-
1
-
-
0000831301
-
Niemann-Pick disease type C: A lipid trafficking disorder
-
C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editors. McGraw-Hill, New York
-
Patterson, M. C., M. T. Vanier, K. Suzuki, J. A. Morris, E. D. Carstea, E. D. Neufeld, E. J. Blanchette-Mackie, and P. G. Pentchev. 2001. Niemann-Pick disease type C: a lipid trafficking disorder. In The Metabolic and Molecular Basis of Inherited Disease. 8th edition. Vol. 3. C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editors. McGraw-Hill, New York. 3611-3633.
-
(2001)
The Metabolic and Molecular Basis of Inherited Disease. 8th Edition
, vol.3
, pp. 3611-3633
-
-
Patterson, M.C.1
Vanier, M.T.2
Suzuki, K.3
Morris, J.A.4
Carstea, E.D.5
Neufeld, E.D.6
Blanchette-Mackie, E.J.7
Pentchev, P.G.8
-
2
-
-
0030863352
-
Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
-
Carstea, E. D., J. A. Morris, K. G. Coleman, S. K. Loftus, D. Zhang, C. Cummings, J. Gu, M. A. Rosenfeld, W. J. Pavan, D. B. Krizman, 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
-
3
-
-
0030768084
-
Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeostasis gene
-
Loftus, S. K., J. A. Morris, E. D. Carstea, J. Z. Gu, C. Cummings, A. Brown, J. Ellison, K. Ohno, M. A. Rosenfeld, D. A. Tagle, et al. 1997. Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. Science. 277: 232-235.
-
(1997)
Science
, vol.277
, pp. 232-235
-
-
Loftus, S.K.1
Morris, J.A.2
Carstea, E.D.3
Gu, J.Z.4
Cummings, C.5
Brown, A.6
Ellison, J.7
Ohno, K.8
Rosenfeld, M.A.9
Tagle, D.A.10
-
4
-
-
0033514298
-
Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization
-
Watari, H., E. J. Blanchette-Mackie, N. K. Dwyer, J. M. Glick, S. Patel, E. B. Neufeld, R. O. Brady, P. G. Pentchev, and J. F. Strauss III. 1999. Niemann-Pick C1 protein: obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization. Proc. Natl. Acad. Sci. USA. 96: 805-810.
-
(1999)
Proc. Natl. Acad. Sci. USA
, vol.96
, pp. 805-810
-
-
Watari, H.1
Blanchette-Mackie, E.J.2
Dwyer, N.K.3
Glick, J.M.4
Patel, S.5
Neufeld, E.B.6
Brady, R.O.7
Pentchev, P.G.8
Strauss III, J.F.9
-
5
-
-
0033618338
-
Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein
-
Watari, H., E. J. Blanchette-Mackie, N. K. Dwyer, M. Watari, E. B. Neufeld, S. Patel, P. G. Pentchev, and J. F. Strauss III. 1999. Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein. J. Biol. Chem. 274: 21861-21866.
-
(1999)
J. Biol. Chem.
, vol.274
, pp. 21861-21866
-
-
Watari, H.1
Blanchette-Mackie, E.J.2
Dwyer, N.K.3
Watari, M.4
Neufeld, E.B.5
Patel, S.6
Pentchev, P.G.7
Strauss III, J.F.8
-
6
-
-
0034637440
-
Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating protein
-
Davies, J. P., and Y. A. Ioannou. 2000. Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating protein. J. Biol. Chem. 275: 24367-24374.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 24367-24374
-
-
Davies, J.P.1
Ioannou, Y.A.2
-
7
-
-
0034714439
-
Determinants of NPC1 expression and action: Key promoter regions, posttranscriptional control, and the importance of a "cysteine-rich" loop
-
Watari, H., E. J. Blanchette-Mackie, N. K. Dwyer, M. Watari, C. G. Burd, S. Patel, P. G. Pentchev, and J. F. Strauss III. 2000. Determinants of NPC1 expression and action: key promoter regions, posttranscriptional control, and the importance of a "cysteine-rich" loop. Exp. Cell Res. 259: 247-256.
-
(2000)
Exp. Cell Res.
, vol.259
, pp. 247-256
-
-
Watari, H.1
Blanchette-Mackie, E.J.2
Dwyer, N.K.3
Watari, M.4
Burd, C.G.5
Patel, S.6
Pentchev, P.G.7
Strauss III, J.F.8
-
8
-
-
0034655917
-
Evidence for a Niemann-Pick C (NPC) gene family: Identification and characterization of NPC1L1
-
Davies, J. P., B. Levy, and Y. A. Ioannou. 2000. Evidence for a Niemann-Pick C (NPC) gene family: identification and characterization of NPC1L1. Genomics. 65: 137-145.
-
(2000)
Genomics
, vol.65
, pp. 137-145
-
-
Davies, J.P.1
Levy, B.2
Ioannou, Y.A.3
-
9
-
-
0033358193
-
Mutations in NPC1 highlight a conserved NPC1-specific cysteine-rich domain
-
Greer, W. L., M. J. Dobson, G. S. Girouard, D. M. Byers, D. C. Riddell, and P. E. Neumann. 1999. Mutations in NPC1 highlight a conserved NPC1-specific cysteine-rich domain. Am. J. Hum. Genet. 65: 1252-1260.
-
(1999)
Am. J. Hum. Genet.
, vol.65
, pp. 1252-1260
-
-
Greer, W.L.1
Dobson, M.J.2
Girouard, G.S.3
Byers, D.M.4
Riddell, D.C.5
Neumann, P.E.6
-
10
-
-
0034987798
-
Niemann-Pick C1 disease: Correlations between NPC1 mutations, levels of NPC1 proteins, and phenotypes emphasize the functional significance of the putative sterol-sensing domain and of the cysteine-rich luminal loop
-
Millat, G., C. Marcais, C. Tomasetto, K. Chikh, A. Fensom, K. Harzer, D. A. Wenger, K. Ohno, and M. T. Vanier. 2001. Niemann-Pick C1 disease: correlations between NPC1 mutations, levels of NPC1 proteins, and phenotypes emphasize the functional significance of the putative sterol-sensing domain and of the cysteine-rich luminal loop. Am. J. Hum. Genet. 68: 1373-1385.
-
(2001)
Am. J. Hum. Genet.
, vol.68
, pp. 1373-1385
-
-
Millat, G.1
Marcais, C.2
Tomasetto, C.3
Chikh, K.4
Fensom, A.5
Harzer, K.6
Wenger, D.A.7
Ohno, K.8
Vanier, M.T.9
-
11
-
-
0034987028
-
Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1
-
Sun, X., D. L. Marks, W. D. Park, C. L. Wheatley, V. Puri, J. F. O'Brien, D. L. Kraft, P. A. Lundquist, M. C. Patterson, R. E. Pagano, et al. 2001. Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1. Am. J. Hum. Genet. 68: 1361-1372.
-
(2001)
Am. J. Hum. Genet.
, vol.68
, pp. 1361-1372
-
-
Sun, X.1
Marks, D.L.2
Park, W.D.3
Wheatley, C.L.4
Puri, V.5
O'Brien, J.F.6
Kraft, D.L.7
Lundquist, P.A.8
Patterson, M.C.9
Pagano, R.E.10
-
12
-
-
0141753992
-
Identification of 58 novel mutations in Niemann-Pick disease type C: Correlation with biochemical phenotype and importance of PTC1-like domains in NPC1
-
Park, W. D., J. F. O'Brien, P. A. Lundquist, D. L. Kraft, C. W. Vockley, P. S. Karnes, M. C. Patterson, and K. Snow. 2003. Identification of 58 novel mutations in Niemann-Pick disease type C: correlation with biochemical phenotype and importance of PTC1-like domains in NPC1. Hum. Mutat. 22: 313-325.
-
(2003)
Hum. Mutat.
, vol.22
, pp. 313-325
-
-
Park, W.D.1
O'Brien, J.F.2
Lundquist, P.A.3
Kraft, D.L.4
Vockley, C.W.5
Karnes, P.S.6
Patterson, M.C.7
Snow, K.8
-
13
-
-
0021346802
-
A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol
-
Pentchev, P. G., A. D. Boothe, H. S. Kruth, H. Weintroub, J. Stivers, and R. O. Brady. 1984. A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol. J. Biol. Chem. 259: 5784-5791.
-
(1984)
J. Biol. Chem.
, vol.259
, pp. 5784-5791
-
-
Pentchev, P.G.1
Boothe, A.D.2
Kruth, H.S.3
Weintroub, H.4
Stivers, J.5
Brady, R.O.6
-
14
-
-
0022980841
-
The cholesterol storage disorder of the mutant BALB/c mouse: A primary genetic lesion closely linked to defective esterification of exogenously derived cholesterol and its relationship to human type C Niemann-Pick disease
-
Pentchev, P. G., M. E. Comly, H. S. Kruth, S. Patel, M. Proestel, and H. Weintroub. 1986. The cholesterol storage disorder of the mutant BALB/c mouse: a primary genetic lesion closely linked to defective esterification of exogenously derived cholesterol and its relationship to human type C Niemann-Pick disease. J. Biol. Chem. 261: 2772-2777.
-
(1986)
J. Biol. Chem.
, vol.261
, pp. 2772-2777
-
-
Pentchev, P.G.1
Comly, M.E.2
Kruth, H.S.3
Patel, S.4
Proestel, M.5
Weintroub, H.6
-
15
-
-
0033049909
-
Cholesterol balance and metabolism in mice with loss of function of Niemann-Pick C protein
-
Xie, C., S. D. Turley, P. G. Pentchev, and J. M. Dietschy. 1999. Cholesterol balance and metabolism in mice with loss of function of Niemann-Pick C protein. Am. J. Physiol. 276: E336-E344.
-
(1999)
Am. J. Physiol.
, vol.276
-
-
Xie, C.1
Turley, S.D.2
Pentchev, P.G.3
Dietschy, J.M.4
-
16
-
-
0034534808
-
Cholesterol is sequestered in the brains of mice with Niemann-Pick type C disease but turnover is increased
-
Xie, C., D. K. Burns, S. D. Turley, and J. M. Dietschy. 2000. Cholesterol is sequestered in the brains of mice with Niemann-Pick type C disease but turnover is increased. J. Neuropathol. Exp. Neurol. 59: 1106-1117.
-
(2000)
J. Neuropathol. Exp. Neurol.
, vol.59
, pp. 1106-1117
-
-
Xie, C.1
Burns, D.K.2
Turley, S.D.3
Dietschy, J.M.4
-
17
-
-
0035971218
-
Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice
-
Sawamura, N., J. S. Gong, W. S. Garver, R. A. Heidenreich, H. Ninomiya, K. Ohno, K. Yanagisawa, and M. Michikawa. 2001. Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice. J. Biol. Chem. 276: 10314-10319.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 10314-10319
-
-
Sawamura, N.1
Gong, J.S.2
Garver, W.S.3
Heidenreich, R.A.4
Ninomiya, H.5
Ohno, K.6
Yanagisawa, K.7
Michikawa, M.8
-
18
-
-
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., S. D. Turley, and J. M. Dietschy. 1999. 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. 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
-
19
-
-
0033847304
-
Centripetal cholesterol flow from the extrahepatic organs through the liver is normal in mice with mutated Niemann-Pick type C protein (NPC1)
-
Xie, C., S. D. Turley, and J. M. Dietschy. 2000. Centripetal cholesterol flow from the extrahepatic organs through the liver is normal in mice with mutated Niemann-Pick type C protein (NPC1). J. Lipid Res. 41: 1278-1289.
-
(2000)
J. Lipid Res.
, vol.41
, pp. 1278-1289
-
-
Xie, C.1
Turley, S.D.2
Dietschy, J.M.3
-
20
-
-
0022917296
-
Type C Niemann-Pick disease. A parallel loss of regulatory responses in both the uptake and esterification of low density lipoprotein-derived cholesterol in cultured fibroblasts
-
Pentchev, P. G., H. S. Kruth, M. E. Comly, J. D. Butler, M. T. Vanier, D. A. Wenger, and S. Patel. 1986. Type C Niemann-Pick disease. A parallel loss of regulatory responses in both the uptake and esterification of low density lipoprotein-derived cholesterol in cultured fibroblasts. J. Biol. Chem. 261: 16775-16780.
-
(1986)
J. Biol. Chem.
, vol.261
, pp. 16775-16780
-
-
Pentchev, P.G.1
Kruth, H.S.2
Comly, M.E.3
Butler, J.D.4
Vanier, M.T.5
Wenger, D.A.6
Patel, S.7
-
21
-
-
0023608155
-
Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts
-
Liscum, L., and J. R. Faust. 1987. Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts. J. Biol. Chem. 262: 17002-17008.
-
(1987)
J. Biol. Chem.
, vol.262
, pp. 17002-17008
-
-
Liscum, L.1
Faust, J.R.2
-
22
-
-
0024539791
-
The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts
-
Liscum, L., R. M. Ruggiero, and J. R. Faust. 1989. The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts. J. Cell Biol. 108: 1625-1636.
-
(1989)
J. Cell Biol.
, vol.108
, pp. 1625-1636
-
-
Liscum, L.1
Ruggiero, R.M.2
Faust, J.R.3
-
23
-
-
0027155888
-
Differential accumulation of cholesterol in Golgi compartments of normal and Niemann-Pick type C fibroblasts incubated with LDL: A cytochemical freeze-fracture study
-
Coxey, R. A., P. G. Pentchev, G. Campbell, and E. J. Blanchette-Mackie. 1993. Differential accumulation of cholesterol in Golgi compartments of normal and Niemann-Pick type C fibroblasts incubated with LDL: a cytochemical freeze-fracture study. J. Lipid Res. 34: 1165-1176.
-
(1993)
J. Lipid Res.
, vol.34
, pp. 1165-1176
-
-
Coxey, R.A.1
Pentchev, P.G.2
Campbell, G.3
Blanchette-Mackie, E.J.4
-
24
-
-
0035655647
-
Monoclonal antibody detection of plasma membrane cholesterol microdomains responsive to cholesterol trafficking
-
Kruth, H. S., I. Ifrim, J. Chang, L. Addadi, D. Perl-Treves, and W. Y. Zhang. 2001. Monoclonal antibody detection of plasma membrane cholesterol microdomains responsive to cholesterol trafficking. J. Lipid Res. 42: 1492-1500.
-
(2001)
J. Lipid Res.
, vol.42
, pp. 1492-1500
-
-
Kruth, H.S.1
Ifrim, I.2
Chang, J.3
Addadi, L.4
Perl-Treves, D.5
Zhang, W.Y.6
-
25
-
-
0036193438
-
Niemann-Pick C1 protein regulates cholesterol transport to the trans-Golgi network and plasma membrane caveolae
-
Garver, W. S., K. Krishnan, J. R. Gallagos, M. Michikawa, G. A. Francis, and R. A. Heidenreich. 2002. Niemann-Pick C1 protein regulates cholesterol transport to the trans-Golgi network and plasma membrane caveolae. J. Lipid Res. 43: 579-589.
-
(2002)
J. Lipid Res.
, vol.43
, pp. 579-589
-
-
Garver, W.S.1
Krishnan, K.2
Gallagos, J.R.3
Michikawa, M.4
Francis, G.A.5
Heidenreich, R.A.6
-
26
-
-
0041825673
-
Impaired ABCA1-dependent lipid efflux and hypoalphalipoproteinemia in human Niemann-Pick type C disease
-
Choi, H. Y., B. Karten, T. Chan, J. E. Vance, W. L. Greer, R. A. Heidenreich, W. S. Garver, and G. A. Francis. 2003. Impaired ABCA1-dependent lipid efflux and hypoalphalipoproteinemia in human Niemann-Pick type C disease. J. Biol. Chem. 278: 32569-32577.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 32569-32577
-
-
Choi, H.Y.1
Karten, B.2
Chan, T.3
Vance, J.E.4
Greer, W.L.5
Heidenreich, R.A.6
Garver, W.S.7
Francis, G.A.8
-
27
-
-
0033515560
-
The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo
-
Neufeld, E. B., M. Wastney, S. Patel, S. Suresh, A. M. Conney, N. K. Dwyer, C. F. Roff, K. Ohno, J. A. Morris, E. D. Carstea, et al. 1999. The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo. J. Biol. Chem. 274: 9627-9635.
-
(1999)
J. Biol. Chem.
, vol.274
, pp. 9627-9635
-
-
Neufeld, E.B.1
Wastney, M.2
Patel, S.3
Suresh, S.4
Conney, A.M.5
Dwyer, N.K.6
Roff, C.F.7
Ohno, K.8
Morris, J.A.9
Carstea, E.D.10
-
28
-
-
0034025322
-
Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments
-
Garver, W. S., R. A. Heidenreich, R. P. Erickson, M. A. Thomas, and J. M. Wilson. 2000. Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments. J. Lipid Res. 41: 673-687.
-
(2000)
J. Lipid Res.
, vol.41
, pp. 673-687
-
-
Garver, W.S.1
Heidenreich, R.A.2
Erickson, R.P.3
Thomas, M.A.4
Wilson, J.M.5
-
29
-
-
0033145510
-
Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport
-
Kobayashi, T., M. H. Beuchat, M. Lindsay, S. Frias, R. D. Palmiter, H. Sakuraba, R. G. Parton, and J. Gruenberg. 1999. Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport. Nat. Cell Biol. 1: 113-118.
-
(1999)
Nat. Cell Biol.
, vol.1
, pp. 113-118
-
-
Kobayashi, T.1
Beuchat, M.H.2
Lindsay, M.3
Frias, S.4
Palmiter, R.D.5
Sakuraba, H.6
Parton, R.G.7
Gruenberg, J.8
-
30
-
-
0035830942
-
The steroidogenic acute regulatory protein homolog MLN64, a late endosomal cholesterol-binding protein
-
Alpy, F., M. E. Stoeckel, A. Dierich, J. M. Escola, C. Wendung, M. P. Chenard, M. T. Vanier, J. Gruenberg, C. Tomasetto, and M. C. Rio. 2001. The steroidogenic acute regulatory protein homolog MLN64, a late endosomal cholesterol-binding protein. J. Biol. Chem. 276: 4261-4269.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 4261-4269
-
-
Alpy, F.1
Stoeckel, M.E.2
Dierich, A.3
Escola, J.M.4
Wendung, C.5
Chenard, M.P.6
Vanier, M.T.7
Gruenberg, J.8
Tomasetto, C.9
Rio, M.C.10
-
31
-
-
4344637102
-
Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain
-
Ohgami, N., D. C. Ko, M. Thomas, M. P. Scott, C. C. Y. Chang, and T-Y. Chang. 2004. Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain. Proc. Natl. Acad. Sci. USA. 101: 12473-12478.
-
(2004)
Proc. Natl. Acad. Sci. USA
, vol.101
, pp. 12473-12478
-
-
Ohgami, N.1
Ko, D.C.2
Thomas, M.3
Scott, M.P.4
Chang, C.C.Y.5
Chang, T.-Y.6
-
32
-
-
0035793562
-
Sterol-modulated glycolipid sorting occurs in Niemann-Pick C1 late endosomes
-
Zhang, M., N. K. Dwyer, E. B. Neufeld, D. C. Love, A. Cooney, M. Comly, S. Patel, H. Watari, J. F. Strauss III, P. G. Pentchev, J. A. Hanover, and E. J. Blanchette-Mackie. 2001. Sterol-modulated glycolipid sorting occurs in Niemann-Pick C1 late endosomes. J. Biol. Chem. 276: 3417-3425.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 3417-3425
-
-
Zhang, M.1
Dwyer, N.K.2
Neufeld, E.B.3
Love, D.C.4
Cooney, A.5
Comly, M.6
Patel, S.7
Watari, H.8
Strauss III, J.F.9
Pentchev, P.G.10
Hanover, J.A.11
Blanchette-Mackie, E.J.12
-
33
-
-
0035836635
-
Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease
-
Zhang, M., N. K. Dwyer, D. C. Love, A. Cooney, M. Comly, E. Neufeld, P. G. Pentchev, E. J. Blanchette-Mackie, and J. A. Hanover. 2001. Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease. Proc. Natl. Acad. Sci. USA. 98: 4466-4471.
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 4466-4471
-
-
Zhang, M.1
Dwyer, N.K.2
Love, D.C.3
Cooney, A.4
Comly, M.5
Neufeld, E.6
Pentchev, P.G.7
Blanchette-Mackie, E.J.8
Hanover, J.A.9
-
34
-
-
0035163949
-
Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events
-
Ko, D. C., M. D. Gordon, J. Y. Jin, and M. P. Scott. 2001. Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events. Mol. Biol. Cell. 12: 601-614.
-
(2001)
Mol. Biol. Cell
, vol.12
, pp. 601-614
-
-
Ko, D.C.1
Gordon, M.D.2
Jin, J.Y.3
Scott, M.P.4
-
35
-
-
0028929262
-
Role of the low density lipoprotein receptor in the flux of cholesterol through the plasma and across the tissues of the mouse
-
Osono, Y., L. A. Woollett, J. Herz, and J. M. Dietschy. 1995. Role of the low density lipoprotein receptor in the flux of cholesterol through the plasma and across the tissues of the mouse. J. Clin. Invest. 95: 1124-1132.
-
(1995)
J. Clin. Invest.
, vol.95
, pp. 1124-1132
-
-
Osono, Y.1
Woollett, L.A.2
Herz, J.3
Dietschy, J.M.4
-
36
-
-
0028833385
-
Role of liver in the synthesis of cholesterol and the clearance of low density lipoproteins in the cynomolgus monkey
-
Turley, S. D., D. K. Spady, and J. M. Dietschy. 1995. Role of liver in the synthesis of cholesterol and the clearance of low density lipoproteins in the cynomolgus monkey. J. Lipid Res. 36: 67-79.
-
(1995)
J. Lipid Res.
, vol.36
, pp. 67-79
-
-
Turley, S.D.1
Spady, D.K.2
Dietschy, J.M.3
-
37
-
-
0020526063
-
A radioiodinated, intracellularly trapped ligand for determining the sites of plasma protein degradation in vivo
-
Pittman, R. C., T. E. Carew, C. K. Glass, S. R. Green, C. A. Taylor, and A. D. Attie. 1983. A radioiodinated, intracellularly trapped ligand for determining the sites of plasma protein degradation in vivo. Biochem J. 212: 791-800.
-
(1983)
Biochem. J.
, vol.212
, pp. 791-800
-
-
Pittman, R.C.1
Carew, T.E.2
Glass, C.K.3
Green, S.R.4
Taylor, C.A.5
Attie, A.D.6
-
38
-
-
0021364271
-
Measurement in vivo of irreversible degradation of low density lipoprotein in the rabbit aorta: Predominance of intimal degradation
-
Carew, T. E., R. C. Pittman, E. R. Marchand, and D. Steinberg. 1984. Measurement in vivo of irreversible degradation of low density lipoprotein in the rabbit aorta: predominance of intimal degradation. Arteriosclerosis. 4: 214-224.
-
(1984)
Arteriosclerosis
, vol.4
, pp. 214-224
-
-
Carew, T.E.1
Pittman, R.C.2
Marchand, E.R.3
Steinberg, D.4
-
39
-
-
0021341040
-
Comparison of various methods for in vitro cholesteryl ester labeling of lipoproteins from hypercholesterolemic rabbits
-
Hough, J. L., and D. B. Zilversmit. 1984. Comparison of various methods for in vitro cholesteryl ester labeling of lipoproteins from hypercholesterolemic rabbits. Biochim. Biophys. Acta. 792: 338-347.
-
(1984)
Biochim. Biophys. Acta
, vol.792
, pp. 338-347
-
-
Hough, J.L.1
Zilversmit, D.B.2
-
40
-
-
0023664054
-
A nonendocytotic mechanism for the selective uptake of high density lipoprotein-associated cholesteryl esters
-
Pittman, R. C., T. P. Knecht, M. S. Rosenbaum, and C. A. Taylor. 1987. A nonendocytotic mechanism for the selective uptake of high density lipoprotein-associated cholesteryl esters. J. Biol. Chem. 1987: 2443-2450.
-
(1987)
J. Biol. Chem.
, vol.1987
, pp. 2443-2450
-
-
Pittman, R.C.1
Knecht, T.P.2
Rosenbaum, M.S.3
Taylor, C.A.4
-
41
-
-
0030896984
-
Kinetic parameters for high density lipoprotein apoprotein AI and cholesteryl ester transport in the hamster
-
Woollett, L. A., and D. K. Spady. 1997. Kinetic parameters for high density lipoprotein apoprotein AI and cholesteryl ester transport in the hamster. J. Clin. Invest. 99: 1704-1713.
-
(1997)
J. Clin. Invest.
, vol.99
, pp. 1704-1713
-
-
Woollett, L.A.1
Spady, D.K.2
-
42
-
-
0000940687
-
Dissociation of tissue uptake of cholesterol ester from that of apoprotein A-I of rat plasma high density lipoprotein: Selective delivery of cholesterol ester to liver, adrenal and gonad
-
Glass, C. K., R. C. Pittman, D. B. Weinstein, and D. Steinberg. 1983. Dissociation of tissue uptake of cholesterol ester from that of apoprotein A-I of rat plasma high density lipoprotein: selective delivery of cholesterol ester to liver, adrenal and gonad. Proc. Natl. Acad. Sci. USA. 80: 5435-5439.
-
(1983)
Proc. Natl. Acad. Sci. USA
, vol.80
, pp. 5435-5439
-
-
Glass, C.K.1
Pittman, R.C.2
Weinstein, D.B.3
Steinberg, D.4
-
43
-
-
0020378382
-
Correlation of low and high density lipoprotein binding in vivo with rates of lipoprotein degradation in the rat: A comparison of lipoproteins of rat and human origin
-
Koelz, H. R., B. C. Sherrill, S. D. Turley, and J. M. Dietschy. 1982. Correlation of low and high density lipoprotein binding in vivo with rates of lipoprotein degradation in the rat: a comparison of lipoproteins of rat and human origin. J. Biol. Chem. 257: 8061-8072.
-
(1982)
J. Biol. Chem.
, vol.257
, pp. 8061-8072
-
-
Koelz, H.R.1
Sherrill, B.C.2
Turley, S.D.3
Dietschy, J.M.4
-
44
-
-
3142662216
-
Rates of receptor-dependent and -independent low density lipoprotein uptake in the hamster
-
Spady, D. K., D. W. Bilheimer, and J. M. Dietschy. 1983. Rates of receptor-dependent and -independent low density lipoprotein uptake in the hamster. Proc. Natl. Acad. Sci. USA. 80: 3499-3503.
-
(1983)
Proc. Natl. Acad. Sci. USA
, vol.80
, pp. 3499-3503
-
-
Spady, D.K.1
Bilheimer, D.W.2
Dietschy, J.M.3
-
45
-
-
0019481094
-
Rates of sterol synthesis and uptake in the major organs of the rat in vivo
-
Turley, S. D., J. M. Andersen, and J. M. Dietschy. 1981. Rates of sterol synthesis and uptake in the major organs of the rat in vivo. J. Lipid Res. 22: 551-569.
-
(1981)
J. Lipid Res.
, vol.22
, pp. 551-569
-
-
Turley, S.D.1
Andersen, J.M.2
Dietschy, J.M.3
-
46
-
-
0021676528
-
Measurement of rates of cholesterol synthesis using tritiated water
-
Dietschy, J. M., and D. K. Spady. 1984. Measurement of rates of cholesterol synthesis using tritiated water. J. Lipid Res. 25: 1469-1476.
-
(1984)
J. Lipid Res.
, vol.25
, pp. 1469-1476
-
-
Dietschy, J.M.1
Spady, D.K.2
-
47
-
-
0031755081
-
Centripetal cholesterol flux to the liver is dictated by events in the peripheral organs and not by the plasma high density lipoprotein or apolipoprotein A-I concentration
-
Jolley, C. D., L. A. Woollett, S. D. Turley, and J. M. Dietschy. 1998. Centripetal cholesterol flux to the liver is dictated by events in the peripheral organs and not by the plasma high density lipoprotein or apolipoprotein A-I concentration. J. Lipid Res. 39: 2143-2149.
-
(1998)
J. Lipid Res.
, vol.39
, pp. 2143-2149
-
-
Jolley, C.D.1
Woollett, L.A.2
Turley, S.D.3
Dietschy, J.M.4
-
48
-
-
0028085843
-
Psyllium augments the cholesterol-lowering action of cholestyramine in hamsters by enhancing sterol loss from the liver
-
Turley, S. D., B. P. Daggy, and J. M. Dietschy. 1994. Psyllium augments the cholesterol-lowering action of cholestyramine in hamsters by enhancing sterol loss from the liver. Gastroenterology. 107: 444-452.
-
(1994)
Gastroenterology
, vol.107
, pp. 444-452
-
-
Turley, S.D.1
Daggy, B.P.2
Dietschy, J.M.3
-
49
-
-
0031705365
-
Gender-related differences in bile acid and sterol metabolism in outbred CD-1 mice fed low- and high-cholesterol diets
-
Turley, S. D., M. Schwarz, D. K. Spady, and J. M. Dietschy. 1998. Gender-related differences in bile acid and sterol metabolism in outbred CD-1 mice fed low- and high-cholesterol diets. Hepatology. 28: 1088-1094.
-
(1998)
Hepatology
, vol.28
, pp. 1088-1094
-
-
Turley, S.D.1
Schwarz, M.2
Spady, D.K.3
Dietschy, J.M.4
-
50
-
-
0028157731
-
Reevaluation and application of the dual-isotope plasma ratio method for the measurement of intestinal cholesterol absorption in the hamster
-
Turley, S. D., M. W. Herndon, and J. M. Dietschy. 1994. Reevaluation and application of the dual-isotope plasma ratio method for the measurement of intestinal cholesterol absorption in the hamster. J. Lipid Res. 35: 328-339.
-
(1994)
J. Lipid Res.
, vol.35
, pp. 328-339
-
-
Turley, S.D.1
Herndon, M.W.2
Dietschy, J.M.3
-
51
-
-
0014949207
-
Cleavage of structural proteins during the assembly of the head of bacteriophage T4
-
Laemmli, U. K. 1970. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature. 227: 680-685.
-
(1970)
Nature
, vol.227
, pp. 680-685
-
-
Laemmli, U.K.1
-
52
-
-
0029134079
-
Hyper- and hypo-responsiveness to dietary fat and cholesterol among inbred mice: Searching for level and variability genes
-
Kirk, E. A., G. L. Moe, M. T. Caldwell, J. A. Lernmark, D. L. Wilson, and R. C. LeBoeuf. 1995. Hyper- and hypo-responsiveness to dietary fat and cholesterol among inbred mice: searching for level and variability genes. J. Lipid Res. 36: 1522-1532.
-
(1995)
J. Lipid Res.
, vol.36
, pp. 1522-1532
-
-
Kirk, E.A.1
Moe, G.L.2
Caldwell, M.T.3
Lernmark, J.A.4
Wilson, D.L.5
Leboeuf, R.C.6
-
53
-
-
0037040184
-
Control of cholesterol turnover in the mouse
-
Dietschy, J. M., and S. D. Turley. 2002. Control of cholesterol turnover in the mouse. J. Biol. Chem. 277: 3801-3804.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 3801-3804
-
-
Dietschy, J.M.1
Turley, S.D.2
-
54
-
-
0023644898
-
Genetic factors controlling structure and expression of apolipoproteins B and E in mice
-
Lusis, A. J., B. A. Taylor, D. Quon, S. Zollman, and R. C. LeBoeuf. 1987. Genetic factors controlling structure and expression of apolipoproteins B and E in mice. J. Biol. Chem. 262: 7594-7604.
-
(1987)
J. Biol. Chem.
, vol.262
, pp. 7594-7604
-
-
Lusis, A.J.1
Taylor, B.A.2
Quon, D.3
Zollman, S.4
Leboeuf, R.C.5
-
55
-
-
0026056014
-
In vivo regulation of low-density lipoprotein receptor and apolipoprotein B gene expression by dietary fat and cholesterol in inbred strains of mice
-
Srivastava, R. A. K., S. Jiao, J. Tang, B. A. Pfleger, R. T. Kitchens, and G. Schonfeld. 1991. In vivo regulation of low-density lipoprotein receptor and apolipoprotein B gene expression by dietary fat and cholesterol in inbred strains of mice. Biochim. Biophys. Acta. 1086: 29-43.
-
(1991)
Biochim. Biophys. Acta
, vol.1086
, pp. 29-43
-
-
Srivastava, R.A.K.1
Jiao, S.2
Tang, J.3
Pfleger, B.A.4
Kitchens, R.T.5
Schonfeld, G.6
-
56
-
-
0030024169
-
Mouse models of atherosclerosis
-
Breslow, J. L. 1996. Mouse models of atherosclerosis. Science. 272: 685-688.
-
(1996)
Science
, vol.272
, pp. 685-688
-
-
Breslow, J.L.1
-
57
-
-
0036933005
-
The role and regulation of the Niemann-Pick C1 gene in adrenal steroidogenesis
-
Gevry, N. Y., and B. D. Murphy. 2002. The role and regulation of the Niemann-Pick C1 gene in adrenal steroidogenesis. Endocr. Res. 28: 403-412.
-
(2002)
Endocr. Res.
, vol.28
, pp. 403-412
-
-
Gevry, N.Y.1
Murphy, B.D.2
-
58
-
-
12444315104
-
Regulation of Niemann-Pick C1 gene expression by the 3′5′- cyclic adenosine monophosphate pathway in steroidogenic cells
-
Gevry, N. Y., E. Lalli, P. Sassone-Corsi, and B. D. Murphy. 2003. Regulation of Niemann-Pick C1 gene expression by the 3′5′-cyclic adenosine monophosphate pathway in steroidogenic cells. Mol. Endocrinol. 17: 704-715.
-
(2003)
Mol. Endocrinol.
, vol.17
, pp. 704-715
-
-
Gevry, N.Y.1
Lalli, E.2
Sassone-Corsi, P.3
Murphy, B.D.4
-
59
-
-
0022168951
-
Receptor-mediated endocytosis
-
Goldstein, J. L., M. S. Brown, R. G. W. Anderson, D. W. Russell, and W. J. Schneider. 1985. Receptor-mediated endocytosis. Annu. Rev. Cell Biol. 1: 1-39.
-
(1985)
Annu. Rev. Cell Biol.
, vol.1
, pp. 1-39
-
-
Goldstein, J.L.1
Brown, M.S.2
Anderson, R.G.W.3
Russell, D.W.4
Schneider, W.J.5
-
60
-
-
0030046797
-
Identification of scavenger receptor SR-B1 as a high density lipoprotein receptor
-
Acton, S., A. Rigotti, K. T. Landschulz, S. Xu, H. H. Hobbs, and M. Krieger. 1996. Identification of scavenger receptor SR-B1 as a high density lipoprotein receptor. Science. 271: 518-520.
-
(1996)
Science
, vol.271
, pp. 518-520
-
-
Acton, S.1
Rigotti, A.2
Landschulz, K.T.3
Xu, S.4
Hobbs, H.H.5
Krieger, M.6
-
61
-
-
0242613116
-
Regulation of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in human fibroblasts by lipoproteins
-
Brown, M. S., S. E. Dana, and J. L. Goldstein. 1973. Regulation of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in human fibroblasts by lipoproteins. Proc. Natl. Acad. Sci. USA. 70: 2162-2166.
-
(1973)
Proc. Natl. Acad. Sci. USA
, vol.70
, pp. 2162-2166
-
-
Brown, M.S.1
Dana, S.E.2
Goldstein, J.L.3
-
62
-
-
0016679756
-
Regulation of the activity of the low density lipoprotein receptor in human fibroblasts
-
Brown, M. S., and J. L. Goldstein. 1975. Regulation of the activity of the low density lipoprotein receptor in human fibroblasts. Cell. 6: 307-316.
-
(1975)
Cell
, vol.6
, pp. 307-316
-
-
Brown, M.S.1
Goldstein, J.L.2
-
63
-
-
0030970279
-
Caveolin mRNA levels are up-regulated by free cholesterol and down-regulated by oxysterols in fibroblast monolayers
-
Fielding, C. J., A. Bist, and P. E. Fielding. 1997. Caveolin mRNA levels are up-regulated by free cholesterol and down-regulated by oxysterols in fibroblast monolayers. Proc. Natl. Acad. Sci. USA. 94: 3753-3758.
-
(1997)
Proc. Natl. Acad. Sci. USA
, vol.94
, pp. 3753-3758
-
-
Fielding, C.J.1
Bist, A.2
Fielding, P.E.3
-
64
-
-
0030941803
-
The SREBP pathway: Regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor
-
Brown, M. S., and J. L. Goldstein. 1997. The SREBP pathway: regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor. Cell. 89: 331-340.
-
(1997)
Cell
, vol.89
, pp. 331-340
-
-
Brown, M.S.1
Goldstein, J.L.2
-
65
-
-
4043124013
-
Niemann-Pick C1 Like 1 (NPC1L1) is the intestinal phytosterol and cholesterol transporter and a key modulator of whole-body cholesterol homeostasis
-
Davis, H. R., L. J. Zhu, L. M. Hoos, G. Tetzloff, M. Maguire, J. Liu, X. Yao, S. P. Iyer, M. H. Lam, E. G. Lund, et al. 2004. Niemann-Pick C1 Like 1 (NPC1L1) is the intestinal phytosterol and cholesterol transporter and a key modulator of whole-body cholesterol homeostasis. J. Biol. Chem. 279: 33586-33592.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 33586-33592
-
-
Davis, H.R.1
Zhu, L.J.2
Hoos, L.M.3
Tetzloff, G.4
Maguire, M.5
Liu, J.6
Yao, X.7
Iyer, S.P.8
Lam, M.H.9
Lund, E.G.10
-
66
-
-
17744376173
-
A PPAR-LXR-ABCA1 pathway in macrophages is involved in cholesterol efflux and atherogenesis
-
Chawla, A., W. A. Boisvert, C. H. Lee, B. A. Laffitte, Y. Barak, S. B. Joseph, D. Liao, L. Nagy, P. A. Edwards, L. K. Curtiss, et al. 2001. A PPAR-LXR-ABCA1 pathway in macrophages is involved in cholesterol efflux and atherogenesis. Mol. Cell. 7: 161-171.
-
(2001)
Mol. Cell
, vol.7
, pp. 161-171
-
-
Chawla, A.1
Boisvert, W.A.2
Lee, C.H.3
Laffitte, B.A.4
Barak, Y.5
Joseph, S.B.6
Liao, D.7
Nagy, L.8
Edwards, P.A.9
Curtiss, L.K.10
-
67
-
-
0032570715
-
Mouse lysosomal acid lipase: Characterization of the gene and analysis of promoter activity
-
Du, H., M. Duanmu, and L. R. Rosa. 1998. Mouse lysosomal acid lipase: characterization of the gene and analysis of promoter activity. Gene. 208: 285-295.
-
(1998)
Gene
, vol.208
, pp. 285-295
-
-
Du, H.1
Duanmu, M.2
Rosa, L.R.3
-
68
-
-
0028800470
-
Regulation and immunolocalization of acyl-coenzyme A:cholesterol acyltransferase in mammalian cells as studied with specific antibodies
-
Chang, C. C. Y., J. Chen, M. A. Thomas, D. Cheng, V. A. Del Priore, R. S. Newton, M. E. Pape, and T. Y. Chang. 1995. Regulation and immunolocalization of acyl-coenzyme A:cholesterol acyltransferase in mammalian cells as studied with specific antibodies. J. Biol. Chem. 270: 29532-29540.
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 29532-29540
-
-
Chang, C.C.Y.1
Chen, J.2
Thomas, M.A.3
Cheng, D.4
Del Priore, V.A.5
Newton, R.S.6
Pape, M.E.7
Chang, T.Y.8
-
69
-
-
0028865098
-
Tissue-specific expression and cholesterol regulation of acyl-coenzyme A:cholesterol acyltransferase (ACAT) in mice
-
Uelmen, P. J., K. Oka, M. Sullivan, C. C. Y. Chang, T. Y. Chang, and L. Chan. 1995. Tissue-specific expression and cholesterol regulation of acyl-coenzyme A:cholesterol acyltransferase (ACAT) in mice. J. Biol. Chem. 1995: 26192-26201.
-
(1995)
J. Biol. Chem.
, vol.1995
, pp. 26192-26201
-
-
Uelmen, P.J.1
Oka, K.2
Sullivan, M.3
Chang, C.C.Y.4
Chang, T.Y.5
Chan, L.6
-
70
-
-
0032479439
-
Topology of SREBP cleavage-activating protein, a polytopic membrane protein with a sterol-sensing domain
-
Nohturfft, A., M. S. Brown, and J. L. Goldstein. 1998. Topology of SREBP cleavage-activating protein, a polytopic membrane protein with a sterol-sensing domain. J. Biol. Chem. 273: 17243-17250.
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 17243-17250
-
-
Nohturfft, A.1
Brown, M.S.2
Goldstein, J.L.3
-
71
-
-
0037245750
-
Accelerated degradation of HMG-CoA reductase mediated by binding of 1nsig-1 to its sterol-sensing domain
-
Sever, N., T. Yang, M. S. Brown, J. L. Goldstein, and R. A. DeBose-Boyd. 2003. Accelerated degradation of HMG-CoA reductase mediated by binding of 1nsig-1 to its sterol-sensing domain. Mol. Cell. 11: 25-33.
-
(2003)
Mol. Cell
, vol.11
, pp. 25-33
-
-
Sever, N.1
Yang, T.2
Brown, M.S.3
Goldstein, J.L.4
Debose-Boyd, R.A.5
-
72
-
-
3242668095
-
Direct binding of cholesterol to the purified membrane region of SCAP: Mechanism for a sterol-sensing domain
-
Radhakrishnan, A., L. P. Sun, H. J. Kwon, M. S. Brown, and J. L. Goldstein. 2004. Direct binding of cholesterol to the purified membrane region of SCAP: mechanism for a sterol-sensing domain. Mol. Cell. 15: 259-268.
-
(2004)
Mol. Cell
, vol.15
, pp. 259-268
-
-
Radhakrishnan, A.1
Sun, L.P.2
Kwon, H.J.3
Brown, M.S.4
Goldstein, J.L.5
|