-
1
-
-
0003014019
-
Ceramidase deficiency: Farber lipogranulomatosis
-
Scriver CR, Beaudet AL, Sly WS, Valle D, editors. New York: McGraw-Hill
-
Moser HW, Moser AB, Chen WW, Schram AW. Ceramidase deficiency: Farber lipogranulomatosis. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The Metabolic Basis of Inherited Disease, 6th ed. New York: McGraw-Hill: 1989;1645-1654.
-
(1989)
The Metabolic Basis of Inherited Disease, 6th Ed.
, pp. 1645-1654
-
-
Moser, H.W.1
Moser, A.B.2
Chen, W.W.3
Schram, A.W.4
-
2
-
-
0015498606
-
Ceramidase deficiency in Farber's disease (lipogranulomatosis)
-
Sugita M, Dulaney JT, Moser HW. Ceramidase deficiency in Farber's disease (lipogranulomatosis). Science 1972;178:1100-1102.
-
(1972)
Science
, vol.178
, pp. 1100-1102
-
-
Sugita, M.1
Dulaney, J.T.2
Moser, H.W.3
-
3
-
-
0017073339
-
Diagnosis of lipogranulomatosis (Farber disease) by use of cultured fibroblasts
-
Dulaney JT, Milunsky A, Sidbury JB, Hobolth N, Moser HW. Diagnosis of lipogranulomatosis (Farber disease) by use of cultured fibroblasts. J Pediatr 1976;89:59-61.
-
(1976)
J Pediatr
, vol.89
, pp. 59-61
-
-
Dulaney, J.T.1
Milunsky, A.2
Sidbury, J.B.3
Hobolth, N.4
Moser, H.W.5
-
4
-
-
0018644078
-
Prenatal diagnosis of Farber 's disease
-
Fensom AH, Benson PF, Neville BRG, Moser HW, Moser AE, Dulaney JT. Prenatal diagnosis of Farber 's disease. Lancet 1979;ii:990-992.
-
(1979)
Lancet
, vol.2
, pp. 990-992
-
-
Fensom, A.H.1
Benson, P.F.2
Neville, B.R.G.3
Moser, H.W.4
Moser, A.E.5
Dulaney, J.T.6
-
5
-
-
0000675223
-
Enzymic hydrolysis and synthesis of ceramides
-
Gatt S. Enzymic hydrolysis and synthesis of ceramides. J Biol Chem 1963;238:3131-3133.
-
(1963)
J Biol Chem
, vol.238
, pp. 3131-3133
-
-
Gatt, S.1
-
6
-
-
0014027942
-
Enzymatic hydrolysis of sphingolipids. I. Hydrolysis and synthesis of ceramides by an enzyme from rat brain
-
Gatt S. Enzymatic hydrolysis of sphingolipids. I. Hydrolysis and synthesis of ceramides by an enzyme from rat brain. J Biol Chem 1966;241:3724-3730.
-
(1966)
J Biol Chem
, vol.241
, pp. 3724-3730
-
-
Gatt, S.1
-
7
-
-
0014501038
-
Enzymatic hydrolysis of sphingolipids. VII. Further purification and properties of rat brain ceramidase
-
Yavin E, Gatt S. Enzymatic hydrolysis of sphingolipids. VII. Further purification and properties of rat brain ceramidase. Biochemistry 1969;8:1692-1698.
-
(1969)
Biochemistry
, vol.8
, pp. 1692-1698
-
-
Yavin, E.1
Gatt, S.2
-
8
-
-
0014663246
-
The presence of sphingomyelin and ceramide cleaving enzymes in the small intestinal tract
-
Nilsson A. The presence of sphingomyelin and ceramide cleaving enzymes in the small intestinal tract. Biochim Biophys Acta 1969;176:339-347.
-
(1969)
Biochim Biophys Acta
, vol.176
, pp. 339-347
-
-
Nilsson, A.1
-
9
-
-
0016682741
-
Ceramidase and ceramide synthesis in human kidney and cerebellum: Description of a new alkaline ceramidase
-
Sugita M, Williams M, Dulaney JT, Moser HW. Ceramidase and ceramide synthesis in human kidney and cerebellum: description of a new alkaline ceramidase. Biochim Biophys Acta 1975;398:125-131.
-
(1975)
Biochim Biophys Acta
, vol.398
, pp. 125-131
-
-
Sugita, M.1
Williams, M.2
Dulaney, J.T.3
Moser, H.W.4
-
10
-
-
0018965399
-
Studies in vitro on the biosynthesis of ceramide and sphingomyelin. A reevaluation of proposed pathways
-
Stoffel W, Melzner I. Studies in vitro on the biosynthesis of ceramide and sphingomyelin. A reevaluation of proposed pathways. Hoppe Seyler's Z Physiol Chem 1980;361:755-771.
-
(1980)
Hoppe Seyler's Z Physiol Chem
, vol.361
, pp. 755-771
-
-
Stoffel, W.1
Melzner, I.2
-
11
-
-
0020378285
-
Substrate specificities of acid and alkaline ceramidase in fibroblasts from patients with Farber disease and controls
-
Momoi T, Ben-Yoseph Y, Nadler HL. Substrate specificities of acid and alkaline ceramidase in fibroblasts from patients with Farber disease and controls. Biochem J 1982;205:419-425.
-
(1982)
Biochem J
, vol.205
, pp. 419-425
-
-
Momoi, T.1
Ben-Yoseph, Y.2
Nadler, H.L.3
-
13
-
-
0024365716
-
Properties of acid ceramidase from human spleen
-
Al BJM, Tiffany CW, Gomes de Mesquita DS, Moser HW, Tager JM, Schram AW. Properties of acid ceramidase from human spleen. Biochim Biophys Acta 1989;1004:245-251.
-
(1989)
Biochim Biophys Acta
, vol.1004
, pp. 245-251
-
-
Al, B.J.M.1
Tiffany, C.W.2
Gomes De Mesquita, D.S.3
Moser, H.W.4
Tager, J.M.5
Schram, A.W.6
-
14
-
-
0019797740
-
Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblasts
-
Chen WW, Moser AB, Moser HW. Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblasts. Arch Biochem Biophys 1981;208:444-455.
-
(1981)
Arch Biochem Biophys
, vol.208
, pp. 444-455
-
-
Chen, W.W.1
Moser, A.B.2
Moser, H.W.3
-
15
-
-
0020071206
-
Metabolism of ceramide containing endocytotic vesicles in human diploid fibroblasts
-
Sutrina SL, Chen WW. Metabolism of ceramide containing endocytotic vesicles in human diploid fibroblasts. J Biol Chem 1982;257:3039-3044.
-
(1982)
J Biol Chem
, vol.257
, pp. 3039-3044
-
-
Sutrina, S.L.1
Chen, W.W.2
-
16
-
-
0019980947
-
Diagnosis of metachromatic leukodystrophy, Krabbe disease and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts
-
Kudoh T, Wenger DA. Diagnosis of metachromatic leukodystrophy, Krabbe disease and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts. J Clin Invest 1982;70:89-97.
-
(1982)
J Clin Invest
, vol.70
, pp. 89-97
-
-
Kudoh, T.1
Wenger, D.A.2
-
17
-
-
0020605030
-
Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage disease
-
Kudoh T, Velkoff MA, Wenger DA. Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage disease. Biochim Biophys Acta 1983;754:82-92.
-
(1983)
Biochim Biophys Acta
, vol.754
, pp. 82-92
-
-
Kudoh, T.1
Velkoff, M.A.2
Wenger, D.A.3
-
18
-
-
0023496330
-
Metabolism of cerebroside sulphate and subcellular distribution of its metabolites in cultured skin fibroblasts derived from controls, metachromatic leukodystrophy, globoid cell leukodystrophy and Farber disease
-
Inui K, Furukawa M, Nishimoto J, Okada S, Yabuuchi H. Metabolism of cerebroside sulphate and subcellular distribution of its metabolites in cultured skin fibroblasts derived from controls, metachromatic leukodystrophy, globoid cell leukodystrophy and Farber disease. J Inher Metab Dis 1987; 10:293-296.
-
(1987)
J Inher Metab Dis
, vol.10
, pp. 293-296
-
-
Inui, K.1
Furukawa, M.2
Nishimoto, J.3
Okada, S.4
Yabuuchi, H.5
-
19
-
-
0024598464
-
A case of combined Farber and Sandhoff disease
-
Fusch C, Huenges R, Moser HW, Sewell AC, Roggendorf W, Kustermann-Kuhn B, Poulos A, Carey WF, Harzer K. A case of combined Farber and Sandhoff disease. Eur J Pediatr 1989;148;558-562.
-
(1989)
Eur J Pediatr
, vol.148
, pp. 558-562
-
-
Fusch, C.1
Huenges, R.2
Moser, H.W.3
Sewell, A.C.4
Roggendorf, W.5
Kustermann-Kuhn, B.6
Poulos, A.7
Carey, W.F.8
Harzer, K.9
-
20
-
-
0027296023
-
The in situ degradation of ceramide, a potential lipid mediator, is not completely impaired in Farber disease
-
Levade T, Tempesta MC, Salvayre R. The in situ degradation of ceramide, a potential lipid mediator, is not completely impaired in Farber disease. FEBS Lett 1993;329:306-312.
-
(1993)
FEBS Lett
, vol.329
, pp. 306-312
-
-
Levade, T.1
Tempesta, M.C.2
Salvayre, R.3
-
21
-
-
0029074311
-
Sulfatide and sphingomyelin loading of living cells as tools for the study of ceramide turnover by lysosomal ceramidase. Implications for the diagnosis of Farber disease
-
Levade T, Tempesta M, Moser HW, Fensom AH, Harzer K, Moser AB, Salvayre R. Sulfatide and sphingomyelin loading of living cells as tools for the study of ceramide turnover by lysosomal ceramidase. Implications for the diagnosis of Farber disease. Biochem Mol Med 1995;54:117-125.
-
(1995)
Biochem Mol Med
, vol.54
, pp. 117-125
-
-
Levade, T.1
Tempesta, M.2
Moser, H.W.3
Fensom, A.H.4
Harzer, K.5
Moser, A.B.6
Salvayre, R.7
-
22
-
-
0343618789
-
Farber's disease: Ceramide accumulation in cultured fibroblasts
-
Philippart M, Nakatani S, Zeilstra K, Tondeur M, Vamos-Hurwitz E, Pelc S. Farber's disease: ceramide accumulation in cultured fibroblasts [abstract]. Trans Am Soc Neurochem 1975;6:152.
-
(1975)
Trans Am Soc Neurochem
, vol.6
, pp. 152
-
-
Philippart, M.1
Nakatani, S.2
Zeilstra, K.3
Tondeur, M.4
Vamos-Hurwitz, E.5
Pelc, S.6
-
24
-
-
0026733541
-
Changes in bioactive lipids, alkylacylglycerol and ceramide, occur in HIV infected cells
-
Van Veldhoven P, Matthews TJ, Bolognesi DP, Bell RM. Changes in bioactive lipids, alkylacylglycerol and ceramide, occur in HIV infected cells. Biochem Biophys Res Commun 1992;187:209-216.
-
(1992)
Biochem Biophys Res Commun
, vol.187
, pp. 209-216
-
-
Van Veldhoven, P.1
Matthews, T.J.2
Bolognesi, D.P.3
Bell, R.M.4
-
25
-
-
0025370010
-
Transfer of immortality by transfection of genomic DNA from SV40 established cell lines into rat embryo fibroblasts
-
Jacquemin-Sablon H, Ganz L, Feunteun J. Transfer of immortality by transfection of genomic DNA from SV40 established cell lines into rat embryo fibroblasts. Biol Cell 1990;68:227-230.
-
(1990)
Biol Cell
, vol.68
, pp. 227-230
-
-
Jacquemin-Sablon, H.1
Ganz, L.2
Feunteun, J.3
-
26
-
-
0027932927
-
Accurate differentiation of neuronopathic and non-neuronopathic forms of Niemann-Pick disease by evaluation of the effective residual lysosomal sphingomyelinase activity in intact cells
-
Graber D, Salvayre R, Levade T. Accurate differentiation of neuronopathic and non-neuronopathic forms of Niemann-Pick disease by evaluation of the effective residual lysosomal sphingomyelinase activity in intact cells. J Neurochem 1994;63:1060-1068.
-
(1994)
J Neurochem
, vol.63
, pp. 1060-1068
-
-
Graber, D.1
Salvayre, R.2
Levade, T.3
-
27
-
-
0022186670
-
Measurement of protein using bicin-choninic acid
-
Smith P, Krohn RI, Hermanson GT, Mallia AK, Gartner FH, Provenzano MD, Fujimoto EK, Goeke NM, Olson BJ, Klenk DC. Measurement of protein using bicin-choninic acid. Anal Biochem 1985;150:76-85.
-
(1985)
Anal Biochem
, vol.150
, pp. 76-85
-
-
Smith, P.1
Krohn, R.I.2
Hermanson, G.T.3
Mallia, A.K.4
Gartner, F.H.5
Provenzano, M.D.6
Fujimoto, E.K.7
Goeke, N.M.8
Olson, B.J.9
Klenk, D.C.10
-
28
-
-
70449158340
-
A simple method for the isolation and purification of total lipids from animal tissues
-
Folch J, Lees M, Sloane Stanley GH. A simple method for the isolation and purification of total lipids from animal tissues. J Biol Chem 1957;275:497-509.
-
(1957)
J Biol Chem
, vol.275
, pp. 497-509
-
-
Folch, J.1
Lees, M.2
Sloane Stanley, G.H.3
-
29
-
-
0015472950
-
Identification and quantification of free ceramides in human ptelets
-
Krivit W, Hammarström S. Identification and quantification of free ceramides in human ptelets. J Lipid Res 1972;13:525-530.
-
(1972)
J Lipid Res
, vol.13
, pp. 525-530
-
-
Krivit, W.1
Hammarström, S.2
-
30
-
-
0028292774
-
Dissociation of endogenous cellular ceramide from NF-xB activation
-
Betts JC, Agranoff AB, Nabel GJ, Shayman JA. Dissociation of endogenous cellular ceramide from NF-xB activation. J Biol Chem 1994;269:8455-8458.
-
(1994)
J Biol Chem
, vol.269
, pp. 8455-8458
-
-
Betts, J.C.1
Agranoff, A.B.2
Nabel, G.J.3
Shayman, J.A.4
-
31
-
-
0028287960
-
Ionizing radiation acts on cellular membranes to generate ceramide and initiate apoptosis
-
Haimovitz-Friedman A, Klan CC, Ehlerter D, Persaud RS, Mc Loughlin M, Fuks Z, Kolesnick RN. Ionizing radiation acts on cellular membranes to generate ceramide and initiate apoptosis. J Exp Med 1994;80:525-535.
-
(1994)
J Exp Med
, vol.80
, pp. 525-535
-
-
Haimovitz-Friedman, A.1
Klan, C.C.2
Ehlerter, D.3
Persaud, R.S.4
Mc Loughlin, M.5
Fuks, Z.6
Kolesnick, R.N.7
-
32
-
-
0016065208
-
High performance liquid chromatography of ceramides: Application to analysis in human tissues and demonstration of ceramide excess in Farber's disease
-
Sugita M, Iwamori M, Evans J, Cluer RHM, Dulaney JT, Moser HW. High performance liquid chromatography of ceramides: application to analysis in human tissues and demonstration of ceramide excess in Farber's disease. J Lipid Res 1974;15:223-226.
-
(1974)
J Lipid Res
, vol.15
, pp. 223-226
-
-
Sugita, M.1
Iwamori, M.2
Evans, J.3
Cluer, R.H.M.4
Dulaney, J.T.5
Moser, H.W.6
-
33
-
-
0018341515
-
Analysis and quantification of free ceramide containing non-hydroxy and 2-hydroxy fatty acids, and phytosphingosine by high-performance liquid chromatography
-
Iwamori M, Costello C, Moser HW. Analysis and quantification of free ceramide containing non-hydroxy and 2-hydroxy fatty acids, and phytosphingosine by high-performance liquid chromatography. J Lipid Res 1979;20:86-96.
-
(1979)
J Lipid Res
, vol.20
, pp. 86-96
-
-
Iwamori, M.1
Costello, C.2
Moser, H.W.3
-
34
-
-
0343183393
-
Farber's disease (lipogranulomatosis)
-
Glew RH, Peters SP, editors. New York: AR Liss
-
Dulaney JT, Moser HW. Farber's disease (lipogranulomatosis). In: Glew RH, Peters SP, editors. Practical Enzymology of the Sphingolipidoses. New York: AR Liss 1977;283-296.
-
(1977)
Practical Enzymology of the Sphingolipidoses
, pp. 283-296
-
-
Dulaney, J.T.1
Moser, H.W.2
-
35
-
-
0024420051
-
Sphingolipid activator protein deficiency in a 16-week-old atypical Gaucher disease patient and his fetal sibling: Biochemical signs of combined sphingolipidoses
-
Harzer K, Paton BC, Poulos A, Kustermann-Kuhn B, Roggendorf W, Grisar T, Popp M. Sphingolipid activator protein deficiency in a 16-week-old atypical Gaucher disease patient and his fetal sibling: biochemical signs of combined sphingolipidoses. Eur J Pediatr 1989;149:31-39.
-
(1989)
Eur J Pediatr
, vol.149
, pp. 31-39
-
-
Harzer, K.1
Paton, B.C.2
Poulos, A.3
Kustermann-Kuhn, B.4
Roggendorf, W.5
Grisar, T.6
Popp, M.7
-
36
-
-
0029133901
-
A family with a combined Farber and Sandhoff, isolated Sandhoff and isolated fetal Farber disease: Postnatal exclusion and prenatal diagnosis of Farber disease using lipid loading tests on intact cultured cells
-
Levade T, Enders H, Schliephacke M, Harzer K. A family with a combined Farber and Sandhoff, isolated Sandhoff and isolated fetal Farber disease: postnatal exclusion and prenatal diagnosis of Farber disease using lipid loading tests on intact cultured cells. Eur J Pediatr 1995;154:643-648.
-
(1995)
Eur J Pediatr
, vol.154
, pp. 643-648
-
-
Levade, T.1
Enders, H.2
Schliephacke, M.3
Harzer, K.4
-
37
-
-
0028032001
-
Functional compartments of sulphatide metabolism in cultured living cells: Evidence for the involvement of a novel sulphatide-degrading pathway
-
Tempesta MC, Salvayre R, Levade T. Functional compartments of sulphatide metabolism in cultured living cells: evidence for the involvement of a novel sulphatide-degrading pathway. Biochem J 1994;297:479-489.
-
(1994)
Biochem J
, vol.297
, pp. 479-489
-
-
Tempesta, M.C.1
Salvayre, R.2
Levade, T.3
-
38
-
-
0017384224
-
The cellular lesions of Farber disease and their experimental reproduction in tissue culture
-
Rutsaert J, Tondeur M, Vamos-Hurwitz E, Dustin P. The cellular lesions of Farber disease and their experimental reproduction in tissue culture. Lab Invest 1977;36:474-480.
-
(1977)
Lab Invest
, vol.36
, pp. 474-480
-
-
Rutsaert, J.1
Tondeur, M.2
Vamos-Hurwitz, E.3
Dustin, P.4
-
39
-
-
0022416525
-
Establishment of human fibroblast cell lines with lysosomal enzyme deficiency by transformation with origin-minus SV40 DNA
-
Furuya T, Momoi T, Suzuki Y. Establishment of human fibroblast cell lines with lysosomal enzyme deficiency by transformation with origin-minus SV40 DNA. J Inher Metab Dis 1985;8:143-144.
-
(1985)
J Inher Metab Dis
, vol.8
, pp. 143-144
-
-
Furuya, T.1
Momoi, T.2
Suzuki, Y.3
|