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UDP-galactose + ceramide
UDP + D-galactosylceramide
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
UDPglucose + 2-(2-hydroxyacyl)sphingosine
glucocerebroside + UDP
-
at 7-10% of the activity with UDP-galactose
-
?
additional information
?
-
UDP-galactose + ceramide
UDP + D-galactosylceramide
-
-
-
?
UDP-galactose + ceramide
UDP + D-galactosylceramide
-
erythro-ceramides, unsaturated ceramides and bound sphingosines are more active than threo-ceramides, saturated and free sphingosines
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
highly specific
product: galactocerebroside containing 2-hydroxy fatty acids linked to the amino group of the sphingosine moiety
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
ceramide containing 2-hydroxystearic acid as the predominant fatty acid
product: galactocerebroside containing 2-hydroxy fatty acids linked to the amino group of the sphingosine moiety
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide, synthesis of cerebroside
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
enzyme may play an important role in the synthesis of at least one type of myelin
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
galactoceramide
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide
i.e. 2-hydroxy fatty acid galactosylceramide
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
key enzymatic step in the biosynthesis of the galactocerebrosides, cell-specific and highly time-regulated expression of the CGT gene in the terminal differentiated oligodendrocytes of CNS and in Schwann cells of PNS
galactocerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
key enzyme in the biosynthesis of galactocerebroside, the most abundant glycosphingolipid in the myelin sheath
galactocerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
highly specific
2-hydroxy fatty acid cerebroside, i.e. 2-hydroxy fatty acid galactosylceramide
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide
2-hydroxy fatty acid cerebroside, i.e. 2-hydroxy fatty acid galactosylceramide
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide, synthesis of cerebroside
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
cerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
i.e. 2-hydroxy fatty acid galactosylceramide
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide
cerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide
cerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide
2-hydroxy fatty acid cerebroside, i.e. 2-hydroxy fatty acid galactosylceramide
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
-
cerebroside
?
additional information
?
-
-
not: galactose, galactose-1-phosphat, ADP-galactose
-
-
?
additional information
?
-
-
-
-
-
?
additional information
?
-
-
final step of galactosylceramide synthesis, which plays a role in myelin formation, signal transduction and oligodendrocyte development
-
-
?
additional information
?
-
-
not: ceramide containing nonhydroxy fatty acids
-
-
?
additional information
?
-
-
key enzyme in the biosynthesis of cerebrosides and sulfatides, cell-specific and highly time-regulated expression of the CGT gene is thought to play an important role in oligodendrocyte and Schwann cell differentiation
-
-
?
additional information
?
-
-
-
-
-
?
additional information
?
-
-
-
-
-
?
additional information
?
-
-
not: glucosylceramide, lactosylceramide, asialo GM2ganglioside
-
-
?
additional information
?
-
-
not: ceramide containing nonhydroxy fatty acids
-
-
?
additional information
?
-
-
oligodendrocyte-specific enzyme is involved in the biosynthesis of the oligodendrocyte- and myelin-specific cerebrosides, time-regulated CGT expression with a peak around postnatal days 15-25
-
-
?
additional information
?
-
-
last step in biosynthesis of cerebrosides
-
-
?
additional information
?
-
-
enzyme ensures a supply of UDP-galactose in the endoplasmic reticulum lumen by retaining UDP-galactose transporter in a molecular complex
-
-
?
additional information
?
-
-
oligodendrocyte-specific enzyme is involved in the biosynthesis of the oligodendrocyte- and myelin-specific cerebrosides, time-regulated CGT expression with a peak around postnatal days 15-25
-
-
?
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UDP-galactose + ceramide
UDP + D-galactosylceramide
-
-
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
additional information
?
-
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide, synthesis of cerebroside
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
enzyme may play an important role in the synthesis of at least one type of myelin
-
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
key enzymatic step in the biosynthesis of the galactocerebrosides, cell-specific and highly time-regulated expression of the CGT gene in the terminal differentiated oligodendrocytes of CNS and in Schwann cells of PNS
galactocerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
key enzyme in the biosynthesis of galactocerebroside, the most abundant glycosphingolipid in the myelin sheath
galactocerebroside
?
UDPgalactose + 2-(2-hydroxyacyl)sphingosine
1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine + UDP
-
i.e. 2-hydroxy fatty acid ceramide, synthesis of cerebroside
-
?
additional information
?
-
-
-
-
-
?
additional information
?
-
-
final step of galactosylceramide synthesis, which plays a role in myelin formation, signal transduction and oligodendrocyte development
-
-
?
additional information
?
-
-
key enzyme in the biosynthesis of cerebrosides and sulfatides, cell-specific and highly time-regulated expression of the CGT gene is thought to play an important role in oligodendrocyte and Schwann cell differentiation
-
-
?
additional information
?
-
-
-
-
-
?
additional information
?
-
-
oligodendrocyte-specific enzyme is involved in the biosynthesis of the oligodendrocyte- and myelin-specific cerebrosides, time-regulated CGT expression with a peak around postnatal days 15-25
-
-
?
additional information
?
-
-
last step in biosynthesis of cerebrosides
-
-
?
additional information
?
-
-
enzyme ensures a supply of UDP-galactose in the endoplasmic reticulum lumen by retaining UDP-galactose transporter in a molecular complex
-
-
?
additional information
?
-
-
oligodendrocyte-specific enzyme is involved in the biosynthesis of the oligodendrocyte- and myelin-specific cerebrosides, time-regulated CGT expression with a peak around postnatal days 15-25
-
-
?
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Ataxia
Disruption of axo-glial junctions causes cytoskeletal disorganization and degeneration of Purkinje neuron axons.
Breast Neoplasms
Association between CLN3 (Neuronal Ceroid Lipofuscinosis, CLN3 Type) Gene Expression and Clinical Characteristics of Breast Cancer Patients.
Breast Neoplasms
Ceramide galactosyltransferase (UGT8) is a molecular marker of breast cancer malignancy and lung metastases.
Breast Neoplasms
Inhibition of UGT8 suppresses basal-like breast cancer progression by attenuating sulfatide-?V?5 axis.
Demyelinating Diseases
Oligodendrocyte-specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT-deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide level.
Demyelinating Diseases
Oligodendrocytes assist in the maintenance of sodium channel clusters independent of the myelin sheath.
Demyelinating Diseases
Tellurium causes dose-dependent coordinate down-regulation of myelin gene expression.
Galactosemias
The molecular relationship between deficient UDP-galactose uridyl transferase (GALT) and ceramide galactosyltransferase (CGT) enzyme function: a possible cause for poor long-term prognosis in classic galactosemia.
Leukodystrophy, Globoid Cell
Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor.
Leukodystrophy, Metachromatic
Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor.
Lung Neoplasms
Expression of Ceramide Galactosyltransferase (UGT8) in Primary and Metastatic Lung Tissues of Non-Small-Cell Lung Cancer.
Neoplasm Metastasis
Ceramide galactosyltransferase (UGT8) is a molecular marker of breast cancer malignancy and lung metastases.
Neoplasms
Ceramide galactosyltransferase (UGT8) as a molecular marker of canine mammary tumor malignancy.
Neoplasms
Ceramide galactosyltransferase (UGT8) is a molecular marker of breast cancer malignancy and lung metastases.
Neoplasms
Microarray analysis of altered sphingolipid metabolism reveals prognostic significance of sphingosine kinase 1 in breast cancer.
Oligodendroglioma
Biosynthesis of galactocerebrosides and glucocerebrosides in glial cell lines.
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Morell, P.; Radin, N.S.
Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing hydroxy fatty acid
Biochemistry
8
506-512
1969
Mus musculus
brenda
Basu, S.; Schultz, A.M.; Basu, M.; Roseman, S.
Enzymatic synthesis of galactocerebroside by a galactosyltransferase from embryonic chicken brain
J. Biol. Chem.
246
4272-4279
1971
Cavia porcellus, Gallus gallus, Rattus norvegicus, Sus scrofa
brenda
Neskovic, N.M.; Mandel, P.; Gatt, S.
UDPgalactose: ceramide galactosyltransferase from rat brain
Methods Enzymol.
71
521-527
1981
Rattus norvegicus
brenda
Constantino-Ceccarini, E.; Cestelli, A.
A novel assay method for the biosynthesis of galactosyl- and glucosylceramides
Methods Enzymol.
72
384-391
1981
Rattus norvegicus
brenda
Neskovic, N.M.; Sarlieve, L.L.; Mandel, P.
Brain UDPgalactose: ceramide galactosyltransferase Purification of a catalytically active protein obtained after proteolytic digestion
Biochim. Biophys. Acta
429
342-351
1976
Rattus norvegicus
brenda
Berardi, A.; Fabrizi, G.M.; Manetti, R.; Costantino-Ceccarini, E.
Purification and characterization of UDP-gal:ceramide galactosyltransferase and reconstitution of its activity by incorporation into liposomes
NATO ASI Ser. A, Life Sci.
150
681-687
1988
Rattus norvegicus
-
brenda
Bosio, A.; Binczek, E.; Stoffel, W.
Molecular cloning and characterization of the mouse CGT gene encoding UDP-galactose ceramide-galactosyltransferase (cerebroside synthetase)
Genomics
35
223-226
1996
Homo sapiens, Mus musculus, Rattus norvegicus
brenda
Kapitonov, D.; Yu, R.K.
Cloning, characterization, and expression of human ceramide galactosyltransferase cDNA
Biochem. Biophys. Res. Commun.
232
449-453
1997
Gallus gallus, Homo sapiens
brenda
Bosio, A.; Binczek, E.; Le Beau, M.M.; Fernald, A.A.; Stoffel, W.
The human gene CGT encoding the UDP-galactose ceramide galactosyl transferase (cerebroside synthase): Cloning, characterization, and assignment to human chromosome 4, band q26
Genomics
34
69-75
1996
Homo sapiens
brenda
Padmini, S.; Rao, P.S.
UDP galactose:ceramide galactosyltransferase, CDP choline:1,2-diacyl-sn-glycerol phosphocholine transferase, and microsomal reductases in major regions of the developing rat brain in nutritional stress
J. Neurosci. Res.
23
310-315
1989
Rattus norvegicus
brenda
Tencomnao, T.; Yu, R.K.; Kapitonov, D.
Characterization of the human UDP-galactose:ceramide galactosyltransferase gene promoter
Biochim. Biophys. Acta
1517
416-423
2001
Homo sapiens
brenda
Schulte, S.; Stoffel, W.
UDP galactose:ceramide galactosyltransferase and glutamate/aspartate transporter. Copurification, separation and characterization of the two glycoproteins
Eur. J. Biochem.
233
947-953
1995
Rattus norvegicus
brenda
Fujino, Y.; Nakano, M.
Enzymic synthesis of cerebroside from ceramide and uridine diphosphate galactose
Biochem. J.
113
573-575
1969
Rattus norvegicus
brenda
Fewou, S.N.; Bussow, H.; Schaeren-Wiemers, N.; Vanier, M.T.; Macklin, W.B.; Gieselmann, V.; Eckhardt, M.
Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase
J. Neurochem.
94
469-481
2005
Rattus norvegicus
brenda
Sprong, H.; Degroote, S.; Nilsson, T.; Kawakita, M.; Ishida, N.; van der Sluijs, P.; van Meer, G.
Association of the Golgi UDP-galactose transporter with UDP-galactose:ceramide galactosyltransferase allows UDP-galactose import in the endoplasmic reticulum
Mol. Biol. Cell
14
3482-3493
2003
Rattus norvegicus
brenda
Niimura, Y.; Moue, T.; Takahashi, N.; Nagai, K.I.
Modification of sphingoglycolipids and sulfolipids in kidney cell lines under heat stress: Activation of monohexosylceramide synthesis as a ceramide scavenger
Glycobiology
20
711-717
2010
Canis lupus familiaris, Chlorocebus sabaeus
brenda
Rzechonek, A.; Cygan, M.; Blasiak, P.; Muszczynska-Bernhard, B.; Bobek, V.; Lubicz, M.; Adamiak, J.
Expression of ceramide calactosyltransferase (UGT8) in primary and metastatic lung tissues of non-small-cell lung cancer
Adv. Exp. Med. Biol.
952
51-58
2016
Homo sapiens (Q16880), Homo sapiens
brenda
Pannuzzo, G.; Graziano, A.C.; Pannuzzo, M.; Masman, M.F.; Avola, R.; Cardile, V.
Zoledronate derivatives as potential inhibitors of uridine diphosphate-galactose ceramide galactosyltransferase 8 A combined molecular docking and dynamic study
J. Neurosci. Res.
94
1318-1326
2016
Homo sapiens (Q16880), Homo sapiens
brenda