-
1
-
-
0025043421
-
Generation of a lysosomal protein targeting signal in the secretory protein pepsinogen
-
Baranski, T. J., Faust, P. L. & Kornfeld, S. (1990) Generation of a lysosomal protein targeting signal in the secretory protein pepsinogen, Cell 63, 281-291.
-
(1990)
Cell
, vol.63
, pp. 281-291
-
-
Baranski, T.J.1
Faust, P.L.2
Kornfeld, S.3
-
2
-
-
0027179238
-
Human β-glucosidase: N-glycosylation site occupancy and the effect of glycosylation on enzymatic activity
-
Berg-Fussman, A., Grace, M. E., Ioannou, Y. & Grabowski, G. A. (1993) Human β-glucosidase: N-glycosylation site occupancy and the effect of glycosylation on enzymatic activity, J. Biol. Chem. 268, 14861-14866.
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 14861-14866
-
-
Berg-Fussman, A.1
Grace, M.E.2
Ioannou, Y.3
Grabowski, G.A.4
-
3
-
-
0030050848
-
Functional expression of Fas and Fas ligand on human gut lamina propria T lymphocytes. A potential role for the acidic sphingomyelinase pathway in normal immunoregulation
-
DeMaria, R., Boirivant, M., Cifone, M. G., Roncaioli, P., Hahne, M., Tschopp, J., Pallone, F., Santoni, A. & Testi, R. (1996) Functional expression of Fas and Fas ligand on human gut lamina propria T lymphocytes. A potential role for the acidic sphingomyelinase pathway in normal immunoregulation, J. Clin. Invest. 97, 316-322.
-
(1996)
J. Clin. Invest.
, vol.97
, pp. 316-322
-
-
DeMaria, R.1
Boirivant, M.2
Cifone, M.G.3
Roncaioli, P.4
Hahne, M.5
Tschopp, J.6
Pallone, F.7
Santoni, A.8
Testi, R.9
-
4
-
-
0028136956
-
Occurrence of two molecular forms of human acid sphingomyclinase
-
Ferlinz, K., Hurwitz R., Vielhaber, G., Suzuki, K. & Sandhoff, K. (1994) Occurrence of two molecular forms of human acid sphingomyclinase, Biochem. J. 301, 855-862.
-
(1994)
Biochem. J.
, vol.301
, pp. 855-862
-
-
Ferlinz, K.1
Hurwitz, R.2
Vielhaber, G.3
Suzuki, K.4
Sandhoff, K.5
-
5
-
-
0026776768
-
In vitro mutagenesis of potential N-glycosylation sites of arylsulfatase A
-
Gieselmann, V., Schmidt, B. & von Figura, K. (1992) In vitro mutagenesis of potential N-glycosylation sites of arylsulfatase A, J. Biol. Chem. 267, 13262-13266.
-
(1992)
J. Biol. Chem.
, vol.267
, pp. 13262-13266
-
-
Gieselmann, V.1
Schmidt, B.2
Von Figura, K.3
-
6
-
-
0025284194
-
Human acid β-glucosidase: Glycosylation is required for catalytic activity
-
Grace, M. E. & Grabowski, G. A. (1990) Human acid β-glucosidase: glycosylation is required for catalytic activity, Biochem. Biophys. Res. Commun. 168, 771-777.
-
(1990)
Biochem. Biophys. Res. Commun.
, vol.168
, pp. 771-777
-
-
Grace, M.E.1
Grabowski, G.A.2
-
7
-
-
0027446596
-
Human lysosomal α-glucosidase: Functional characterization of the glycosylation sites
-
Hermans, M. M. P., Wisselaar, H. A., Kroos, M. A., Oostra, B. A. & Reuser, A. J. J. (1993) Human lysosomal α-glucosidase: functional characterization of the glycosylation sites, Biochem. J. 289, 681-685.
-
(1993)
Biochem. J.
, vol.289
, pp. 681-685
-
-
Hermans, M.M.P.1
Wisselaar, H.A.2
Kroos, M.A.3
Oostra, B.A.4
Reuser, A.J.J.5
-
8
-
-
0024520745
-
Site-directed mutagenesis by overlap extension using the polymerase chain reaction
-
Ho, S. N., Hunt, H. D., Horton, R. M., Pullen, J. K. & Pease, L. R. (1989) Site-directed mutagenesis by overlap extension using the polymerase chain reaction, Gene (Amst.) 77, 51-59.
-
(1989)
Gene (Amst.)
, vol.77
, pp. 51-59
-
-
Ho, S.N.1
Hunt, H.D.2
Horton, R.M.3
Pullen, J.K.4
Pease, L.R.5
-
9
-
-
0027984782
-
Processing of human acid sphingomyelinase in normal and I-cell fibroblasts
-
Hurwitz R., Ferlinz, K., Vielhaber, G., Moczall, H. & Sandhoff, K. (1994) Processing of human acid sphingomyelinase in normal and I-cell fibroblasts. J. Biol. Chem. 269, 5440-5445.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 5440-5445
-
-
Hurwitz, R.1
Ferlinz, K.2
Vielhaber, G.3
Moczall, H.4
Sandhoff, K.5
-
10
-
-
0007247166
-
Identification of the components necessary for the adenovirus translational control and their utilization in cDNA expression vectors
-
Kaufman, R. J. (1985) Identification of the components necessary for the adenovirus translational control and their utilization in cDNA expression vectors. Proc. Natl Acad. Sci. USA 82, 689-693.
-
(1985)
Proc. Natl Acad. Sci. USA
, vol.82
, pp. 689-693
-
-
Kaufman, R.J.1
-
11
-
-
0028275240
-
Sphingolipid activator protein D (sap D) stimulates the 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 degradation of ceramide in vivo. Biochem. Biophys. Res. Commun. 200, 1440-1448.
-
(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
-
12
-
-
0026735823
-
Ceramide: A novel second messenger
-
Kolesnick, R. (1992) Ceramide: a novel second messenger, Trends Cell Biol. 2, 232-236.
-
(1992)
Trends Cell Biol.
, vol.2
, pp. 232-236
-
-
Kolesnick, R.1
-
14
-
-
0000820864
-
I-cell disease and pseudo-Hurler polydystrophy: Disorders of lysosomal enzyme phosphorylation and localization
-
(Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D., eds) 7th edn, McGraw Hill, New York
-
Kornfeld, S. & Sly, W. S. (1995) I-cell disease and pseudo-Hurler polydystrophy: disorders of lysosomal enzyme phosphorylation and localization, in The metabolic and molecular basis of inherited disease (Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D., eds) vol. 2, 7th edn, pp. 2495-2508, McGraw Hill, New York.
-
(1995)
The Metabolic and Molecular Basis of Inherited Disease
, vol.2
, pp. 2495-2508
-
-
Kornfeld, S.1
Sly, W.S.2
-
15
-
-
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
-
16
-
-
0021676811
-
Lysosomal enzyme phosphorylation. Recognition of a protein-dependent determinant allows specific phosphorylation of oligosaccharides present on lysosomal enzymes
-
Lang, L., Reitman, M., Tang, J., Roberts, R. M. & Kornfeld, S. (1984) Lysosomal enzyme phosphorylation. Recognition of a protein-dependent determinant allows specific phosphorylation of oligosaccharides present on lysosomal enzymes, J. Biol. Chem. 259, 14663-14671.
-
(1984)
J. Biol. Chem.
, vol.259
, pp. 14663-14671
-
-
Lang, L.1
Reitman, M.2
Tang, J.3
Roberts, R.M.4
Kornfeld, S.5
-
17
-
-
0024561806
-
Mannose processing is an important determinant in the assembly of phosphorylated high-mannose type oligosaccharides
-
Lazzarino, D. A. & Gabel, C. A. (1988) Mannose processing is an important determinant in the assembly of phosphorylated high-mannose type oligosaccharides, J. Biol. Chem. 264, 5015-5023.
-
(1988)
J. Biol. Chem.
, vol.264
, pp. 5015-5023
-
-
Lazzarino, D.A.1
Gabel, C.A.2
-
18
-
-
0028353499
-
Acid sphingomyelinase possesses a domain homologous to its activator proteins: Saposins B and D
-
Ponting, C. P. (1994) Acid sphingomyelinase possesses a domain homologous to its activator proteins: saposins B and D, Protein Sci. 3, 359-361.
-
(1994)
Protein Sci.
, vol.3
, pp. 359-361
-
-
Ponting, C.P.1
-
19
-
-
0023574186
-
Acid sphingomyelinase from human urine: Purification and characterization
-
Quintern, L. E., Weitz, G., Nehrkorn, H., Tager, J. M., Schram, A. W. & Sandhoff, K. (1987) Acid sphingomyelinase from human urine: purification and characterization, Biochim. Biophys. Acta 922, 323-336.
-
(1987)
Biochim. Biophys. Acta
, vol.922
, pp. 323-336
-
-
Quintern, L.E.1
Weitz, G.2
Nehrkorn, H.3
Tager, J.M.4
Schram, A.W.5
Sandhoff, K.6
-
20
-
-
0024423426
-
Isolation of cDNA clones encoding human acid sphingomyelin: Occurrence of alternativly processed transcripts
-
Quintern, L. E., Schuchman, E. H., Levran, O., Suchi, M., Ferlinz, K., Reinke, H., Sandhoff, K. & Desnick, R. J. (1989) Isolation of cDNA clones encoding human acid sphingomyelin: occurrence of alternativly processed transcripts, EMBO J. 8, 2469-2473.
-
(1989)
EMBO J.
, vol.8
, pp. 2469-2473
-
-
Quintern, L.E.1
Schuchman, E.H.2
Levran, O.3
Suchi, M.4
Ferlinz, K.5
Reinke, H.6
Sandhoff, K.7
Desnick, R.J.8
-
21
-
-
0024672126
-
Structural requirements for protein N-glycosylation: Influence of acceptor peptides on cotranslational glycosylation of yeast invertase and site-directed mutagenesis around a sequon sequence
-
Roitsch, T. & Lehle, L. (1989) Structural requirements for protein N-glycosylation: influence of acceptor peptides on cotranslational glycosylation of yeast invertase and site-directed mutagenesis around a sequon sequence, Eur. J. Biochem. 181, 525-529.
-
(1989)
Eur. J. Biochem.
, vol.181
, pp. 525-529
-
-
Roitsch, T.1
Lehle, L.2
-
22
-
-
0004136246
-
-
Cold Spring Harbor Laboratory Press, Cold Spring Harbor NY
-
Sambrook, J., Fritsch, E. F. & Maniatis, T. (1989) Molecular cloning: a laboratory manual, 2nd edn. Cold Spring Harbor Laboratory Press, Cold Spring Harbor NY.
-
(1989)
Molecular Cloning: A Laboratory Manual, 2nd Edn.
-
-
Sambrook, J.1
Fritsch, E.F.2
Maniatis, T.3
-
23
-
-
0017681196
-
DNA sequencing with chain-terminating inhibitors
-
Sanger, F., Nicklen, S. & Coulson, A. R. (1977) DNA sequencing with chain-terminating inhibitors, Proc. Natl Acad. Sci. USA 74, 5463-5467.
-
(1977)
Proc. Natl Acad. Sci. USA
, vol.74
, pp. 5463-5467
-
-
Sanger, F.1
Nicklen, S.2
Coulson, A.R.3
-
24
-
-
0029666484
-
2+-stimulated sphingomyelinase is secreted in many cell types and is a product of the acid sphingomyelinase gene: Evidence for a second metabolic detect in types A and B Niemann-Pick disease
-
2+-stimulated sphingomyelinase is secreted in many cell types and is a product of the acid sphingomyelinase gene: evidence for a second metabolic detect in types A and B Niemann-Pick disease, J. Biol. Chem. 271, 18431-18436.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 18431-18436
-
-
Schissel, S.L.1
Schuchman, E.H.2
Williams, K.J.3
Tabas, I.4
-
25
-
-
0025819971
-
Human acid sphingomyelinase: Isolation, nucleotide sequence, and expression of the full-length and alternatively spliced cDNAs
-
Schuchman, E. H., Suchi, M., Takahashi, T., Sandhoff, K. & Desnick, R. J. (1991) Human acid sphingomyelinase: isolation, nucleotide sequence, and expression of the full-length and alternatively spliced cDNAs, J. Biol. Chem. 266, 8531-8539.
-
(1991)
J. Biol. Chem.
, vol.266
, pp. 8531-8539
-
-
Schuchman, E.H.1
Suchi, M.2
Takahashi, T.3
Sandhoff, K.4
Desnick, R.J.5
-
26
-
-
0001745899
-
Niemann-Pick disease types A and B: Acid sphingomyelinase deficiences
-
(Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D., eds) 7th edn, McGraw Hill, New York
-
Schuchman, E. H. & Desnick, R. J. (1995) Niemann-Pick disease types A and B: acid sphingomyelinase deficiences, in The metabolic and molecular basis of inherited disease (Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D., eds) vol. 2, 7th edn, pp. 2601-2624, McGraw Hill, New York.
-
(1995)
The Metabolic and Molecular Basis of Inherited Disease
, vol.2
, pp. 2601-2624
-
-
Schuchman, E.H.1
Desnick, R.J.2
-
27
-
-
0026458406
-
TNF activates NF-κB by phosphatidylcholine-specific phospholipase C-induced acidic sphingomyelin breakdown
-
Schütze, S., Potthoff, K., Machleidt, T., Berkovic, D., Wiegmann, K. & Krönke, M. (1992) TNF activates NF-κB by phosphatidylcholine-specific phospholipase C-induced acidic sphingomyelin breakdown, Cell 71, 765-776.
-
(1992)
Cell
, vol.71
, pp. 765-776
-
-
Schütze, S.1
Potthoff, K.2
Machleidt, T.3
Berkovic, D.4
Wiegmann, K.5
Krönke, M.6
-
28
-
-
0027288659
-
The role of glycosylation and phosphorylation in the expression of active human β-glucocerebrosidase
-
Shipley, J. M., Grubb, J. H. & Sly, W. S. (1993) The role of glycosylation and phosphorylation in the expression of active human β-glucocerebrosidase, J. Biol. Chem. 268, 12193-12198.
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 12193-12198
-
-
Shipley, J.M.1
Grubb, J.H.2
Sly, W.S.3
-
30
-
-
0022555844
-
Lysosomal enzymes and their receptors
-
von Figura, K. & Hasilik, A. (1986) Lysosomal enzymes and their receptors, Annu. Rev. Biochem. 55, 167-193.
-
(1986)
Annu. Rev. Biochem.
, vol.55
, pp. 167-193
-
-
Von Figura, K.1
Hasilik, A.2
-
31
-
-
0026767475
-
Analysis of the glycosylation and phosphorylation of the α-subunit of the lysosomal enzyme, β-hexosaminidase A, by site-directed mutagenesis
-
Weitz, G. & Proia, R. L. (1992) Analysis of the glycosylation and phosphorylation of the α-subunit of the lysosomal enzyme, β-hexosaminidase A, by site-directed mutagenesis, J. Biol. Chem. 267, 10039-10044.
-
(1992)
J. Biol. Chem.
, vol.267
, pp. 10039-10044
-
-
Weitz, G.1
Proia, R.L.2
-
32
-
-
0028108015
-
Functional dichotomy of neutral and acidic sphingomyelinases in tumor necrosis factor signaling
-
Wiegmann, K., Schütze, S., Machleidt, D., Witte, D. & Krönke, M. (1994) Functional dichotomy of neutral and acidic sphingomyelinases in tumor necrosis factor signaling, Cell 78, 1005-1015.
-
(1994)
Cell
, vol.78
, pp. 1005-1015
-
-
Wiegmann, K.1
Schütze, S.2
Machleidt, D.3
Witte, D.4
Krönke, M.5
-
33
-
-
0028097921
-
The structure and function of proline-rich regions in proteins
-
Williamson, M. P. (1994) The structure and function of proline-rich regions in proteins, Biochem. J. 297, 249-260.
-
(1994)
Biochem. J.
, vol.297
, pp. 249-260
-
-
Williamson, M.P.1
-
34
-
-
0028979815
-
Fluorescence based selection of retrovirally transduced genes in the absence of a marker gene: Direct selection of type B Niemann-Pick cells and evidence for bystander correction
-
Yeyati, P. L., Agmon, V., Fillat, C., Dinur, T., Dagan, A., Desnick, R. J., Gatt, S. & Schuchman, E. H. (1995) Fluorescence based selection of retrovirally transduced genes in the absence of a marker gene: direct selection of type B Niemann-Pick cells and evidence for bystander correction, Hum. Gene Ther. 6, 975-983.
-
(1995)
Hum. Gene Ther.
, vol.6
, pp. 975-983
-
-
Yeyati, P.L.1
Agmon, V.2
Fillat, C.3
Dinur, T.4
Dagan, A.5
Desnick, R.J.6
Gatt, S.7
Schuchman, E.H.8
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