Glycolipid abnormalities in a myoclonic variant of late infantile amaurotic idiocy

Glycolipids were isolated from the brain of a patient with a myoclonic variant of late infantile amaurotic idiocy. There was an abnormal glycolipid pattern in gray and white matter. The observed high concentration of gangliosides was due to a uniform accumulation of all four major gangliosides and w...

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Published inJournal of lipid research Vol. 11; no. 3; pp. 241 - 247
Main Author Bartsch, G G
Format Journal Article
LanguageEnglish
Published United States Elsevier 01.05.1970
Subjects
Brain - metabolism
Carbohydrates - analysis
ceramide lactoside
ceramide tetrahexoside
Cerebrosides - metabolism
Child
Chromatography, Thin Layer
gangliosides
Gangliosides - metabolism
gangliosidosis-GM1
Glycolipids - metabolism
gray matter
Hexoses - analysis
Humans
Lipidoses - metabolism
Male
Tay-Sachs disease
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Abstract Glycolipids were isolated from the brain of a patient with a myoclonic variant of late infantile amaurotic idiocy. There was an abnormal glycolipid pattern in gray and white matter. The observed high concentration of gangliosides was due to a uniform accumulation of all four major gangliosides and was not limited to one species such as ganglioside A(1), as in Tay-Sachs disease, or ganglioside A(2), as in gangliosidosis-Gm1. Two additional stored substances were identified as ceramide lactoside and ceramide tetrahexoside. Partial and total hydrolysis of these ceramide hexosides revealed that their ceramide moiety is identical with the ceramide portion of gangliosides. The sequence of hexoses in the carbohydrate chain of the ceramide dihexoside and ceramide tetrahexoside further suggests a metabolic and chemical relation to gangliosides. Some implications of these findings for the theories of the metabolic defects in gangliosidoses are discussed.
AbstractList Glycolipids were isolated from the brain of a patient with a myoclonic variant of late infantile amaurotic idiocy. There was an abnormal glycolipid pattern in gray and white matter. The observed high concentration of gangliosides was due to a uniform accumulation of all four major gangliosides and was not limited to one species such as ganglioside A(1), as in Tay-Sachs disease, or ganglioside A(2), as in gangliosidosis-Gm1. Two additional stored substances were identified as ceramide lactoside and ceramide tetrahexoside. Partial and total hydrolysis of these ceramide hexosides revealed that their ceramide moiety is identical with the ceramide portion of gangliosides. The sequence of hexoses in the carbohydrate chain of the ceramide dihexoside and ceramide tetrahexoside further suggests a metabolic and chemical relation to gangliosides. Some implications of these findings for the theories of the metabolic defects in gangliosidoses are discussed.
Glycolipids were isolated from the brain of a patient with a myoclonic variant of late infantile amaurotic idiocy. There was an abnormal glycolipid pattern in gray and white matter. The observed high concentration of gangliosides was due to a uniform accumulation of all four major gangliosides and was not limited to one species such as ganglioside A1, as in Tay-Sachs disease, or ganglioside A2, as in gangliosidosis-GM1. Two additional stored substances were identified as ceramide lactoside and ceramide tetrahexoside. Partial and total hydrolysis of these ceramide hexosides revealed that their ceramide moiety is identical with the ceramide portion of gangliosides. The sequence of hexoses in the carbohydrate chain of the ceramide dihexoside and ceramide tetrahexoside further suggests a metabolic and chemical relation to gangliosides. Some implications of these findings for the theories of the metabolic defects in gangliosidoses are discussed.
Author Bartsch, G G
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Cites_doi 10.1192/bjp.104.434.91
10.1111/j.1471-4159.1959.tb12629.x
10.1002/cber.19420751231
10.1016/S0021-9258(18)69851-5
10.1111/j.1471-4159.1963.tb05036.x
10.1002/ange.19600722204
10.1097/00005072-196504000-00012
10.1038/174930a0
10.1097/00005072-196901000-00003
10.1097/00005072-196301000-00005
10.1093/oxfordjournals.jbchem.a127654
10.1016/S0021-9258(18)74577-8
10.1007/BF02533185
10.1016/S0021-9258(18)91935-6
10.1515/bchm2.1939.262.3-5.128
10.1097/00005072-196504000-00011
10.1111/j.1471-4159.1966.tb04290.x
10.1111/j.1471-4159.1966.tb04284.x
10.1515/bchm2.1960.319.1.283
10.1515/znb-1963-0708
10.1126/science.160.3831.1002
10.1001/archpedi.1965.02090020340014
10.1515/bchm2.1962.330.1.218
10.1016/S0022-2275(20)38922-7
10.1111/j.1471-4159.1964.tb06735.x
10.1111/j.1471-4159.1963.tb08933.x
10.3891/acta.chem.scand.02-0467
10.1515/bchm2.1935.235.1-2.24
10.1038/166444b0
10.1111/j.1471-4159.1957.tb12073.x
10.1515/bchm2.1961.326.1.144
10.1016/0006-291X(65)90743-6
10.1515/bchm2.1941.270.3-4.185
10.1021/bi00886a040
10.1042/bj0271824
10.1111/j.1471-4159.1965.tb04256.x
10.1016/S0021-9258(18)91936-8
10.1016/S0021-9258(18)64849-5
10.1007/BF01467396
10.1002/cber.19630960329
10.1097/00005072-196807000-00001
10.1016/0006-3002(63)90764-9
10.1016/0006-291X(62)90030-X
10.1515/bchm2.1967.348.1.1261
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References Okada (10.1016/S0022-2275(20)42990-6_bib56) 1968; 160
Blix (10.1016/S0022-2275(20)42990-6_bib38) 1948; 2
Klenk (10.1016/S0022-2275(20)42990-6_bib34) 1941; 270
Weicker (10.1016/S0022-2275(20)42990-6_bib7) 1960; 19
Klenk (10.1016/S0022-2275(20)42990-6_bib1) 1935; 235
Suzuki (10.1016/S0022-2275(20)42990-6_bib23) 1967; 8
Warren (10.1016/S0022-2275(20)42990-6_bib59) 1959; 234
Trevelyan (10.1016/S0022-2275(20)42990-6_bib40) 1950; 166
Somogyi (10.1016/S0022-2275(20)42990-6_bib37) 1937; 117
Jatzkewitz (10.1016/S0022-2275(20)42990-6_bib19) 1963; 70
Gatt (10.1016/S0022-2275(20)42990-6_bib27) 1963; 10
Duffy (10.1016/S0022-2275(20)42990-6_bib47) 1968; 27
Wagner (10.1016/S0022-2275(20)42990-6_bib55) 1963; 22
Tingey (10.1016/S0022-2275(20)42990-6_bib44) 1958; 104
Klenk (10.1016/S0022-2275(20)42990-6_bib3) 1942; 75
Yip (10.1016/S0022-2275(20)42990-6_bib52) 1969; 4
Samuels (10.1016/S0022-2275(20)42990-6_bib26) 1963; 22
Basu (10.1016/S0022-2275(20)42990-6_bib50) 1968; 243
Klenk (10.1016/S0022-2275(20)42990-6_bib4) 1960; 319
Svennerholm (10.1016/S0022-2275(20)42990-6_bib15) 1963; 10
Brady (10.1016/S0022-2275(20)42990-6_bib57) 1965; 18
Georgias (10.1016/S0022-2275(20)42990-6_bib14) 1966
Klenk (10.1016/S0022-2275(20)42990-6_bib9) 1963; 330
Klenk (10.1016/S0022-2275(20)42990-6_bib5) 1961; 326
Jatzkewitz (10.1016/S0022-2275(20)42990-6_bib29) 1965; 30
Pilz (10.1016/S0022-2275(20)42990-6_bib25) 1966; 13
Svennerholm (10.1016/S0022-2275(20)42990-6_bib42) 1964; 11
Svennerholm (10.1016/S0022-2275(20)42990-6_bib17) 1962; 9
Suzuki (10.1016/S0022-2275(20)42990-6_bib24) 1969; 28
Klenk (10.1016/S0022-2275(20)42990-6_bib2) 1939; 262
Suzuki (10.1016/S0022-2275(20)42990-6_bib31) 1965; 12
Klenk (10.1016/S0022-2275(20)42990-6_bib16) 1965; 21
Klenk (10.1016/S0022-2275(20)42990-6_bib48) 1967; 348
Boehm (10.1016/S0022-2275(20)42990-6_bib35) 1954; 32
Kaufman (10.1016/S0022-2275(20)42990-6_bib49) 1966
Elson (10.1016/S0022-2275(20)42990-6_bib39) 1933; 27
O'Brien (10.1016/S0022-2275(20)42990-6_bib21) 1965; 109
Eberhagen (10.1016/S0022-2275(20)42990-6_bib36) 1962; 3
Kuhn (10.1016/S0022-2275(20)42990-6_bib10) 1963; 96
Kolodny (10.1016/S0022-2275(20)42990-6_bib53) 1969; 28
Dain (10.1016/S0022-2275(20)42990-6_bib8) 1961; 20
Gonatas (10.1016/S0022-2275(20)42990-6_bib20) 1965; 24
Anet (10.1016/S0022-2275(20)42990-6_bib41) 1954; 174
Ledeen (10.1016/S0022-2275(20)42990-6_bib18) 1965; 4
Handa (10.1016/S0022-2275(20)42990-6_bib54) 1966; 25
Klenk (10.1016/S0022-2275(20)42990-6_bib30) 1957; 1
Kuhn (10.1016/S0022-2275(20)42990-6_bib6) 1960; 72
Hof (10.1016/S0022-2275(20)42990-6_bib13) 1965
Makita (10.1016/S0022-2275(20)42990-6_bib28) 1963; 33
Folch (10.1016/S0022-2275(20)42990-6_bib33) 1957; 226
Borri (10.1016/S0022-2275(20)42990-6_bib46) 1966; 13
Kaufman (10.1016/S0022-2275(20)42990-6_bib51) 1968; 243
Tingey (10.1016/S0022-2275(20)42990-6_bib45) 1959; 3
Svennerholm (10.1016/S0022-2275(20)42990-6_bib58) 1966
Kunau (10.1016/S0022-2275(20)42990-6_bib12) 1964
Seitelberger (10.1016/S0022-2275(20)42990-6_bib32) 1966
Ledeen (10.1016/S0022-2275(20)42990-6_bib22) 1965; 24
Kuhn (10.1016/S0022-2275(20)42990-6_bib11) 1963; 18b
Yamakawa (10.1016/S0022-2275(20)42990-6_bib43) 1963; 53
References_xml – year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib14
  contributor:
    fullname: Georgias
– volume: 104
  start-page: 91
  year: 1958
  ident: 10.1016/S0022-2275(20)42990-6_bib44
  publication-title: J. Ment. Sci.
  doi: 10.1192/bjp.104.434.91
  contributor:
    fullname: Tingey
– volume: 3
  start-page: 230
  year: 1959
  ident: 10.1016/S0022-2275(20)42990-6_bib45
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1959.tb12629.x
  contributor:
    fullname: Tingey
– volume: 75
  start-page: 1632
  year: 1942
  ident: 10.1016/S0022-2275(20)42990-6_bib3
  publication-title: Ber. Dt. Chem. Ges.
  doi: 10.1002/cber.19420751231
  contributor:
    fullname: Klenk
– volume: 234
  start-page: 1971
  year: 1959
  ident: 10.1016/S0022-2275(20)42990-6_bib59
  publication-title: J. Biol. Ch.
  doi: 10.1016/S0021-9258(18)69851-5
  contributor:
    fullname: Warren
– volume: 10
  start-page: 43
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib27
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1963.tb05036.x
  contributor:
    fullname: Gatt
– volume: 72
  start-page: 805
  year: 1960
  ident: 10.1016/S0022-2275(20)42990-6_bib6
  publication-title: Angew. Chem.
  doi: 10.1002/ange.19600722204
  contributor:
    fullname: Kuhn
– volume: 24
  start-page: 341
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib22
  publication-title: J. Neuropathol. Exp. Neurol.
  doi: 10.1097/00005072-196504000-00012
  contributor:
    fullname: Ledeen
– volume: 28
  start-page: 1879
  year: 1969
  ident: 10.1016/S0022-2275(20)42990-6_bib53
  publication-title: Fed. Proc.
  contributor:
    fullname: Kolodny
– volume: 174
  start-page: 930
  year: 1954
  ident: 10.1016/S0022-2275(20)42990-6_bib41
  publication-title: Nature (London).
  doi: 10.1038/174930a0
  contributor:
    fullname: Anet
– start-page: 169
  year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib58
  contributor:
    fullname: Svennerholm
– volume: 28
  start-page: 25
  year: 1969
  ident: 10.1016/S0022-2275(20)42990-6_bib24
  publication-title: J. Neuropathd. Exp. Neurol.
  doi: 10.1097/00005072-196901000-00003
  contributor:
    fullname: Suzuki
– volume: 22
  start-page: 81
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib26
  publication-title: J. Neuropathol. Exp. Neurol.
  doi: 10.1097/00005072-196301000-00005
  contributor:
    fullname: Samuels
– volume: 53
  start-page: 28
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib43
  publication-title: J. Biochem. (Tokyo).
  doi: 10.1093/oxfordjournals.jbchem.a127654
  contributor:
    fullname: Yamakawa
– volume: 117
  start-page: 771
  year: 1937
  ident: 10.1016/S0022-2275(20)42990-6_bib37
  publication-title: J. Biol. Chem.
  doi: 10.1016/S0021-9258(18)74577-8
  contributor:
    fullname: Somogyi
– volume: 4
  start-page: 270
  year: 1969
  ident: 10.1016/S0022-2275(20)42990-6_bib52
  publication-title: Lipids.
  doi: 10.1007/BF02533185
  contributor:
    fullname: Yip
– volume: 243
  start-page: 5802
  year: 1968
  ident: 10.1016/S0022-2275(20)42990-6_bib50
  publication-title: J. Biol. Ch.
  doi: 10.1016/S0021-9258(18)91935-6
  contributor:
    fullname: Basu
– volume: 262
  start-page: 128
  year: 1939
  ident: 10.1016/S0022-2275(20)42990-6_bib2
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1939.262.3-5.128
  contributor:
    fullname: Klenk
– volume: 25
  start-page: 587
  year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib54
  publication-title: Fed. Proc.
  contributor:
    fullname: Handa
– volume: 24
  start-page: 318
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib20
  publication-title: J. Neuropathol. Exp. Neurol.
  doi: 10.1097/00005072-196504000-00011
  contributor:
    fullname: Gonatas
– year: 1964
  ident: 10.1016/S0022-2275(20)42990-6_bib12
  contributor:
    fullname: Kunau
– volume: 13
  start-page: 1273
  year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib25
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1966.tb04290.x
  contributor:
    fullname: Pilz
– start-page: 43
  year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib32
  contributor:
    fullname: Seitelberger
– volume: 13
  start-page: 1249
  year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib46
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1966.tb04284.x
  contributor:
    fullname: Borri
– volume: 319
  start-page: 283
  year: 1960
  ident: 10.1016/S0022-2275(20)42990-6_bib4
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1960.319.1.283
  contributor:
    fullname: Klenk
– volume: 18b
  start-page: 541
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib11
  publication-title: Z. Naturforsch.
  doi: 10.1515/znb-1963-0708
  contributor:
    fullname: Kuhn
– volume: 160
  start-page: 1002
  year: 1968
  ident: 10.1016/S0022-2275(20)42990-6_bib56
  publication-title: Science (Washington).
  doi: 10.1126/science.160.3831.1002
  contributor:
    fullname: Okada
– volume: 33
  start-page: 361
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib28
  publication-title: Jap. J. Exp. Med.
  contributor:
    fullname: Makita
– year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib13
  contributor:
    fullname: Hof
– volume: 109
  start-page: 338
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib21
  publication-title: Amer. J. Dis. Child.
  doi: 10.1001/archpedi.1965.02090020340014
  contributor:
    fullname: O'Brien
– start-page: 193
  year: 1966
  ident: 10.1016/S0022-2275(20)42990-6_bib49
  contributor:
    fullname: Kaufman
– volume: 22
  start-page: 1039
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib55
  publication-title: Fed. Proc.
  contributor:
    fullname: Wagner
– volume: 330
  start-page: 218
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib9
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1962.330.1.218
  contributor:
    fullname: Klenk
– volume: 8
  start-page: 105
  year: 1967
  ident: 10.1016/S0022-2275(20)42990-6_bib23
  publication-title: J. Lipid Res.
  doi: 10.1016/S0022-2275(20)38922-7
  contributor:
    fullname: Suzuki
– volume: 11
  start-page: 839
  year: 1964
  ident: 10.1016/S0022-2275(20)42990-6_bib42
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1964.tb06735.x
  contributor:
    fullname: Svennerholm
– volume: 10
  start-page: 613
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib15
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1963.tb08933.x
  contributor:
    fullname: Svennerholm
– volume: 2
  start-page: 467
  year: 1948
  ident: 10.1016/S0022-2275(20)42990-6_bib38
  publication-title: Acta Chm. Scand.
  doi: 10.3891/acta.chem.scand.02-0467
  contributor:
    fullname: Blix
– volume: 235
  start-page: 24
  year: 1935
  ident: 10.1016/S0022-2275(20)42990-6_bib1
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1935.235.1-2.24
  contributor:
    fullname: Klenk
– volume: 3
  start-page: 382
  year: 1962
  ident: 10.1016/S0022-2275(20)42990-6_bib36
  publication-title: J. LipidRes.
  contributor:
    fullname: Eberhagen
– volume: 166
  start-page: 444
  year: 1950
  ident: 10.1016/S0022-2275(20)42990-6_bib40
  publication-title: Nature (London).
  doi: 10.1038/166444b0
  contributor:
    fullname: Trevelyan
– volume: 20
  start-page: 269
  year: 1961
  ident: 10.1016/S0022-2275(20)42990-6_bib8
  publication-title: Fed. Proc.
  contributor:
    fullname: Dain
– volume: 1
  start-page: 203
  year: 1957
  ident: 10.1016/S0022-2275(20)42990-6_bib30
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1957.tb12073.x
  contributor:
    fullname: Klenk
– volume: 326
  start-page: 144
  year: 1961
  ident: 10.1016/S0022-2275(20)42990-6_bib5
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1961.326.1.144
  contributor:
    fullname: Klenk
– volume: 18
  start-page: 221
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib57
  publication-title: Biochem. Biophys. Res. Commun.
  doi: 10.1016/0006-291X(65)90743-6
  contributor:
    fullname: Brady
– volume: 270
  start-page: 185
  year: 1941
  ident: 10.1016/S0022-2275(20)42990-6_bib34
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1941.270.3-4.185
  contributor:
    fullname: Klenk
– volume: 4
  start-page: 2225
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib18
  publication-title: Biochemistry.
  doi: 10.1021/bi00886a040
  contributor:
    fullname: Ledeen
– volume: 30
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib29
  publication-title: J. Neuro
  contributor:
    fullname: Jatzkewitz
– volume: 27
  start-page: 1824
  year: 1933
  ident: 10.1016/S0022-2275(20)42990-6_bib39
  publication-title: Biochem. J.
  doi: 10.1042/bj0271824
  contributor:
    fullname: Elson
– volume: 12
  start-page: 629
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib31
  publication-title: J. Neurochem.
  doi: 10.1111/j.1471-4159.1965.tb04256.x
  contributor:
    fullname: Suzuki
– volume: 243
  start-page: 5804
  year: 1968
  ident: 10.1016/S0022-2275(20)42990-6_bib51
  publication-title: J. Biol. Chem.
  doi: 10.1016/S0021-9258(18)91936-8
  contributor:
    fullname: Kaufman
– volume: 226
  start-page: 497
  year: 1957
  ident: 10.1016/S0022-2275(20)42990-6_bib33
  publication-title: I. Biol. Chem.
  doi: 10.1016/S0021-9258(18)64849-5
  contributor:
    fullname: Folch
– volume: 32
  start-page: 289
  year: 1954
  ident: 10.1016/S0022-2275(20)42990-6_bib35
  publication-title: Klin. Wochenschr.
  doi: 10.1007/BF01467396
  contributor:
    fullname: Boehm
– volume: 96
  start-page: 866
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib10
  publication-title: Chem. Ber.
  doi: 10.1002/cber.19630960329
  contributor:
    fullname: Kuhn
– volume: 19
  start-page: 219
  year: 1960
  ident: 10.1016/S0022-2275(20)42990-6_bib7
  publication-title: Fed. Proc.
  contributor:
    fullname: Weicker
– volume: 21
  start-page: 405
  year: 1965
  ident: 10.1016/S0022-2275(20)42990-6_bib16
  publication-title: Acta Med. Acad. Sci. Hung.
  contributor:
    fullname: Klenk
– volume: 27
  start-page: 351
  year: 1968
  ident: 10.1016/S0022-2275(20)42990-6_bib47
  publication-title: J. Neuropathol. Exp. Neurol.
  doi: 10.1097/00005072-196807000-00001
  contributor:
    fullname: Duffy
– volume: 70
  start-page: 354
  year: 1963
  ident: 10.1016/S0022-2275(20)42990-6_bib19
  publication-title: Biochim. Biophys. Acta.
  doi: 10.1016/0006-3002(63)90764-9
  contributor:
    fullname: Jatzkewitz
– volume: 9
  start-page: 436
  year: 1962
  ident: 10.1016/S0022-2275(20)42990-6_bib17
  publication-title: Biochem. Biophys. Res. Commun.
  doi: 10.1016/0006-291X(62)90030-X
  contributor:
    fullname: Svennerholm
– volume: 348
  start-page: 1261
  year: 1967
  ident: 10.1016/S0022-2275(20)42990-6_bib48
  publication-title: Z. Phys. Chem.
  doi: 10.1515/bchm2.1967.348.1.1261
  contributor:
    fullname: Klenk
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Snippet Glycolipids were isolated from the brain of a patient with a myoclonic variant of late infantile amaurotic idiocy. There was an abnormal glycolipid pattern in...
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SubjectTerms Brain - metabolism
Carbohydrates - analysis
ceramide lactoside
ceramide tetrahexoside
Cerebrosides - metabolism
Child
Chromatography, Thin Layer
gangliosides
Gangliosides - metabolism
gangliosidosis-GM1
Glycolipids - metabolism
gray matter
Hexoses - analysis
Humans
Lipidoses - metabolism
Male
Tay-Sachs disease
Title Glycolipid abnormalities in a myoclonic variant of late infantile amaurotic idiocy
URI https://www.ncbi.nlm.nih.gov/pubmed/5441250
https://search.proquest.com/docview/84567091
https://doaj.org/article/c6f6a8d4279b43dc96066bb80aa8b35f
Volume 11
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