., Sugimoto, Y., Suzuki, M., Hosokawa, H., Yoshimori, T., Davies, J. P., Ioannou, Y. A., Vanier, M. T., Ohno, K. and Ninomiya, H. (2006). Cholesterol depletion facilitates ubiquitylation of NPCI and its association with SKD1/Vps4. J Cell Sci. 119, 2643-2653.
-
(2006)
J Cell Sci.
, vol.119
, pp. 2643-2653
-
-
Ohsaki, Y.1
Sugimoto, Y.2
Suzuki, M.3
Hosokawa, H.4
Yoshimori, T.5
Davies, J.P.6
Ioannou, Y.A.7
Vanier, M.T.8
Ohno, K.9
Ninomiya, H.10
-
43
-
-
33745477974
-
The OSBP-related proteins (ORPs): Global sterol sensors for co-ordination of cellular lipid metabolism, membrane trafficking and signalling processes?
-
Olkkonen, V M., Johansson, M., Suchanek, M., Yan, D., Hynynen, R., Elmholm, C., Jauhiainen, M., Thiele, C. and Lehto, M. (2006). The OSBP-related proteins (ORPs): global sterol sensors for co-ordination of cellular lipid metabolism, membrane trafficking and signalling processes? Biochem. Soc. Trans. 34, 389-391.
-
(2006)
Biochem. Soc. Trans.
, vol.34
, pp. 389-391
-
-
Olkkonen, V.M.1
Johansson, M.2
Suchanek, M.3
Yan, D.4
Hynynen, R.5
Elmholm, C.6
Jauhiainen, M.7
Thiele, C.8
Lehto, M.9
-
44
-
-
4544375506
-
Caveolin-stabilized membrane domains as multifunctional transport and sorting devices in endocytic membrane traffic
-
Pelkmans, L., Burli, T., Zerial, M. and Helenius, A. (2004). Caveolin-stabilized membrane domains as multifunctional transport and sorting devices in endocytic membrane traffic. Cell 118, 767-780.
-
(2004)
Cell
, vol.118
, pp. 767-780
-
-
Pelkmans, L.1
Burli, T.2
Zerial, M.3
Helenius, A.4
-
45
-
-
22144476236
-
Mechanisms of lipid malabsorption in Cystic Fibrosis: The impact of essential fatty acids deficiency
-
Peretti, N., Marcil, V., Drouin, E. and Levy, E. (2005). Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency. Nutr. Metab. Lond 2, 11.
-
(2005)
Nutr. Metab. Lond
, vol.2
, pp. 11
-
-
Peretti, N.1
Marcil, V.2
Drouin, E.3
Levy, E.4
-
46
-
-
0033203501
-
Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases
-
Puri, V., Watanabe, R., Dominguez, M., Sun, X., Wheatley, C. L., Marks, D. L. and Pagano, R. E. (1999). Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases. Nat. Cell Biol. 1, 386-388.
-
(1999)
Nat. Cell Biol.
, vol.1
, pp. 386-388
-
-
Puri, V.1
Watanabe, R.2
Dominguez, M.3
Sun, X.4
Wheatley, C.L.5
Marks, D.L.6
Pagano, R.E.7
-
47
-
-
0038756797
-
Sphingolipid storage induces accumulation of intracellular cholesterol by stimulating SREBP-1 cleavage
-
Puri, V., Jefferson, J. R., Singh, R. D., Wheatley, C. L., Marks, D. L. and Pagano, R. E. (2003). Sphingolipid storage induces accumulation of intracellular cholesterol by stimulating SREBP-1 cleavage. J. Biol. Chem. 278, 20961-20970.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 20961-20970
-
-
Puri, V.1
Jefferson, J.R.2
Singh, R.D.3
Wheatley, C.L.4
Marks, D.L.5
Pagano, R.E.6
-
48
-
-
0032913809
-
Physiological basis of cystic fibrosis: A historical perspective
-
Quinton, P. M. (1999). Physiological basis of cystic fibrosis: a historical perspective. Physiol. Rev. 79, S3-S22.
-
(1999)
Physiol. Rev.
, vol.79
-
-
Quinton, P.M.1
-
49
-
-
33745018593
-
Dynamin is involved in endolysosomal cholesterol delivery to the endoplasmic reticulum: Role in cholesterol homeostasis
-
Robinet, P., Fradagrada, A., Monier, M. N., Marchetti, M., Cogny, A., Moatti, N., Paul, J. L., Vedie, B. and Lamaze, C. (2006). Dynamin is involved in endolysosomal cholesterol delivery to the endoplasmic reticulum: role in cholesterol homeostasis. Traffic 7, 811-823.
-
(2006)
Traffic
, vol.7
, pp. 811-823
-
-
Robinet, P.1
Fradagrada, A.2
Monier, M.N.3
Marchetti, M.4
Cogny, A.5
Moatti, N.6
Paul, J.L.7
Vedie, B.8
Lamaze, C.9
-
50
-
-
0028906638
-
cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation
-
Seibert, F. S., Talacharani, J. A., Chang, X. B., Dulhanty, A. M., Mathews, C., Hanrahan, J. W. and Riordan, J. R. (1995). cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation. J Biol. Chem. 270, 2158-2162.
-
(1995)
J Biol. Chem.
, vol.270
, pp. 2158-2162
-
-
Seibert, F.S.1
Talacharani, J.A.2
Chang, X.B.3
Dulhanty, A.M.4
Mathews, C.5
Hanrahan, J.W.6
Riordan, J.R.7
-
51
-
-
0027405520
-
The molecular biology of cystic fibrosis
-
Sierra, T. J. and Collins, F. S. (1993). The molecular biology of cystic fibrosis. Annu. Rev. Med. 44, 133-144.
-
(1993)
Annu. Rev. Med.
, vol.44
, pp. 133-144
-
-
Sierra, T.J.1
Collins, F.S.2
-
52
-
-
0035937847
-
Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments
-
Sharma, M., Benharouga, M., Hu, W. and Lukaes, G. L. (2001). Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments. J. Biol. Chem. 276, 8942-8950.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 8942-8950
-
-
Sharma, M.1
Benharouga, M.2
Hu, W.3
Lukaes, G.L.4
-
53
-
-
12144287602
-
Misfolding diverts CFTR from recycling to degradation: Quality control at early endosomes
-
Sharma, M., Pampinella, F., Nemes, C., Benharouga, M., So, J., Du, K., Bache, K. G., Papsin, B., Zerangue, N., Stenmark, H. et al. (2004). Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes. J Cell Biol. 164, 923-933.
-
(2004)
J Cell Biol.
, vol.164
, pp. 923-933
-
-
Sharma, M.1
Pampinella, F.2
Nemes, C.3
Benharouga, M.4
So, J.5
Du, K.6
Bache, K.G.7
Papsin, B.8
Zerangue, N.9
Stenmark, H.10
-
54
-
-
0034332917
-
Jamming the endosomal system: Lipid rafts and lysosomal storage diseases
-
Simons, K. and Gruenberg, J. (2000). Jamming the endosomal system: lipid rafts and lysosomal storage diseases. Trends Cell Biol. 10, 459-462.
-
(2000)
Trends Cell Biol.
, vol.10
, pp. 459-462
-
-
Simons, K.1
Gruenberg, J.2
-
55
-
-
11144355005
-
Genetic evidence for nomedundant functional cooperativity between NPCI and NPC!2 in lipid transport
-
Sleat, D. E., Wiseman, J. A., El-Banna, M., Price, S, M, Verot, L., Shen, M. M., Tint, G. S., Vanier, M. T., Walkley, S. U. and Lobel, P. (2004). Genetic evidence for nomedundant functional cooperativity between NPCI and NPC!2 in lipid transport. Proc. Natl. Acad. Sci. USA 101, 5886-5891.
-
(2004)
Proc. Natl. Acad. Sci. USA
, vol.101
, pp. 5886-5891
-
-
Sleat, D.E.1
Wiseman, J.A.2
El-Banna, M.3
Price, S.M.4
Verot, L.5
Shen, M.M.6
Tint, G.S.7
Vanier, M.T.8
Walkley, S.U.9
Lobel, P.10
-
56
-
-
0023850575
-
Type C Niemann-Pick disease. Lysosomal accumulation and defective intraceflular mobilization of low density lipoprotein cholesterol
-
Sokol, J., Blanchette-Mackie, J., Kruth, H. S., Dwyer, N. K., Amende, L. M., Butler, J. D., Robinson, E., Patel, S., Brady, R. O., Condy, M. E. et al. (1988). Type C Niemann-Pick disease. Lysosomal accumulation and defective intraceflular mobilization of low density lipoprotein cholesterol. J. Biol. Chem. 263, 3411-3417.
-
(1988)
J. Biol. Chem.
, vol.263
, pp. 3411-3417
-
-
Sokol, J.1
Blanchette-Mackie, J.2
Kruth, H.S.3
Dwyer, N.K.4
Amende, L.M.5
Butler, J.D.6
Robinson, E.7
Patel, S.8
Brady, R.O.9
Condy, M.E.10
-
57
-
-
0035192463
-
Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis
-
Strandvik, B., Gronowitz, E., Enlund, F., Martinsson, T. and Wahistrom, J. (2001). Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis. J. Pediatr 139, 650-655.
-
(2001)
J. Pediatr
, vol.139
, pp. 650-655
-
-
Strandvik, B.1
Gronowitz, E.2
Enlund, F.3
Martinsson, T.4
Wahistrom, J.5
-
58
-
-
7744231974
-
Mechanistic similarities between cultured cell models of cystic fibrosis and niemann-pick type C
-
White, N. M., Corey, D. A. and Kelley, T. J. (2004). Mechanistic similarities between cultured cell models of cystic fibrosis and niemann-pick type C. An J. Respir. Cell Mol. Biol. 31, 538-543.
-
(2004)
An J. Respir. Cell Mol. Biol.
, vol.31
, pp. 538-543
-
-
White, N.M.1
Corey, D.A.2
Kelley, T.J.3
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