-
1
-
-
0021243689
-
Enzyme-linked immunosorbent assay for the ganglioside GM2-activator protein screening of normal human tissues and bodyfluids, of tissues of GM2-gangliosidosis, and for its subcellular localization
-
M2-gangliosidosis, and for its subcellular localization Hoppe-Seyler's Z. Physiol. Chem. 365 1984 347 356
-
(1984)
Hoppe-Seyler's Z. Physiol. Chem.
, vol.365
, pp. 347-356
-
-
Banerjee, A.1
Burg, J.2
Conzelmann, E.3
Carroll, M.4
Sandhoff, K.5
-
2
-
-
0016588265
-
Subunit structure of human hexosaminidase verified: Interconvertibility of hexosaminidase isoenzymes
-
E. Beutler J. Kuhl Subunit structure of human hexosaminidase verified: Interconvertibility of hexosaminidase isoenzymes Nature (Lond.) 258 1975 262 264
-
(1975)
Nature (Lond.)
, vol.258
, pp. 262-264
-
-
Beutler, E.1
Kuhl, J.2
-
3
-
-
0015698806
-
Late onset GM2 gangliosidosis: Clinical, pathological, and biochemical studies on eight patients
-
M2 gangliosidosis: Clinical, pathological, and biochemical studies on eight patients Arch. Dis. Child. 48 1973 775 785
-
(1973)
Arch. Dis. Child.
, vol.48
, pp. 775-785
-
-
Brett, E.M.1
Ellis, R.B.2
Haas, L.3
Ikonne, J.U.4
Lake, B.D.5
Patrick, A.D.6
Stephens, R.7
-
5
-
-
0022272863
-
Molecular forms of GM2-activator protein—A study on its biosynthesis in human skin fibroblasts
-
M2-activator protein—A study on its biosynthesis in human skin fibroblasts Biol. Chem. Hoppe-Seyler. 366 1985 887 891
-
(1985)
Biol. Chem. Hoppe-Seyler.
, vol.366
, pp. 887-891
-
-
Burg, J.1
Banerjee, A.2
Sandhoff, K.3
-
7
-
-
0014229619
-
Incorporation of selected isotopes into lipids of humans with cerebral lipidoses: Studies on D-glucosamine-1–14C
-
R. Burton S. Handa R.E. Howard T. Vietti Incorporation of selected isotopes into lipids of humans with cerebral lipidoses: Studies on D -glucosamine-1–14C Pathol. Eur. 3 1968 424 430
-
(1968)
Pathol. Eur.
, vol.3
, pp. 424-430
-
-
Burton, R.1
Handa, S.2
Howard, R.E.3
Vietti, T.4
-
8
-
-
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
-
A2 Proc. Natl. Acad. Sci. (USA) 75 1978 3979 3983
-
(1978)
, pp. 3979-3983
-
-
Conzelmann, E.1
Sandhoff, K.2
-
9
-
-
0018567163
-
Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A
-
A2 by hexosaminidase A Hoppe-Seyler's Z. Physiol. Chem. 360 1979 1837 1849
-
(1979)
Hoppe-Seyler's Z. Physiol. Chem.
, vol.360
, pp. 1837-1849
-
-
Conzelmann, E.1
Sandhoff, K.2
-
10
-
-
0021085107
-
Partial enzyme deficiencies: Residual activities and the development of neurological disorders
-
E. Conzelmann K. Sandhoff Partial enzyme deficiencies: Residual activities and the development of neurological disorders Dev. Neurosci. 6 1983/1984 58 71
-
(1984)
Dev. Neurosci.
, vol.6
, pp. 58-71
-
-
Conzelmann, E.1
Sandhoff, K.2
-
13
-
-
0020642981
-
Ganglioside GM2 N-acetyl-β-galactosaminidase activity in cultured fibroblasts of late infantile and adult GM2-gangliosidosis patients and of healthy probands with low hexosaminidase level
-
M2-gangliosidosis patients and of healthy probands with low hexosaminidase level Am. J. Hum. Genet. 35 1983 900 913
-
(1983)
Am. J. Hum. Genet.
, vol.35
, pp. 900-913
-
-
Conzelmann, E.1
Kytzia, H.-J.2
Navon, R.3
Sandhoff, K.4
-
14
-
-
0022269742
-
Prenatal diagnosis of GM2-gangliosidosis with high residual hexosaminidase A activity (variant B 1; pseudo AB variant)
-
M2-gangliosidosis with high residual hexosaminidase A activity (variant B 1; pseudo AB variant) Pediatr. Res. 19 1985 1220 1224
-
(1985)
Pediatr. Res.
, vol.19
, pp. 1220-1224
-
-
Conzelmann, E.1
Nehrkorn, H.2
Kytzia, H.-J.3
Sandhoff, K.4
Macek, M.5
Lehovsky, M.6
Elleder, M.7
Jirasek, A.8
Kobilkova, J.9
-
15
-
-
0016857209
-
GM2 gangliosidosis, AB variant. Clinico-pathological study of a case
-
M2 gangliosidosis, AB variant. Clinico-pathological study of a case Acta Neuropathol. 33 1975 207 226
-
(1975)
Acta Neuropathol.
, vol.33
, pp. 207-226
-
-
de Baeque, C.M.1
Suzuki, K.2
Rapin, I.3
Johnson, A.B.4
Whethers, D.L.5
Suzuki, K.6
-
16
-
-
34250575611
-
Uber die Prozessausbreitung der amaurotischen Idiotie im Zentralnervensystem in verschiedenen Lebensaltern und Besonderheiten der Spätform gegenüber der Pigmentatrophie
-
Escola Uber die Prozessausbreitung der amaurotischen Idiotie im Zentralnervensystem in verschiedenen Lebensaltern und Besonderheiten der Spätform gegenüber der Pigmentatrophie Arch. Psychiat. Nervenkr. 202 1961 95 112
-
(1961)
Arch. Psychiat. Nervenkr.
, vol.202
, pp. 95-112
-
-
Escola1
-
17
-
-
0014127346
-
Isolation of gb-N-acetylglucosaminidase and gb-N-acetylgalactosaminidase from calf brain
-
Y.Z. Frohwein S. Gatt Isolation of gb- N -acetylglucosaminidase and gb- N -acetylgalactosaminidase from calf brain Biochemistry 6 1967 2775 2783
-
(1967)
Biochemistry
, vol.6
, pp. 2775-2783
-
-
Frohwein, Y.Z.1
Gatt, S.2
-
18
-
-
0016260137
-
Tay-Sachs and Sandhoff's disease, intergenic complementation after somatic cell hybridization
-
H. Galjaard A. Hoogeven H.A. de Wit-Verbeek A.J.J. Reuser W. Keijzer A. Westerveldt D. Bootsma Tay-Sachs and Sandhoff's disease, intergenic complementation after somatic cell hybridization Exp. Cell Res. 87 1974 444 448
-
(1974)
Exp. Cell Res.
, vol.87
, pp. 444-448
-
-
Galjaard, H.1
Hoogeven, A.2
de Wit-Verbeek, H.A.3
Reuser, A.J.J.4
Keijzer, W.5
Westerveldt, A.6
Bootsma, D.7
-
19
-
-
0017153536
-
Chemical characterization and subunit structure of human N-acetylhexosaminidases A and B
-
B. Geiger R. Arnon Chemical characterization and subunit structure of human N-acetylhexosaminidases A and B Biochemistry 15 1976 3484 3493
-
(1976)
Biochemistry
, vol.15
, pp. 3484-3493
-
-
Geiger, B.1
Arnon, R.2
-
20
-
-
0019268468
-
The AB-Variant of GM2-gangliosidosis
-
M2-gangliosidosis Acta Neuropathol. (Berlin) Clinical, biochemical and pathological studies of two patients 52 1980 189 202
-
(1980)
Acta Neuropathol. (Berlin) Clinical, biochemical and pathological studies of two patients
, vol.52
, pp. 189-202
-
-
Goldmann, J.E.1
Yamanaka, T.2
Rapin, I.3
Adachi, M.4
Suzuki, K.5
Suzuki, K.6
-
21
-
-
85120140823
-
Enzymatische Untersuchungen im Blut von Überträgern einer Variante der Tay-Sachs'schen Erkrankung (Variante 0)
-
K. Harzer K. Sandhoff H. Schall F. Kollmann Enzymatische Untersuchungen im Blut von Überträgern einer Variante der Tay-Sachs'schen Erkrankung (Variante 0) Klin. Wschr. 49 1971 1187 1191
-
(1971)
Klin. Wschr.
, vol.49
, pp. 1187-1191
-
-
Harzer, K.1
Sandhoff, K.2
Schall, H.3
Kollmann, F.4
-
23
-
-
0000875001
-
Histochemical demonstration of N-acetyl-β-glucosaminidase employing naphtol ASBI N-acetyl-β-glucosaminide as substrate
-
M. Hayashi Histochemical demonstration of N-acetyl-β-glucosaminidase employing naphtol ASBI N-acetyl-β-glucosaminide as substrate J. Histochem. Cytochem. 13 1965 355 360
-
(1965)
J. Histochem. Cytochem.
, vol.13
, pp. 355-360
-
-
Hayashi, M.1
-
24
-
-
0020662323
-
The protein activator specific for the enzymic hydrolysis of GM2 ganglioside in normal human brain and in brains of three types of GM2 gangliosidosis
-
M2 ganglioside in normal human brain and in brains of three types of GM2 gangliosidosis J. Neurochem. 40 1983 168 175
-
(1983)
J. Neurochem.
, vol.40
, pp. 168-175
-
-
Hirabayashi, Y.1
Li, Y.-T.2
Li, S.-C.3
-
25
-
-
0014695039
-
N-Acetylhexosaminidase activities in Tay-Sachs disease
-
B. Hultberg N-Acetylhexosaminidase activities in Tay-Sachs disease Lancet II 1969 1195
-
(1969)
Lancet
, vol.II
, pp. 1195
-
-
Hultberg, B.1
-
26
-
-
0021339782
-
Progressive accumulation of toxic metabolite in a genetic leukodystrophy
-
H. Igisu K. Suzuki Progressive accumulation of toxic metabolite in a genetic leukodystrophy Science 224 1984 753 755
-
(1984)
Science
, vol.224
, pp. 753-755
-
-
Igisu, H.1
Suzuki, K.2
-
27
-
-
50549200398
-
On a biochemically special form of infantile amaurotic idiocy
-
H. Jatzkewitz K. Sandhoff On a biochemically special form of infantile amaurotic idiocy Biochim. Biophys. Acta 70 1963 354 356
-
(1963)
Biochim. Biophys. Acta
, vol.70
, pp. 354-356
-
-
Jatzkewitz, H.1
Sandhoff, K.2
-
28
-
-
84984084613
-
Quantitative Bestimmungen von Gangliosiden und ihren neuraminsäurefreien Derivaten bei infantilen, juvenilen und adulten Formen der amaurotischen Idiotie und einer spätinfantilen biochemischen Sonderform
-
H. Jatzkewitz H. Pilz K. Sandhoff Quantitative Bestimmungen von Gangliosiden und ihren neuraminsäurefreien Derivaten bei infantilen, juvenilen und adulten Formen der amaurotischen Idiotie und einer spätinfantilen biochemischen Sonderform J. Neurochem. 12 1965 135 144
-
(1965)
J. Neurochem.
, vol.12
, pp. 135-144
-
-
Jatzkewitz, H.1
Pilz, H.2
Sandhoff, K.3
-
29
-
-
84871390834
-
Die Fettstoffe des Gehirns bei amaurotischer Idiotie und Niemann-Pick'scher Krankheit
-
E. Klenk Die Fettstoffe des Gehirns bei amaurotischer Idiotie und Niemann-Pick'scher Krankheit Ber. Ges. Physiol. 96 1937 659 660
-
(1937)
Ber. Ges. Physiol.
, vol.96
, pp. 659-660
-
-
Klenk, E.1
-
31
-
-
34548766435
-
Beitrage zur Chemie der Lipoidosen
-
E. Klenk Beitrage zur Chemie der Lipoidosen Hoppe-Seyler's Z. Physiol. Chem. 267 1940 128 144
-
(1940)
Hoppe-Seyler's Z. Physiol. Chem.
, vol.267
, pp. 128-144
-
-
Klenk, E.1
-
32
-
-
84941507430
-
Über die Ganglioside, eine neue Gruppe von zuckerhaltigen Gehirnlipoiden
-
E. Klenk Über die Ganglioside, eine neue Gruppe von zuckerhaltigen Gehirnlipoiden Hoppe Seyler's Z. Physiol. Chem. 273 1942 76 86
-
(1942)
Hoppe Seyler's Z. Physiol. Chem.
, vol.273
, pp. 76-86
-
-
Klenk, E.1
-
33
-
-
85171972958
-
Über die Ganglioside des Gehirns bei der infantilen amaurotischen Idiotie vom Typ Tay-Sachs
-
E. Klenk Über die Ganglioside des Gehirns bei der infantilen amaurotischen Idiotie vom Typ Tay-Sachs Ber. Dtsch. Chem. Ges. 75 1942 1632 1636
-
(1942)
Ber. Dtsch. Chem. Ges.
, vol.75
, pp. 1632-1636
-
-
Klenk, E.1
-
35
-
-
0014687950
-
Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease
-
E.H. Kolodny R.O. Brady B.W. Yolk Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease Biochem. Biophys. Res. Commun. 37 1969 526 531
-
(1969)
Biochem. Biophys. Res. Commun.
, vol.37
, pp. 526-531
-
-
Kolodny, E.H.1
Brady, R.O.2
Yolk, B.W.3
-
36
-
-
84939082957
-
A gangliosidase system
-
S.R. Korey A. Stein A gangliosidase system J. Folch-Pi H. Bauer Brain Lipids and Lipoproteins and the Leucodystrophies, VII. International Congress of Neurology Rome, 1961 1963 Elsevier Amsterdam 71 82
-
(1963)
, pp. 71-82
-
-
Korey, S.R.1
Stein, A.2
-
37
-
-
0019772309
-
Liberation of N-acetylglucosamine-6-sulfate by human β-N-acetylhexosaminidase A
-
H. Kresse W. Fuchs J. Glossel D. Holtfrerich W. Gilberg Liberation of N-acetylglucosamine-6-sulfate by human β-N-acetylhexosaminidase A J. Biol. Chem. 256 1981 12926 12932
-
(1981)
J. Biol. Chem.
, vol.256
, pp. 12926-12932
-
-
Kresse, H.1
Fuchs, W.2
Glossel, J.3
Holtfrerich, D.4
Gilberg, W.5
-
38
-
-
0039905229
-
Die Konstitution der Ganglio-N-tetraose und des Gangliosides GI
-
E. Kuhn H. Wiegandt Die Konstitution der Ganglio-N-tetraose und des Gangliosides GI Chem. Ber. 96 1963 866 880
-
(1963)
Chem. Ber.
, vol.96
, pp. 866-880
-
-
Kuhn, E.1
Wiegandt, H.2
-
39
-
-
0021885280
-
Evidence for two different active sites on human hexosaminidase—Interaction of GM2 activator protein with hexosaminidase A
-
M2 activator protein with hexosaminidase A J. Biol. Chem. 260 1985 7568 7572
-
(1985)
J. Biol. Chem.
, vol.260
, pp. 7568-7572
-
-
Kytzia, H.-J.1
Sandhoff, K.2
-
41
-
-
0021233226
-
Diagnosis of infantile and juvenile forms of GM2 gangliosidosis variant 0—Residual activities toward natural and different synthetic substrates
-
M2 gangliosidosis variant 0—Residual activities toward natural and different synthetic substrates Hum. Genet. 67 1984 414 418
-
(1984)
Hum. Genet.
, vol.67
, pp. 414-418
-
-
Kytzia, H.-J.1
Hinrichs, U.2
Sandhoff, K.3
-
42
-
-
0010355316
-
Structure of the Tay-Sachs' ganglioside
-
R. Ledeen K. Salsman Structure of the Tay-Sachs' ganglioside Biochemistry 4 1965 2225 2233
-
(1965)
Biochemistry
, vol.4
, pp. 2225-2233
-
-
Ledeen, R.1
Salsman, K.2
-
43
-
-
0026572112
-
Quantitative correlation between the residual activity of β-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease
-
P. Leinekugel S. Michel E. Conzelmann K. Sandhoff Quantitative correlation between the residual activity of β-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease Hum. Genet. 88 1992 513 523
-
(1992)
Hum. Genet.
, vol.88
, pp. 513-523
-
-
Leinekugel, P.1
Michel, S.2
Conzelmann, E.3
Sandhoff, K.4
-
44
-
-
0017254436
-
An activator stimulating the enzymic hydrolysis of sphingoglycolipids
-
S.-C. Li Y.-T. Li An activator stimulating the enzymic hydrolysis of sphingoglycolipids J. Biol. Chem. 254 1976 1159 1163
-
(1976)
J. Biol. Chem.
, vol.254
, pp. 1159-1163
-
-
Li, S.-C.1
Li, Y.-T.2
-
47
-
-
0010394808
-
The glycolipids of the brain of Tay-Sachs disease. The chemical structure of globoside and main ganglioside
-
A. Makita T. Yamakawa The glycolipids of the brain of Tay-Sachs disease. The chemical structure of globoside and main ganglioside Jon. J. Exp. Med. 33 1963 361 368
-
(1963)
Jon. J. Exp. Med.
, vol.33
, pp. 361-368
-
-
Makita, A.1
Yamakawa, T.2
-
49
-
-
0015496948
-
Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase
-
T. Miyatake K. Suzuki Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase Biochem. Biophys. Res. Commun. 48 1972 538 543
-
(1972)
Biochem. Biophys. Res. Commun.
, vol.48
, pp. 538-543
-
-
Miyatake, T.1
Suzuki, K.2
-
52
-
-
0020403317
-
The occurence of psychosine and other glycolipids in spleen and liver from the three major types of Gaucher's disease
-
O. Nilsson J.-E. Mansson G. Hakansson L. Svennerholm The occurence of psychosine and other glycolipids in spleen and liver from the three major types of Gaucher's disease Biochim. Biophys. Acta 712 1982 453 463
-
(1982)
Biochim. Biophys. Acta
, vol.712
, pp. 453-463
-
-
Nilsson, O.1
Mansson, J.-E.2
Hakansson, G.3
Svennerholm, L.4
-
53
-
-
0014678521
-
Tay-Sachs disease: Generalized absence of a β-d-N- acetyl-hexosaminidase component
-
S. Okada J.S. O'Brien Tay-Sachs disease: Generalized absence of a β- d -N- acetyl-hexosaminidase component Science 165 1969 698 700
-
(1969)
Science
, vol.165
, pp. 698-700
-
-
Okada, S.1
O'Brien, J.S.2
-
55
-
-
0017114511
-
Distortion of neuronal geometry and formation of aberrant synapses in neuronal storage disease
-
D.P. Purpura K. Suzuki Distortion of neuronal geometry and formation of aberrant synapses in neuronal storage disease Brain Res. 116 1976 1 21
-
(1976)
Brain Res.
, vol.116
, pp. 1-21
-
-
Purpura, D.P.1
Suzuki, K.2
-
56
-
-
85120107517
-
N-acetyl-glucosaminidases in human spleen
-
D. Robinson J.L. Stirling N-acetyl-glucosaminidases in human spleen Biochem. J. 101 1966 18
-
(1966)
Biochem. J.
, vol.101
, pp. 18
-
-
Robinson, D.1
Stirling, J.L.2
-
57
-
-
0014274419
-
N-Acetyl-β-d-glucosaminidases in human spleen
-
D. Robinson J.L. Stirling N-Acetyl-β- d -glucosaminidases in human spleen Biochem. J. 107 1968 321 327
-
(1968)
Biochem. J.
, vol.107
, pp. 321-327
-
-
Robinson, D.1
Stirling, J.L.2
-
58
-
-
0014056462
-
Separation and properties of β-galactosidase, β-glucosidase, β-glucuronidase and N-acetyl-β-glucosaminidase from rat kidney
-
D. Robinson R.G. Price N. Dance Separation and properties of β-galactosidase, β-glucosidase, β-glucuronidase and N-acetyl-β-glucosaminidase from rat kidney Biochem. J. 102 1967 525 532
-
(1967)
Biochem. J.
, vol.102
, pp. 525-532
-
-
Robinson, D.1
Price, R.G.2
Dance, N.3
-
59
-
-
0023179714
-
Composition of gangliosides and neutral glycosphingolipids of brain in classical Tay-Sachs and Sandhoff disease: More lyso-GM2 in Sandhoff disease?
-
M2 in Sandhoff disease? J. Neurochem. 49 1987 834 840
-
(1987)
J. Neurochem.
, vol.49
, pp. 834-840
-
-
Rosengren, B.1
Mansson, J.-E.2
Svennerholm, L.3
-
60
-
-
0001291486
-
On arrested cerebral development with special reference to its cortical pathology
-
B. Sachs On arrested cerebral development with special reference to its cortical pathology J. Nerv. Ment. Dis. 14 1887 541 553
-
(1887)
J. Nerv. Ment. Dis.
, vol.14
, pp. 541-553
-
-
Sachs, B.1
-
61
-
-
84939085791
-
A family form of idiocy, generally fatal associated with early blindness
-
B. Sachs A family form of idiocy, generally fatal associated with early blindness J. Nerv. Ment. Dis. 21 1896 475 479
-
(1896)
J. Nerv. Ment. Dis.
, vol.21
, pp. 475-479
-
-
Sachs, B.1
-
62
-
-
0009782043
-
A family form of idiocy, generally fatal, associated with early blindness (amaurotic family idiocy)
-
B. Sachs A family form of idiocy, generally fatal, associated with early blindness (amaurotic family idiocy) NY State J. Med. 63 1896 697 703
-
(1896)
NY State J. Med.
, vol.63
, pp. 697-703
-
-
Sachs, B.1
-
64
-
-
0021483386
-
The biochemical basis of gangliosidoses
-
K. Sandhoff E. Conzelmann The biochemical basis of gangliosidoses Neuropediatrics 15 1984 85 92 (Suppl)
-
(1984)
Neuropediatrics
, vol.15
, pp. 85-92
-
-
Sandhoff, K.1
Conzelmann, E.2
-
65
-
-
84942968610
-
Über den enzymatischen Abbau von N-acetyl-neuraminsäurefreien Gangliosidresten (Ceramid-oligosacchariden)
-
K. Sandhoff H. Pilz H. Jatzkewitz Über den enzymatischen Abbau von N-acetyl-neuraminsäurefreien Gangliosidresten (Ceramid-oligosacchariden) Hoppe-Seyler's Z. Physiol. Chem. 338 1964 281 285
-
(1964)
Hoppe-Seyler's Z. Physiol. Chem.
, vol.338
, pp. 281-285
-
-
Sandhoff, K.1
Pilz, H.2
Jatzkewitz, H.3
-
66
-
-
84871388928
-
Disseration: Die Amaurotische Idiotie des Menschen als Storung im Glyko-sphingolipidstoffwechsel
-
K. Sandhoff Disseration: Die Amaurotische Idiotie des Menschen als Storung im Glyko-sphingolipidstoffwechsel 1965 Universitfit Munchen
-
(1965)
-
-
Sandhoff, K.1
-
67
-
-
0014329346
-
Auftrennung der Sauger-N-Acetyl-β-d-hexosaminidase in multiple Formen durch Elektrofokussierung
-
K. Sandhoff Auftrennung der Sauger-N-Acetyl-β- d -hexosaminidase in multiple Formen durch Elektrofokussierung Hoppe-Seyler's Z. Physiol. Chem. 349 1968 1095 1098
-
(1968)
Hoppe-Seyler's Z. Physiol. Chem.
, vol.349
, pp. 1095-1098
-
-
Sandhoff, K.1
-
68
-
-
0000604351
-
Variation of β-N-acetylhexosaminidase-pattern in Tay-Sachs disease
-
K. Sandhoff Variation of β-N-acetylhexosaminidase-pattern in Tay-Sachs disease FEBS Lett. 4 1969 351 354
-
(1969)
FEBS Lett.
, vol.4
, pp. 351-354
-
-
Sandhoff, K.1
-
69
-
-
0344081930
-
The hydrolysis of Tay-Sachs ganglioside (TSG) by human N-acetyl-β-d-hexosaminidase A
-
K. Sandhoff The hydrolysis of Tay-Sachs ganglioside (TSG) by human N-acetyl-β- d -hexosaminidase A FEBS Lett. 11 1970 342 344
-
(1970)
FEBS Lett.
, vol.11
, pp. 342-344
-
-
Sandhoff, K.1
-
70
-
-
0015106429
-
Anreicherung und Charakterisierungz̃weier Formen der menschlichen N-Acetyl-β-d-hexosaminidase
-
K. Sandhoff W. Wassle Anreicherung und Charakterisierungz̃weier Formen der menschlichen N-Acetyl-β- d -hexosaminidase Hoppe-Seyler's Z. Physiol. Chem. 352 1971 1119 1133
-
(1971)
Hoppe-Seyler's Z. Physiol. Chem.
, vol.352
, pp. 1119-1133
-
-
Sandhoff, K.1
Wassle, W.2
-
71
-
-
0015602778
-
Multiple human hexosaminidases
-
K. Sandhoff Multiple human hexosaminidases 1973 The National Foundation USA 214 222
-
(1973)
, pp. 214-222
-
-
Sandhoff, K.1
-
72
-
-
0014225180
-
Deficient hexosaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs
-
K. Sandhoff U. Andreae H. Jatzkewitz Deficient hexosaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs Pathol. Eur. 3 1968 278 285
-
(1968)
Pathol. Eur.
, vol.3
, pp. 278-285
-
-
Sandhoff, K.1
Andreae, U.2
Jatzkewitz, H.3
-
73
-
-
0015179162
-
Enzyme alterations and lipid storage in three variants of Tay-Sachs disease
-
K. Sandhoff K. Harzer W. Wässle H. Jatzkewitz Enzyme alterations and lipid storage in three variants of Tay-Sachs disease J. Neurochem. 18 1971 2469 2489
-
(1971)
J. Neurochem.
, vol.18
, pp. 2469-2489
-
-
Sandhoff, K.1
Harzer, K.2
Wässle, W.3
Jatzkewitz, H.4
-
74
-
-
0017588481
-
Specificity of human liver hexosaminidases A and B against glycosphingolipids GM2 and GA2. Purification of the enzymes by affinity chromatography employing specific elution
-
A2. Purification of the enzymes by affinity chromatography employing specific elution Hoppe Seyler's Z. Physiol. Chem. 358 1977 779 787
-
(1977)
Hoppe Seyler's Z. Physiol. Chem.
, vol.358
, pp. 779-787
-
-
Sandhoff, K.1
Conzelmann, E.2
Nehrkorn, H.3
-
75
-
-
0029113867
-
Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism
-
K. Sango S. Yamanaka A. Hoffmann Y. Okuda A. Grinberg H. Westphal M.P. McDonald J.N. Crawley K. Sandhoff K. Suzuki R.L. Proia Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism Nat. Genet. 11 1995 170 176
-
(1995)
Nat. Genet.
, vol.11
, pp. 170-176
-
-
Sango, K.1
Yamanaka, S.2
Hoffmann, A.3
Okuda, Y.4
Grinberg, A.5
Westphal, H.6
McDonald, M.P.7
Crawley, J.N.8
Sandhoff, K.9
Suzuki, K.10
Proia, R.L.11
-
79
-
-
0022381832
-
Genetic complementation in somatic cell hybrids of four variants of infantile GM2 gangliosidosis
-
S. Sonderfeld S. Brendler K. Sandhoff H. Galjaard A.T. Hoogeveen Genetic complementation in somatic cell hybrids of four variants of infantile GM2 gangliosidosis Hum. Genet. 71 1985 196 200
-
(1985)
Hum. Genet.
, vol.71
, pp. 196-200
-
-
Sonderfeld, S.1
Brendler, S.2
Sandhoff, K.3
Galjaard, H.4
Hoogeveen, A.T.5
-
81
-
-
0015926887
-
Hexosaminidase-A and Hexosaminidase-B: Studies in Tay-Sachs and Sandhoff's disease
-
S.K. Srivastava E. Beutler Hexosaminidase-A and Hexosaminidase-B: Studies in Tay-Sachs and Sandhoff's disease Nature (Lond.) 241 1973 463
-
(1973)
Nature (Lond.)
, vol.241
, pp. 463
-
-
Srivastava, S.K.1
Beutler, E.2
-
82
-
-
0001505815
-
The chemical structure of normal human brain and Tay-Sachs gangliosides
-
L. Svennerholm The chemical structure of normal human brain and Tay-Sachs gangliosides Biochem. Biophys. Res. Commun. 9 1962 436 441
-
(1962)
Biochem. Biophys. Res. Commun.
, vol.9
, pp. 436-441
-
-
Svennerholm, L.1
-
83
-
-
0018821962
-
Krabbe disease: A galactosylsphingosine (psychosine) lipidosis
-
L. Svennerholm M.-T. Vanier J.-E. Mansson Krabbe disease: A galactosylsphingosine (psychosine) lipidosis J. Lipid Res. 21 1980 53 64
-
(1980)
J. Lipid Res.
, vol.21
, pp. 53-64
-
-
Svennerholm, L.1
Vanier, M.-T.2
Mansson, J.-E.3
-
84
-
-
0000717468
-
Symetrical changes in the region of the yellow spot in each eye of an infant
-
W. Tay Symetrical changes in the region of the yellow spot in each eye of an infant Trans. Ophthalmol. Soc. UK 1 1881 55 57
-
(1881)
Trans. Ophthalmol. Soc. UK
, vol.1
, pp. 55-57
-
-
Tay, W.1
-
85
-
-
3542994691
-
Membranous cytoplasmic granules in infantile amaurotic idiocy
-
R.D. Terry S.R. Korey Membranous cytoplasmic granules in infantile amaurotic idiocy Nature (Lond.) 188 1960 1000 1002
-
(1960)
Nature (Lond.)
, vol.188
, pp. 1000-1002
-
-
Terry, R.D.1
Korey, S.R.2
-
86
-
-
2042443454
-
Studies in Tay-Sachs disease: II Ultrastructure of the cerebrum
-
R.D. Terry M. Weiss Studies in Tay-Sachs disease: II Ultrastructure of the cerebrum J. Neuropathol. Exp. Neurol. 22 1963 18 55
-
(1963)
J. Neuropathol. Exp. Neurol.
, vol.22
, pp. 18-55
-
-
Terry, R.D.1
Weiss, M.2
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