-
1
-
-
0037422574
-
Crystal structure of saposin B reveals a dimeric shell for lipid binding
-
Ahn VE, Faull KF, Whitelegge JP, Fluharty AL, Prive GG. 2003. Crystal structure of saposin B reveals a dimeric shell for lipid binding. Proc. Natl. Acad. Sci. USA 100:38-43
-
(2003)
Proc. Natl. Acad. Sci. USA
, vol.100
, pp. 38-43
-
-
Ahn, V.E.1
Faull, K.F.2
Whitelegge, J.P.3
Fluharty, A.L.4
Prive, G.G.5
-
2
-
-
0029953488
-
Transacylase and phospholipases in the synthesis of bis(monoacylglycero) phosphate
-
Amidon B, Brown A, Waite M. 1996. Transacylase and phospholipases in the synthesis of bis(monoacylglycero)phosphate. Biochemistry 35:13995-4002
-
(1996)
Biochemistry
, vol.35
, pp. 13995-14002
-
-
Amidon, B.1
Brown, A.2
Waite, M.3
-
3
-
-
20144372085
-
The cellular pathway of CD1c in immature and maturing dendritic cells
-
Anǵnieux C, Fraisier V, Maître B, Racine V, van der Wel N, et al. 2005. The cellular pathway of CD1c in immature and maturing dendritic cells. Traffic 6:286-302
-
(2005)
Traffic
, vol.6
, pp. 286-302
-
-
Anǵnieux, C.1
Fraisier, V.2
Maître, B.3
Racine, V.4
Van Der Wel, N.5
-
4
-
-
0019513677
-
Mechanism of activation of glucocerebrosidase by co-β-glucosidase (glucosidase activator protein)
-
Berent SL, Radin NS. 1981. Mechanism of activation of glucocerebrosidase by co-β-glucosidase (glucosidase activator protein). Biochim. Biophys. Acta 664:572-82
-
(1981)
Biochim. Biophys. Acta
, vol.664
, pp. 572-582
-
-
Berent, S.L.1
Radin, N.S.2
-
5
-
-
0029070822
-
Purification, characterization, and biosynthesis of human acid ceramidase
-
Bernardo K, Hurwitz R, Zenk T, Desnick RJ, Ferlinz K, et al. 1995. Purification, characterization, and biosynthesis of human acid ceramidase. J. Biol. Chem. 70:11098-102
-
(1995)
J. Biol. Chem.
, vol.70
, pp. 11098-11102
-
-
Bernardo, K.1
Hurwitz, R.2
Zenk, T.3
Desnick, R.J.4
Ferlinz, K.5
-
6
-
-
0027186175
-
Prosaposin deficiency: Further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphingolipid storage disease
-
Bradova V, Smid F, Ulrich-Bott B, Roggendorf W, Paton BC, Harzer K. 1993. Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphingolipid storage disease. Hum. Genet. 92:143-52
-
(1993)
Hum. Genet.
, vol.92
, pp. 143-152
-
-
Bradova, V.1
Smid, F.2
Ulrich-Bott, B.3
Roggendorf, W.4
Paton, B.C.5
Harzer, K.6
-
7
-
-
0016121227
-
Novel stereoconfiguration in lyso-bis-phosphatidic acid of cultured BHK-cells
-
Brotherus J, Renkonen O, Herrmann J, Fischer W, Herrmann, J. 1974. Novel stereoconfiguration in lyso-bis-phosphatidic acid of cultured BHK-cells. Chem. Phys. Lipids 13:178-82
-
(1974)
Chem. Phys. Lipids
, vol.13
, pp. 178-182
-
-
Brotherus, J.1
Renkonen, O.2
Herrmann, J.3
Fischer, W.4
Herrmann, J.5
-
8
-
-
0034625373
-
Structure and function of sphingolipid- And cholesterol-rich membrane rafts
-
Brown DA, London E. 2000. Structure and function of sphingolipid- and cholesterol-rich membrane rafts. J. Biol. Chem. 275:17221-24
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 17221-17224
-
-
Brown, D.A.1
London, E.2
-
9
-
-
0343052042
-
Accumulation of sphingolipids in SAP-precursor (prosaposin)-deficient fibroblasts occurs as intralysosomal membrane structures and can be completely reversed by treatment with human SAP-precursor
-
Burkhardt JK, Hüttler S, Klein A, Möbius W, Habermann A, et al. 1997. Accumulation of sphingolipids in SAP-precursor (prosaposin)-deficient fibroblasts occurs as intralysosomal membrane structures and can be completely reversed by treatment with human SAP-precursor. Eur. J. Cell Biol. 73:10-18
-
(1997)
Eur. J. Cell Biol.
, vol.73
, pp. 10-18
-
-
Burkhardt, J.K.1
Hüttler, S.2
Klein, A.3
Möbius, W.4
Habermann, A.5
-
10
-
-
0030863352
-
Niemann-Pickl disease gene: Homology to mediators of cholesterol homeostasis
-
Carstea ED, Morris JA, Coleman KG, Loftus SK, Zhang D, et al. 1997. Niemann-Pickl disease gene: homology to mediators of cholesterol homeostasis. Science 277:228-31
-
(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
-
11
-
-
0023886858
-
The biological role of dolichol
-
Chojnacki T, Dallner, G. 1988. The biological role of dolichol. Biochem. J. 251:1-9
-
(1988)
Biochem. J.
, vol.251
, pp. 1-9
-
-
Chojnacki, T.1
Dallner, G.2
-
12
-
-
0022782844
-
Immunochemical characterization of two activator proteins stimulating enzymic sphingomyelin degradation in vitro. Absence of one of them in a human Gaucher disease variant
-
Christomanou H, Aignesberger A, Linke RP. 1986. Immunochemical characterization of two activator proteins stimulating enzymic sphingomyelin degradation in vitro. Absence of one of them in a human Gaucher disease variant. Biol. Chem. Hoppe-Seyler 367:879-90
-
(1986)
Biol. Chem. Hoppe-Seyler
, vol.367
, pp. 879-890
-
-
Christomanou, H.1
Aignesberger, A.2
Linke, R.P.3
-
13
-
-
0035943633
-
Saposin D solubilizes anionic phospholipid-containing membranes
-
Ciaffoni F, Salvioli R, Tatti M, Arancia G, Crateri P, Vaccaro AM. 2001. Saposin D solubilizes anionic phospholipid-containing membranes. J. Biol. Chem. 276:31583-89
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 31583-31589
-
-
Ciaffoni, F.1
Salvioli, R.2
Tatti, M.3
Arancia, G.4
Crateri, P.5
Vaccaro, A.M.6
-
14
-
-
0032557156
-
Physiological properties and functions of intracellular fatty acid-binding proteins
-
Coe NR, Bernlohr DA. 1998. Physiological properties and functions of intracellular fatty acid-binding proteins. Biochim. Biophys. Acta 1391:287-306
-
(1998)
Biochim. Biophys. Acta
, vol.1391
, pp. 287-306
-
-
Coe, N.R.1
Bernlohr, D.A.2
-
15
-
-
0020321725
-
Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2
-
Conzelmann E, Burg J, Stephan G, Sandhoff K. 1982. Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2. Eur. J. Biochem. 123:455-64
-
(1982)
Eur. J. Biochem.
, vol.123
, pp. 455-464
-
-
Conzelmann, E.1
Burg, J.2
Stephan, G.3
Sandhoff, K.4
-
16
-
-
2642688117
-
AB variant of infantile GM2 gangliosidosis: Deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2
-
Conzelmann E, Sandhoff K. 1978. AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2. Proc. Natl. Acad. Sci. USA 75:3979-83
-
(1978)
Proc. Natl. Acad. Sci. USA
, vol.75
, pp. 3979-3983
-
-
Conzelmann, E.1
Sandhoff, K.2
-
17
-
-
0018567163
-
Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A
-
Conzelmann E, Sandhoff K. 1979. Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A. Hoppe Seylers Z. Physiol. Chem. 360:1837-49
-
(1979)
Hoppe Seylers Z. Physiol. Chem.
, vol.360
, pp. 1837-1849
-
-
Conzelmann, E.1
Sandhoff, K.2
-
18
-
-
0034704244
-
Transmembrane molecular pump activity of Niemann-Pick C1 protein
-
Davies JP, Chen FW, Ioannou YA. 2000. Transmembrane molecular pump activity of Niemann-Pick C1 protein. Science 290:2295-98
-
(2000)
Science
, vol.290
, pp. 2295-2298
-
-
Davies, J.P.1
Chen, F.W.2
Ioannou, Y.A.3
-
19
-
-
0347625853
-
Solution structure of human saposin C: PH-dependent interaction with phospholipid vesicles
-
de Alba E, Weiler S, Tjandra, N. 2003. Solution structure of human saposin C: pH-dependent interaction with phospholipid vesicles. Biochemistry 42:14729-40
-
(2003)
Biochemistry
, vol.42
, pp. 14729-14740
-
-
Alba, E.1
Weiler, S.2
Tjandra, N.3
-
20
-
-
0037334339
-
At the acidic edge: Emerging functions for lysosomal membrane proteins
-
Eskelinen E-L, Tanaka Y, Saftig P. 2003. At the acidic edge: emerging functions for lysosomal membrane proteins. Trends Cell Biol. 13:137-45
-
(2003)
Trends Cell Biol
, vol.13
, pp. 137-145
-
-
Eskelinen, E.-L.1
Tanaka, Y.2
Saftig, P.3
-
21
-
-
0025824346
-
Human acid β-glucosidase. Use of inhibitory and activating monoclonal antibodies to investigate the enzyme's catalytic mechanism and saposin A and C binding sites
-
Fabbro D, Grabowski GA. 1991. Human acid β-glucosidase. Use of inhibitory and activating monoclonal antibodies to investigate the enzyme's catalytic mechanism and saposin A and C binding sites. J. Biol. Chem. 266:15021-27
-
(1991)
J. Biol. Chem.
, vol.266
, pp. 15021-15027
-
-
Fabbro, D.1
Grabowski, G.A.2
-
22
-
-
0017599450
-
The activator of cerebroside sulphatase. Binding studies with enzyme and substrate demonstrating the detergent function of the activator protein
-
Fischer G, Jatzkewitz H. 1977. The activator of cerebroside sulphatase. Binding studies with enzyme and substrate demonstrating the detergent function of the activator protein. Biochim. Biophys. Acta 481:561-72
-
(1977)
Biochim. Biophys. Acta
, vol.481
, pp. 561-572
-
-
Fischer, G.1
Jatzkewitz, H.2
-
23
-
-
0037418188
-
Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease
-
Friedland N, Liou HL, Lobel P, Stock AM. 2003. Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease. Proc. Natl. Acad. Sci. USA 100:2512-17
-
(2003)
Proc. Natl. Acad. Sci. USA
, vol.100
, pp. 2512-2517
-
-
Friedland, N.1
Liou, H.L.2
Lobel, P.3
Stock, A.M.4
-
24
-
-
0023947493
-
The precursor of sulfatide activator protein is processed to three different proteins
-
Fürst W, Machleidt W, Sandhoff K. 1988. The precursor of sulfatide activator protein is processed to three different proteins. Biol. Chem. Hoppe Seyler 369:317-28
-
(1988)
Biol. Chem. Hoppe Seyler
, vol.369
, pp. 317-328
-
-
Fürst, W.1
Machleidt, W.2
Sandhoff, K.3
-
25
-
-
0026747197
-
Activator proteins and topology of lysosomal sphingolipid catabolism
-
Fürst W, Sandhoff K. 1992. Activator proteins and topology of lysosomal sphingolipid catabolism. Biochim. Biophys. Acta 1126:1-16
-
(1992)
Biochim. Biophys. Acta
, vol.1126
, pp. 1-16
-
-
Fürst, W.1
Sandhoff, K.2
-
26
-
-
0029982572
-
Targeted disruption of the mouse sphingolipid activator protein gene: A complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids
-
Fujita N, Suzuki K, Vanier MT, Popko B, Maeda N, et al. 1996. Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids. Hum. Mol. Genet. 5: 711-25
-
(1996)
Hum. Mol. Genet.
, vol.5
, pp. 711-725
-
-
Fujita, N.1
Suzuki, K.2
Vanier, M.T.3
Popko, B.4
Maeda, N.5
-
27
-
-
0035984523
-
Structure of human CD1b with bound ligands at 2.3 Å, a maze for alkyl chains
-
Gadola SD, Zaccai NR, Harlos K, Shepherd D, Castro-Palomino JC, et al. 2002. Structure of human CD1b with bound ligands at 2.3 Å, a maze for alkyl chains. Nat. Immunol. 3:721-26
-
(2002)
Nat. Immunol.
, vol.3
, pp. 721-726
-
-
Gadola, S.D.1
Zaccai, N.R.2
Harlos, K.3
Shepherd, D.4
Castro-Palomino, J.C.5
-
28
-
-
0000223691
-
Interaction of the GM2-activator protein with phospholipid-ganglioside bilayer membranes and with monolayers at the air-water interface
-
Giehl A, Lemm T, Bartelsen O, Sandhoff K, Blume A. 1999. Interaction of the GM2-activator protein with phospholipid-ganglioside bilayer membranes and with monolayers at the air-water interface. Eur. J. Biochem. 261:650-58
-
(1999)
Eur. J. Biochem.
, vol.261
, pp. 650-658
-
-
Giehl, A.1
Lemm, T.2
Bartelsen, O.3
Sandhoff, K.4
Blume, A.5
-
29
-
-
0037201939
-
Sphingomyelinases: Enzymology and membrane activity
-
Goni FM, Alonso A. 2002. Sphingomyelinases: enzymology and membrane activity. FEBS Lett. 531:38-46
-
(2002)
FEBS Lett
, vol.531
, pp. 38-46
-
-
Goni, F.M.1
Alonso, A.2
-
30
-
-
0042026495
-
Interaction of amoebapores and NK-lysin with symmetric phospholipid and asymmetric lipopolysaccharide/phospholipid bilayers
-
Gutsmann T, Riekens B, Bruhn H, Wiese A, Seydel U, Leippe M. 2003. Interaction of amoebapores and NK-lysin with symmetric phospholipid and asymmetric lipopolysaccharide/phospholipid bilayers. Biochemistry 42:9804-12
-
(2003)
Biochemistry
, vol.42
, pp. 9804-9812
-
-
Gutsmann, T.1
Riekens, B.2
Bruhn, H.3
Wiese, A.4
Seydel, U.5
Leippe, M.6
-
31
-
-
0347611095
-
Molecular machinery for non-vesicular trafficking of ceramide
-
Hanada K, Kumagai K, Yasuda S, Miura Y, Kawano M, et al. 2003. Molecular machinery for non-vesicular trafficking of ceramide. Nature 426:803-9
-
(2003)
Nature
, vol.426
, pp. 803-809
-
-
Hanada, K.1
Kumagai, K.2
Yasuda, S.3
Miura, Y.4
Kawano, M.5
-
32
-
-
0024420051
-
Sphingolipid activator protein (SAP) deficiency in a 16-week old atypical Gaucher disease patient and his fetal sibling; biochemical signs of combined sphingolipidosis
-
Harzer K, Paton BC, Poulos A. 1989. Sphingolipid activator protein (SAP) deficiency in a 16-week old atypical Gaucher disease patient and his fetal sibling; biochemical signs of combined sphingolipidosis. Eur. J. Pediatr. 149:31-39
-
(1989)
Eur. J. Pediatr.
, vol.149
, pp. 31-39
-
-
Harzer, K.1
Paton, B.C.2
Poulos, A.3
-
33
-
-
2342445863
-
Solution structure of the pore forming protein of entamoeba histolytic
-
Hecht O, Van Nuland NA, Schleinkofer K, Dingley AJ, Bruhn H, et al. 2004. Solution structure of the pore forming protein of entamoeba histolytic. J. Biol. Chem. 279:17834-41
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 17834-17841
-
-
Hecht, O.1
Van Nuland, N.A.2
Schleinkofer, K.3
Dingley, A.J.4
Bruhn, H.5
-
34
-
-
0036841874
-
Triton promotes domain formation in lipid raft mixtures
-
Heerklotz H. 2002. Triton promotes domain formation in lipid raft mixtures. Biophys. J. 83:2693-701
-
(2002)
Biophys. J.
, vol.83
, pp. 2693-2701
-
-
Heerklotz, H.1
-
35
-
-
0038730762
-
The sensitivity of lipid domains to small perturbations demonstrated by the effect of Triton
-
Heerklotz H, Szadkowska H, Anderson T, Seelig J. 2003. The sensitivity of lipid domains to small perturbations demonstrated by the effect of Triton. J. Mol. Biol. 329:793-99
-
(2003)
J. Mol. Biol.
, vol.329
, pp. 793-799
-
-
Heerklotz, H.1
Szadkowska, H.2
Anderson, T.3
Seelig, J.4
-
37
-
-
0037169518
-
Physiological substrates for human lysosomal β-hexosaminidase S
-
Hepbildikler ST, Sandhoff R, Kölzer M, Proia RL, Sandhoff K. 2002. Physiological substrates for human lysosomal β-hexosaminidase S. J. Biol. Chem. 277:2562-72
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 2562-2572
-
-
Hepbildikler, S.T.1
Sandhoff, R.2
Kölzer, M.3
Proia, R.L.4
Sandhoff, K.5
-
38
-
-
0032541422
-
Cellular uptake of saposin (SAP) precursor and lysosomal delivery by the low density lipoprotein receptor-related protein (LRP)
-
Hiesberger T, Hüttler S, Rohlmann A, Schneider W, Sandhoff K, Herz J. 1998. Cellular uptake of saposin (SAP) precursor and lysosomal delivery by the low density lipoprotein receptor-related protein (LRP). EMBO J. 17:4617-25
-
(1998)
EMBO J.
, vol.17
, pp. 4617-4625
-
-
Hiesberger, T.1
Hüttler, S.2
Rohlmann, A.3
Schneider, W.4
Sandhoff, K.5
Herz, J.6
-
39
-
-
0015154711
-
Gaucher's disease: Deficiency of 'acid' β-glucosidase and reconstitution of enzyme activity in vitro
-
Ho MW, O'Brien JS. 1971. Gaucher's disease: deficiency of 'acid' β-glucosidase and reconstitution of enzyme activity in vitro. Proc. Natl. Acad. Sci. USA 68:2810-13
-
(1971)
Proc. Natl. Acad. Sci. USA
, vol.68
, pp. 2810-2813
-
-
Ho, M.W.1
O'Brien, J.S.2
-
40
-
-
0025281993
-
Movement of internalized ligand-receptor complexes along a continuous endosomal reticulum
-
Hopkins CR, Gibson A, Shipman M, Miller K. 1990. Movement of internalized ligand-receptor complexes along a continuous endosomal reticulum. Nature 346:335-39
-
(1990)
Nature
, vol.346
, pp. 335-339
-
-
Hopkins, C.R.1
Gibson, A.2
Shipman, M.3
Miller, K.4
-
41
-
-
1142263116
-
Saposins facilitate CD1d-restricted presentation of an exogenous lipid antigen to T cells
-
Kang SJ, Cresswell P. 2004. Saposins facilitate CD1d-restricted presentation of an exogenous lipid antigen to T cells. Nature Immunol. 5:175-81
-
(2004)
Nature Immunol
, vol.5
, pp. 175-181
-
-
Kang, S.J.1
Cresswell, P.2
-
42
-
-
0035958546
-
Ubiquitin-dependent sorting into the multivesicular body pathway requires the function of a conserved endosomal protein sorting complex, ESCRT-I
-
Katzmann DJ, Babst M, Emr SD. 2001. Ubiquitin-dependent sorting into the multivesicular body pathway requires the function of a conserved endosomal protein sorting complex, ESCRT-I. Cell 106:145-55
-
(2001)
Cell
, vol.106
, pp. 145-155
-
-
Katzmann, D.J.1
Babst, M.2
Emr, S.D.3
-
43
-
-
0028275240
-
Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo
-
Klein A, Henseler M, Klein C, Suzuki K, Harzer K, Sandhoff K. 1994. Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo. Biochem. Biophys. Res. Commun. 200:1440-48
-
(1994)
Biochem. Biophys. Res. Commun.
, vol.200
, pp. 1440-1448
-
-
Klein, A.1
Henseler, M.2
Klein, C.3
Suzuki, K.4
Harzer, K.5
Sandhoff, K.6
-
44
-
-
0033145510
-
Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport
-
Kobayashi T, Beuchat M-H, Lindsay M, Frias S, Palmiter RD. 1999. Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport. Nature Cell Biol. 1:113-18
-
(1999)
Nature Cell Biol
, vol.1
, pp. 113-118
-
-
Kobayashi, T.1
Beuchat, M.-H.2
Lindsay, M.3
Frias, S.4
Palmiter, R.D.5
-
45
-
-
0032510559
-
A lipid associated with the antiphospholipid syndrome regulates endosome structure and function
-
Kobayashi T, Stang E, Fang KS, de Moerloose P, Parton RG, Gruenberg J. 1998. A lipid associated with the antiphospholipid syndrome regulates endosome structure and function. Nature 392:193-97
-
(1998)
Nature
, vol.392
, pp. 193-197
-
-
Kobayashi, T.1
Stang, E.2
Fang, K.S.3
De Moerloose, P.4
Parton, R.G.5
Gruenberg, J.6
-
46
-
-
0037135608
-
Minireview: Combinatorial ganglioside biosynthesis
-
Kolter T, Proia RL, Sandhoff K. 2002. Minireview: combinatorial ganglioside biosynthesis. J. Biol. Chem. 277:25859-62
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 25859-25862
-
-
Kolter, T.1
Proia, R.L.2
Sandhoff, K.3
-
47
-
-
0031973892
-
Recent advances in the biochemistry of sphingolipidoses
-
Kolter T, Sandhoff K. 1998. Recent advances in the biochemistry of sphingolipidoses. Brain Pathol. 8:79-100
-
(1998)
Brain Pathol
, vol.8
, pp. 79-100
-
-
Kolter, T.1
Sandhoff, K.2
-
48
-
-
0033152727
-
Sphingolipids-their metabolic pathways and the pathobiochemistry of neurodegenerative diseases
-
Kolter T, Sandhoff K. 1999. Sphingolipids-their metabolic pathways and the pathobiochemistry of neurodegenerative diseases. Angew. Chem. Int. Ed. 38:1532-68
-
(1999)
Angew. Chem. Int. Ed.
, vol.38
, pp. 1532-1568
-
-
Kolter, T.1
Sandhoff, K.2
-
49
-
-
0025321793
-
Characterization of a mutation in a family with saposin B deficiency: A glycosylation site defect
-
Kretz KA, Carson GS, Morimoto S, Kishimoto Y, Fluharty AL, O'Brien JS. 1990. Characterization of a mutation in a family with saposin B deficiency: a glycosylation site defect. Proc. Natl. Acad. Sci. USA 87:2541-44
-
(1990)
Proc. Natl. Acad. Sci. USA
, vol.87
, pp. 2541-2544
-
-
Kretz, K.A.1
Carson, G.S.2
Morimoto, S.3
Kishimoto, Y.4
Fluharty, A.L.5
O'Brien, J.S.6
-
50
-
-
0345732689
-
The lysosomal trafficking of sphingolipid activator proteins (SAPs) is mediated by sortilin
-
Lefrancois S, Zeng J, Hassan AJ, Canuel M, Morales CR. 2003. The lysosomal trafficking of sphingolipid activator proteins (SAPs) is mediated by sortilin. EMBO J. 22:6430-37
-
(2003)
EMBO J
, vol.22
, pp. 6430-6437
-
-
Lefrancois, S.1
Zeng, J.2
Hassan, A.J.3
Canuel, M.4
Morales, C.R.5
-
51
-
-
0021946347
-
Activator protein required for the enzymatic hydrolysis of cerebroside sulfate. Deficiency in urine of patients affected with cerebroside sulfatase activator deficiency and identity with activators for the enzymatic hydrolysis of GM1 ganglioside and globotriaosylceramide
-
Li SC, Kihara H, Serizawa S, Li YT, Fluharty AL, Mayes JS, Shapiro LJ. 1985. Activator protein required for the enzymatic hydrolysis of cerebroside sulfate. Deficiency in urine of patients affected with cerebroside sulfatase activator deficiency and identity with activators for the enzymatic hydrolysis of GM1 ganglioside and globotriaosylceramide. J. Biol. Chem. 260:1867-71
-
(1985)
J. Biol. Chem.
, vol.260
, pp. 1867-1871
-
-
Li, S.C.1
Kihara, H.2
Serizawa, S.3
Li, Y.T.4
Fluharty, A.L.5
Mayes, J.S.6
Shapiro, L.J.7
-
52
-
-
0023919106
-
Characterization of a nonspecific activator protein for the enzymatic hydrolysis of glycolipids
-
Li SC, Sonnino S, Tettamanti G, Li YT. 1988. Characterization of a nonspecific activator protein for the enzymatic hydrolysis of glycolipids. J. Biol. Chem. 263:6588-91
-
(1988)
J. Biol. Chem.
, vol.263
, pp. 6588-6591
-
-
Li, S.C.1
Sonnino, S.2
Tettamanti, G.3
Li, Y.T.4
-
54
-
-
0035077616
-
Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins
-
Linke T, Wilkening G, Lansmann S, Moczall H, Bartelsen O, et al. 2001a. Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins. Biol. Chem. 382:283-90
-
(2001)
Biol. Chem.
, vol.382
, pp. 283-290
-
-
Linke, T.1
Wilkening, G.2
Lansmann, S.3
Moczall, H.4
Bartelsen, O.5
-
55
-
-
0035937146
-
Interfacial regulation of acid ceramidase activity. Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins
-
Linke T, Wilkening G, Sadeghlar F, Mozcall H, Bernardo K, et al. 2001b. Interfacial regulation of acid ceramidase activity. Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins. J. Biol. Chem. 276:5760-68
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 5760-5768
-
-
Linke, T.1
Wilkening, G.2
Sadeghlar, F.3
Mozcall, H.4
Bernardo, K.5
-
56
-
-
0034639928
-
Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse
-
Liu Y, Wu Y-P, Wada R, Neufeld EB, Mullin KA, et al. 2000. Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse. Hum. Mol. Genet. 9:1087-92
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 1087-1092
-
-
Liu, Y.1
Wu, Y.-P.2
Wada, R.3
Neufeld, E.B.4
Mullin, K.A.5
-
57
-
-
1642293929
-
Ceramide selectively displaces cholesterol from ordered lipid domains (rafts): Implications for lipid raft structure and function
-
London M, London E. 2004. Ceramide selectively displaces cholesterol from ordered lipid domains (rafts): implications for lipid raft structure and function. J. Biol. Chem. 279:9997-10004
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 9997-10004
-
-
London, M.1
London, E.2
-
58
-
-
0036694756
-
Biochemical and structural information transduction at the mesoscopic level in biointerfaces containing sphingolipids
-
Maggio B, Fanani ML, Oliveira RG. 2002. Biochemical and structural information transduction at the mesoscopic level in biointerfaces containing sphingolipids. Neurochem. Res. 27:547-57
-
(2002)
Neurochem. Res.
, vol.27
, pp. 547-557
-
-
Maggio, B.1
Fanani, M.L.2
Oliveira, R.G.3
-
59
-
-
0037414455
-
The X-ray crystal structure of human β-hexosaminidase B provides new insights into Sandhoff disease
-
Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W. 2003. The X-ray crystal structure of human β-hexosaminidase B provides new insights into Sandhoff disease. J. Mol. Biol. 328:669-81
-
(2003)
J. Mol. Biol.
, vol.328
, pp. 669-681
-
-
Maier, T.1
Strater, N.2
Schuette, C.G.3
Klingenstein, R.4
Sandhoff, K.5
Saenger, W.6
-
60
-
-
4344716055
-
Structural basis for glycosphingolipid transfer specificity
-
Malinina L, Malakhova ML, Teplov A, Brown RE, Patel DJ. 2004. Structural basis for glycosphingolipid transfer specificity. Nature 430:1048-53
-
(2004)
Nature
, vol.430
, pp. 1048-1053
-
-
Malinina, L.1
Malakhova, M.L.2
Teplov, A.3
Brown, R.E.4
Patel, D.J.5
-
61
-
-
0344837327
-
Crystal structure of human β-hexosaminidase B: Understanding the molecular basis of Sandhoff and Tay-Sachs disease
-
Mark BL, Mahuran DJ, Cherney MM, Zhao D, Knapp S, James MN. 2003. Crystal structure of human β-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease. J. Mol. Biol. 327:1093-109
-
(2003)
J. Mol. Biol.
, vol.327
, pp. 1093-1109
-
-
Mark, B.L.1
Mahuran, D.J.2
Cherney, M.M.3
Zhao, D.4
Knapp, S.5
James, M.N.6
-
62
-
-
0030606012
-
Lateral pressure in membranes
-
Marsh D. 1996. Lateral pressure in membranes. Biochim. Biophys. Acta 1286:183-223
-
(1996)
Biochim. Biophys. Acta
, vol.1286
, pp. 183-223
-
-
Marsh, D.1
-
63
-
-
0035830645
-
Interaction of ceramides with phosphatidylcholine, sphingomyelin and sphingomyelin/cholesterol bilayers
-
Massey JB. 2001. Interaction of ceramides with phosphatidylcholine, sphingomyelin and sphingomyelin/cholesterol bilayers. Biochim. Biophys. Acta 1510:167-84
-
(2001)
Biochim. Biophys. Acta
, vol.1510
, pp. 167-184
-
-
Massey, J.B.1
-
64
-
-
0035873272
-
A mutation in the saposin a domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse
-
Matsuda J, Vanier MT, Saito Y, Tohyama J, Suzuki K. 2001. A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse. Hum. Mol. Genet. 10:1191-99
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1191-1199
-
-
Matsuda, J.1
Vanier, M.T.2
Saito, Y.3
Tohyama, J.4
Suzuki, K.5
-
65
-
-
8444224225
-
Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse
-
Matsuda J, Kido M, Tadano-Aritomi K, Ishizuka I, Tominaga K, et al. 2004. Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse. Hum. Mol. Genet. 13:2709-23
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 2709-2723
-
-
Matsuda, J.1
Kido, M.2
Tadano-Aritomi, K.3
Ishizuka, I.4
Tominaga, K.5
-
66
-
-
0018786885
-
Degradation of bis(monoacylglycero)phosphate by an acid phosphodiesterase in rat liver lysosomes
-
Matsuzawa Y, Hostetler KY. 1979. Degradation of bis(monoacylglycero) phosphate by an acid phosphodiesterase in rat liver lysosomes. J. Biol. Chem. 254:5997-6001
-
(1979)
J. Biol. Chem.
, vol.254
, pp. 5997-6001
-
-
Matsuzawa, Y.1
Hostetler, K.Y.2
-
69
-
-
0037135536
-
De novo sphingolipid biosynthesis: A necessary, but dangerous, pathway
-
Merrill AH Jr. 2002. De novo sphingolipid biosynthesis: a necessary, but dangerous, pathway. J. Biol. Chem. 277:25843-46
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 25843-25846
-
-
Merrill Jr., A.H.1
-
70
-
-
0033361755
-
Niemann-Pick C1 disease: The 1106 IT substitution is a frequent mutant allele in patients of Western European descent and correlates with a classic juvenile phenotype
-
Millat G, Marcais C, Rafi MA, Yamamoto T, Morris JA, et al. 1999. Niemann-Pick C1 disease: the 1106 IT substitution is a frequent mutant allele in patients of Western European descent and correlates with a classic juvenile phenotype. Am. J. Hum. Genet. 65:1321-29
-
(1999)
Am. J. Hum. Genet.
, vol.65
, pp. 1321-1329
-
-
Millat, G.1
Marcais, C.2
Rafi, M.A.3
Yamamoto, T.4
Morris, J.A.5
-
71
-
-
0034755958
-
Niemann-Pick disease type C: Spectrum of HE1 mutations and genotype/phenotype correlations in the NPC2 group
-
Millat G, Chikh K, Naureckiene S, Sleat DE, Fensom AH, et al. 2001. Niemann-Pick disease type C: spectrum of HE1 mutations and genotype/phenotype correlations in the NPC2 group. Am. J. Hum. Genet. 69:1013-21
-
(2001)
Am. J. Hum. Genet.
, vol.69
, pp. 1013-1021
-
-
Millat, G.1
Chikh, K.2
Naureckiene, S.3
Sleat, D.E.4
Fensom, A.H.5
-
72
-
-
0032799717
-
Intracellular distribution of a biotin-labeled ganglioside GM1 by immunoelectron microscopy after endocytosis in fibroblasts
-
Möbius W, Herzog V, Sandhoff K, Schwarzmann G. 1999a. Intracellular distribution of a biotin-labeled ganglioside GM1 by immunoelectron microscopy after endocytosis in fibroblasts. J. Histochem. Cytochem. 47:1005-14
-
(1999)
J. Histochem. Cytochem.
, vol.47
, pp. 1005-1014
-
-
Möbius, W.1
Herzog, V.2
Sandhoff, K.3
Schwarzmann, G.4
-
73
-
-
0032716881
-
Gangliosides are transported from the plasma membrane to intralysosomal membranes as revealed by immuno-electron microscopy
-
Möbius W, Herzog V, Sandhoff K, Schwarzmann G. 1999b. Gangliosides are transported from the plasma membrane to intralysosomal membranes as revealed by immuno-electron microscopy. Biosci. Rep. 19:307-16
-
(1999)
Biosci. Rep.
, vol.19
, pp. 307-316
-
-
Möbius, W.1
Herzog, V.2
Sandhoff, K.3
Schwarzmann, G.4
-
74
-
-
0038620205
-
Recycling compartments and the internal vesicles of multivesicular bodies harbor most of the cholesterol found in the endocytic pathway
-
Möbius W, van Donselaar E, Ohno-Iwashita Y, Shimada Y, Heijnen HF, et al. 2003. Recycling compartments and the internal vesicles of multivesicular bodies harbor most of the cholesterol found in the endocytic pathway. Traffic 4:222-31
-
(2003)
Traffic
, vol.4
, pp. 222-231
-
-
Möbius, W.1
Van Donselaar, E.2
Ohno-Iwashita, Y.3
Shimada, Y.4
Heijnen, H.F.5
-
76
-
-
0029126584
-
Saposin-like proteins (SAPLIP) carry out diverse functions on a common backbone structure
-
Munford RS, Sheppard PO, O'Hara PJ. 1995. Saposin-like proteins (SAPLIP) carry out diverse functions on a common backbone structure. J. Lipid. Res. 36:1653-63
-
(1995)
J. Lipid. Res.
, vol.36
, pp. 1653-1663
-
-
Munford, R.S.1
Sheppard, P.O.2
O'Hara, P.J.3
-
77
-
-
0344585437
-
Lipid rafts: Elusive or illusive?
-
Munro S. 2003. Lipid rafts: elusive or illusive? Cell 115:377-88
-
(2003)
Cell
, vol.115
, pp. 377-388
-
-
Munro, S.1
-
78
-
-
0024593996
-
Structure of full-length cDNA coding for sulfatide activator, a Co-β-glucosidase and two other homologous proteins: Two alternate forms of the sulfatide activator
-
Nakano T, Sandhoff K, Stumper J, Christomanou H, Suzuki K. 1989. Structure of full-length cDNA coding for sulfatide activator, a Co-β-glucosidase and two other homologous proteins: two alternate forms of the sulfatide activator. J. Biochem. 105:152-54
-
(1989)
J. Biochem.
, vol.105
, pp. 152-154
-
-
Nakano, T.1
Sandhoff, K.2
Stumper, J.3
Christomanou, H.4
Suzuki, K.5
-
79
-
-
0034704245
-
Identification of HE1 as the second gene of Niemann-Pick C disease
-
Naureckiene S, Sleat DE, Lackland H, Fensom A, Vanier MT, et al. 2000. Identification of HE1 as the second gene of Niemann-Pick C disease. Science 290:2298-301
-
(2000)
Science
, vol.290
, pp. 2298-2301
-
-
Naureckiene, S.1
Sleat, D.E.2
Lackland, H.3
Fensom, A.4
Vanier, M.T.5
-
80
-
-
0024297787
-
Coding of two sphingolipid activator proteins (SAP-1 and SAP-2) by same genetic locus
-
O'Brien JS, Kretz KA, Dewji N, Wenger DA, Esch F, Fluharty AL. 1988. Coding of two sphingolipid activator proteins (SAP-1 and SAP-2) by same genetic locus. Science 241: 1098-1101
-
(1988)
Science
, vol.241
, pp. 1098-1101
-
-
O'Brien, J.S.1
Kretz, K.A.2
Dewji, N.3
Wenger, D.A.4
Esch, F.5
Fluharty, A.L.6
-
81
-
-
0033542422
-
A porcine homolog of the major secretory protein of human epididymis, HE1, specifically binds cholesterol
-
Okamura N, Kiuchi S, Tamba M, Kashima T, Hiramoto S, et al. 1999. A porcine homolog of the major secretory protein of human epididymis, HE1, specifically binds cholesterol. Biochim. Biophys. Acta 1438:377-87
-
(1999)
Biochim. Biophys. Acta
, vol.1438
, pp. 377-387
-
-
Okamura, N.1
Kiuchi, S.2
Tamba, M.3
Kashima, T.4
Hiramoto, S.5
-
82
-
-
0037450544
-
Specific lipid requirement of membrane proteins-a putative bottleneck in heterologous expression
-
Opekarová M, Tanner W. 2003. Specific lipid requirement of membrane proteins-a putative bottleneck in heterologous expression. Biochim. Biophys. Acta 1610:11-22
-
(2003)
Biochim. Biophys. Acta
, vol.1610
, pp. 11-22
-
-
Opekarová, M.1
Tanner, W.2
-
83
-
-
0023716489
-
Human acid β-glucosidase: Inhibition studies using glucose analogues and pH variation to characterize the normal and Gaucher disease glycon binding sites
-
Osiecki-Newman K, Legler G, Grace M, Dinur T, Gatt S, et al. 1988. Human acid β-glucosidase: inhibition studies using glucose analogues and pH variation to characterize the normal and Gaucher disease glycon binding sites. Enzyme 40:173-88
-
(1988)
Enzyme
, vol.40
, pp. 173-188
-
-
Osiecki-Newman, K.1
Legler, G.2
Grace, M.3
Dinur, T.4
Gatt, S.5
-
84
-
-
0344875214
-
A riddle wrapped in a mystery: Understanding Niemann-Pick disease, type C
-
Patterson MC. 2003. A riddle wrapped in a mystery: understanding Niemann-Pick disease, type C. Neurology 9:301-10
-
(2003)
Neurology
, vol.9
, pp. 301-310
-
-
Patterson, M.C.1
-
85
-
-
0000831301
-
Niemann-Pick disease type C: A lipid trafficking disorder
-
ed. CR Scriver, AL Beaudet, WS Sly, D Valle, New York: McGraw-Hill. 8th ed.
-
Patterson MC, Vanier MT, Suzuki K, Morris JA, Carstea E, et al. 2001. Niemann-Pick disease type C: a lipid trafficking disorder. In The Metabolic and Molecular Bases of Inherited Disease, ed. CR Scriver, AL Beaudet, WS Sly, D Valle, III:3611-33. New York: McGraw-Hill. 8th ed.
-
(2001)
The Metabolic and Molecular Bases of Inherited Disease
, vol.3
, pp. 3611-3633
-
-
Patterson, M.C.1
Vanier, M.T.2
Suzuki, K.3
Morris, J.A.4
Carstea, E.5
-
86
-
-
0021346802
-
A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol
-
Pentchev PG, Boothe AD, Kruth HS, Weintroub H, Stivers J, Brady RO. 1984. A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol. J. Biol. Chem. 259:5784-91
-
(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
-
87
-
-
0022980841
-
The cholesterol storage disorder of the mutant BALB/c mouse
-
Pentchev PG, Comly ME, Kruth HS, Patel S, Proestel M, Weintroub H. 1986. The cholesterol storage disorder of the mutant BALB/c mouse. J. Biol. Chem. 261:2772-77
-
(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
-
88
-
-
10644282870
-
Lysosomal defects and storage
-
ed. FM Platt, SU Walkley, New York: Oxford Univ. Press
-
Platt FM, Walkley SU. 2004. Lysosomal defects and storage. In Lysosomal Disorders of the Brain, ed. FM Platt, SU Walkley, pp. 32-49. New York: Oxford Univ. Press
-
(2004)
Lysosomal Disorders of the Brain
, pp. 32-49
-
-
Platt, F.M.1
Walkley, S.U.2
-
89
-
-
0034697239
-
Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids
-
Salvioli R, Tatti M, Ciaffoni F, Vaccaro AM. 2000. Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids. FEBS Lett. 472:17-21
-
(2000)
FEBS Lett
, vol.472
, pp. 17-21
-
-
Salvioli, R.1
Tatti, M.2
Ciaffoni, F.3
Vaccaro, A.M.4
-
90
-
-
0000857916
-
Sphingolipid activator proteins
-
ed. CR Scriver, AL Beaudet, WS Sly, D Valle, New York: McGraw-Hill. 8th ed.
-
Sandhoff K, Kolter T, Harzer K. 2001. Sphingolipid activator proteins. In The Metabolic and Molecular Bases of Inherited Disease, ed. CR Scriver, AL Beaudet, WS Sly, D Valle, III:3371-88. New York: McGraw-Hill. 8th ed.
-
(2001)
The Metabolic and Molecular Bases of Inherited Disease
, vol.3
, pp. 3371-3388
-
-
Sandhoff, K.1
Kolter, T.2
Harzer, K.3
-
91
-
-
0029670774
-
Topology of glycosphingolipid degradation
-
Sandhoff K, Kolter T. 1996. Topology of glycosphingolipid degradation. Trends Cell Biol. 6:98-103
-
(1996)
Trends Cell Biol
, vol.6
, pp. 98-103
-
-
Sandhoff, K.1
Kolter, T.2
-
92
-
-
0022894809
-
Specificity of human glucosylceramide β-glucosidase towards synthetic glucosylsphingolipids inserted into liposomes. Kinetic studies in a detergent-free assay system
-
Sarmientos F, Schwarzmann G, Sandhoff K. 1986. Specificity of human glucosylceramide β-glucosidase towards synthetic glucosylsphingolipids inserted into liposomes. Kinetic studies in a detergent-free assay system. Eur. J. Biochem. 160:527-35
-
(1986)
Eur. J. Biochem.
, vol.160
, pp. 527-535
-
-
Sarmientos, F.1
Schwarzmann, G.2
Sandhoff, K.3
-
93
-
-
0026705846
-
Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene
-
Schnabel D, Schröder M, Fürst W, Klein A, Hurwitz R, et al. 1992. Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene. J. Biol. Chem. 267:3312-15
-
(1992)
J. Biol. Chem.
, vol.267
, pp. 3312-3315
-
-
Schnabel, D.1
Schröder, M.2
Fürst, W.3
Klein, A.4
Hurwitz, R.5
-
94
-
-
0025762364
-
Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease
-
Schnabel D, Schröder M, Sandhoff K. 1991. Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease. FEBS Lett. 284:57-59
-
(1991)
FEBS Lett
, vol.284
, pp. 57-59
-
-
Schnabel, D.1
Schröder, M.2
Sandhoff, K.3
-
95
-
-
0030949124
-
Functional rafts in cell membranes
-
Simons K, Ikonen E. 1997. Functional rafts in cell membranes. Nature 387:569-72
-
(1997)
Nature
, vol.387
, pp. 569-572
-
-
Simons, K.1
Ikonen, E.2
-
96
-
-
12844280581
-
A mutation in the saposin a coding region of the prosaposin gene in an infant presenting as Krabbe disease: First report of saposin a deficiency in humans
-
Spiegel R, Bach G, Sury V, Mengistu G, Meidan B, et al. 2005. A mutation in the saposin A coding region of the prosaposin gene in an infant presenting as Krabbe disease: first report of saposin A deficiency in humans. Mol. Genet. Metab. 84:160-66
-
(2005)
Mol. Genet. Metab.
, vol.84
, pp. 160-166
-
-
Spiegel, R.1
Bach, G.2
Sury, V.3
Mengistu, G.4
Meidan, B.5
-
97
-
-
0041355292
-
Saposin C is required for normal resistance of acid β-glucosidase to proteolytic degradation
-
Sun Y, Qi X, Grabowski GA. 2003. Saposin C is required for normal resistance of acid β-glucosidase to proteolytic degradation. J. Biol. Chem. 278:31918-23
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 31918-31923
-
-
Sun, Y.1
Qi, X.2
Grabowski, G.A.3
-
98
-
-
0013256537
-
Genetic disorders of lipid, glycoprotein, and mucopolysaccharide metabolism
-
ed. GJ Siegel, BW Agranoff, RW Albers, PB Molinoff, New York: Raven. 5th ed.
-
Suzuki K. 1994. Genetic disorders of lipid, glycoprotein, and mucopolysaccharide metabolism. In Basic Neurochemistry: Molecular, Cellular, and Medical Aspects, ed. GJ Siegel, BW Agranoff, RW Albers, PB Molinoff, pp. 793-812. New York: Raven. 5th ed.
-
(1994)
Basic Neurochemistry: Molecular, Cellular, and Medical Aspects
, pp. 793-812
-
-
Suzuki, K.1
-
99
-
-
0014224649
-
GM1-gangliosidosis (generalized gangliosidosis). Morphology and chemical pathology
-
Suzuki K, Chen GC. 1968. GM1-gangliosidosis (generalized gangliosidosis). Morphology and chemical pathology. Pathol. Eur. 3:389-408
-
(1968)
Pathol. Eur.
, vol.3
, pp. 389-408
-
-
Suzuki, K.1
Chen, G.C.2
-
100
-
-
0343844648
-
Lysosomal and peroxisomal diseases
-
ed. GJ Siegel, BW Agranoff, RW Albers, SK Fisher, MD Uhler, Philadelphia: Lippincott-Raven. 6th ed.
-
Suzuki K, Vanier MT. 1999. Lysosomal and peroxisomal diseases. In Basic Neurochemistry-Molecular, Cellular and Medical Aspects, ed. GJ Siegel, BW Agranoff, RW Albers, SK Fisher, MD Uhler, pp. 821-39. Philadelphia: Lippincott-Raven. 6th ed.
-
(1999)
Basic Neurochemistry-Molecular, Cellular and Medical Aspects
, pp. 821-839
-
-
Suzuki, K.1
Vanier, M.T.2
-
101
-
-
3543085572
-
Ganglioside/glycosphingolipid turnover: New concepts
-
Tettamanti G. 2004. Ganglioside/glycosphingolipid turnover: new concepts. Glycoconjugate J. 20:301-17
-
(2004)
Glycoconjugate J
, vol.20
, pp. 301-317
-
-
Tettamanti, G.1
-
102
-
-
1342268375
-
The roles of ubiquitin and lipids in protein sorting along the endocytic pathway
-
Umebayashi K. 2003. The roles of ubiquitin and lipids in protein sorting along the endocytic pathway. Cell. Struct. Funct. 28:443-53
-
(2003)
Cell. Struct. Funct.
, vol.28
, pp. 443-453
-
-
Umebayashi, K.1
-
103
-
-
0029417339
-
PH-dependent conformational properties of saposins and their interactions with phospholipid membranes
-
Vaccaro AM, Ciaffoni F, Tatti M, Salvioli R, Barca A, et al. 1995. pH-dependent conformational properties of saposins and their interactions with phospholipid membranes. J. Biol. Chem. 270:30576-80
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 30576-30580
-
-
Vaccaro, A.M.1
Ciaffoni, F.2
Tatti, M.3
Salvioli, R.4
Barca, A.5
-
105
-
-
0037135518
-
Sphingolipid transport: Rafts and translocators
-
van Meer G, Lisman Q. 2002. Sphingolipid transport: rafts and translocators. J. Biol. Chem. 277:25855-58
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 25855-25858
-
-
Meer, G.1
Lisman, Q.2
-
106
-
-
0027532181
-
Demonstration of the existence of a second, non-lysosomal glucocerebrosidase that is not deficient in Gaucher disease
-
van Weely S, Brandsma M, Strijland A, Tager JM, Aerts JM. 1993. Demonstration of the existence of a second, non-lysosomal glucocerebrosidase that is not deficient in Gaucher disease. Biochim. Biophys. Acta 1181:55-62
-
(1993)
Biochim. Biophys. Acta
, vol.1181
, pp. 55-62
-
-
Weely, S.1
Brandsma, M.2
Strijland, A.3
Tager, J.M.4
Aerts, J.M.5
-
107
-
-
0025787230
-
Glycosphingolipid specificity of the human sulfatide activator protein
-
Vogel A, Schwarzmann G, Sandhoff K. 1991. Glycosphingolipid specificity of the human sulfatide activator protein. Eur. J. Biochem. 200:591-97
-
(1991)
Eur. J. Biochem.
, vol.200
, pp. 591-597
-
-
Vogel, A.1
Schwarzmann, G.2
Sandhoff, K.3
-
109
-
-
0842330527
-
Photoaffinity labelling of the human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation
-
Wendeler M, Hoernschemeyer J, Hoffmann D, Kolter T, Schwarzmann G, Sandhoff K. 2004. Photoaffinity labelling of the human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation. Eur. J. Biochem. 271:614-27
-
(2004)
Eur. J. Biochem.
, vol.271
, pp. 614-627
-
-
Wendeler, M.1
Hoernschemeyer, J.2
Hoffmann, D.3
Kolter, T.4
Schwarzmann, G.5
Sandhoff, K.6
-
110
-
-
0035918181
-
Degradation of membrane-bound ganglioside GM2 by β-hexosaminidase A. Stimulation by GM2 activator protein and lysosomal lipids
-
Werth N, Schuette CG, Wilkening G, Lemm T, Sandhoff K. 2001. Degradation of membrane-bound ganglioside GM2 by β-hexosaminidase A. Stimulation by GM2 activator protein and lysosomal lipids. J. Biol. Chem. 276:12685-90
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 12685-12690
-
-
Werth, N.1
Schuette, C.G.2
Wilkening, G.3
Lemm, T.4
Sandhoff, K.5
-
111
-
-
0032514902
-
Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators
-
Wilkening G, Linke T, Sandhoff K. 1998. Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators. J. Biol. Chem. 273:30271-78
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 30271-30278
-
-
Wilkening, G.1
Linke, T.2
Sandhoff, K.3
-
113
-
-
10744232076
-
Saposin C is required for lipid presentation by human CD1b
-
Winau F, Schwierzeck V, Hurwitz R, Remmel N, Sieling PA, et al. 2004. Saposin C is required for lipid presentation by human CD1b. Nat. Immunol. 5:169-74
-
(2004)
Nat. Immunol.
, vol.5
, pp. 169-174
-
-
Winau, F.1
Schwierzeck, V.2
Hurwitz, R.3
Remmel, N.4
Sieling, P.A.5
-
114
-
-
24044463804
-
Primary defects in lysosomal enzymes
-
ed. FM Platt, SU Walkley, New York: Oxford Univ. Press
-
Winchester BG. 2004. Primary defects in lysosomal enzymes. In Lysosomal Disorders of the Brain, ed. FM Platt, SU Walkley, pp. 81-130. New York: Oxford Univ. Press
-
(2004)
Lysosomal Disorders of the Brain
, pp. 81-130
-
-
Winchester, B.G.1
-
115
-
-
0034406526
-
Crystal structure of human GM2-activator protein with a novel β-cup topology
-
Wright CS, Li SC, Rastinejad F. 2000. Crystal structure of human GM2-activator protein with a novel β-cup topology. J. Mol. Biol. 304:411-22
-
(2000)
J. Mol. Biol.
, vol.304
, pp. 411-422
-
-
Wright, C.S.1
Li, S.C.2
Rastinejad, F.3
-
116
-
-
0042667012
-
Structural analysis of lipid complexes of GM2-activator protein
-
Wright CS, Zhao Q, Rastinejad F. 2003. Structural analysis of lipid complexes of GM2-activator protein. J. Mol. Biol. 331:951-64
-
(2003)
J. Mol. Biol.
, vol.331
, pp. 951-964
-
-
Wright, C.S.1
Zhao, Q.2
Rastinejad, F.3
-
117
-
-
0035823586
-
Effect of the structure of natural sterols and sphingolipids on the formation of ordered sphingolipid/sterol domains (rafts). Comparison of cholesterol to plant, fungal, and disease-associated sterols and comparison of sphingomyelin, cerebrosides, and ceramide
-
Xu X, Bittman R, Duportail G, Heissler D, Vilcheze C, London E. 2001. Effect of the structure of natural sterols and sphingolipids on the formation of ordered sphingolipid/sterol domains (rafts). Comparison of cholesterol to plant, fungal, and disease-associated sterols and comparison of sphingomyelin, cerebrosides, and ceramide. J. Biol. Chem. 276:33540-46
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 33540-33546
-
-
Xu, X.1
Bittman, R.2
Duportail, G.3
Heissler, D.4
Vilcheze, C.5
London, E.6
-
118
-
-
0037452930
-
Enhanced insulin sensitivity in mice lacking ganglioside GM3
-
Yamashita T, Hashiramoto A, Haluzik M, Mizukami H, Beck S, et al. 2003. Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proc. Natl. Acad. Sci. USA 100:3445-49
-
(2003)
Proc. Natl. Acad. Sci. USA
, vol.100
, pp. 3445-3449
-
-
Yamashita, T.1
Hashiramoto, A.2
Haluzik, M.3
Mizukami, H.4
Beck, S.5
-
119
-
-
0043198070
-
Crystal structure of CD1a in complex with a sulfatide self antigen at a resolution of 2.15 Å
-
Zajonc DM, Elsliger MA, Teyton L, Wilson IA. 2003. Crystal structure of CD1a in complex with a sulfatide self antigen at a resolution of 2.15 Å. Nat. Immunol. 4:808-15
-
(2003)
Nat. Immunol.
, vol.4
, pp. 808-815
-
-
Zajonc, D.M.1
Elsliger, M.A.2
Teyton, L.3
Wilson, I.A.4
-
120
-
-
0035142802
-
Neurons in Niemann-Pick disease Type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations
-
Zervas M, Dobrenis K, Walkley SU. 2001. Neurons in Niemann-Pick disease Type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. J. Neuropathol. Exp. Neurol. 60:49-64
-
(2001)
J. Neuropathol. Exp. Neurol.
, vol.60
, pp. 49-64
-
-
Zervas, M.1
Dobrenis, K.2
Walkley, S.U.3
-
121
-
-
9144256638
-
Editing of CD1d-bound lipid antigens by endosomal lipid transfer proteins
-
Zhou D, Cantu C 3rd, Sagiv Y, Schrantz N, Kulkarni AB, et al. 2004. Editing of CD1d-bound lipid antigens by endosomal lipid transfer proteins. Science 303:523-27
-
(2004)
Science
, vol.303
, pp. 523-527
-
-
Zhou, D.1
Cantu III, C.2
Sagiv, Y.3
Schrantz, N.4
Kulkarni, A.B.5
|