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3.2.1.46: galactosylceramidase

This is an abbreviated version!
For detailed information about galactosylceramidase, go to the full flat file.

Word Map on EC 3.2.1.46

Reaction

a D-galactosyl-N-acylsphingosine
+
H2O
=
D-galactose
+
a ceramide

Synonyms

beta-galactocerebrosidase, beta-galactosylceramidase, ceramidase, galacatosyl-, ceramide galactosidase, cerebroside beta-galactosidase, cerebroside galactosidase, galactoceramidase, galactocerebrosidase, galactocerebroside beta-galactosidase, galactocerebroside galactosidase, galactocerebroside-beta-D-galactosidase, Galactosylceramidase, galactosylceramidase I, galactosylceramide beta-galactosidase, galactosylcerebrosidase, GALC, galcerase, GM1 ganglioside beta-galactosidase, lactosylceramidase, lactosylceramidase I

ECTree

     3 Hydrolases
         3.2 Glycosylases
             3.2.1 Glycosidases, i.e. enzymes that hydrolyse O- and S-glycosyl compounds
                3.2.1.46 galactosylceramidase

Reference

Reference on EC 3.2.1.46 - galactosylceramidase

Please use the Reference Search for a specific query.
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Goda, S.; Kobayashi, T.; Goto, I.
Hydrolysis of galactosylsphingosine and lactosylsphingosine by beta-galactosidases in human brain and cultured fibroblasts
Biochim. Biophys. Acta
920
259-265
1987
Homo sapiens
Manually annotated by BRENDA team
Kobayashi, T.; Shinnoh, N.; Kuroiwa, Y.
Metabolism of galactosylceramide in the twitcher mouse, an animal model of human globoid cell leukodystrophy
Biochim. Biophys. Acta
879
215-220
1986
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Kobayashi, T.; Shinnoh, N.; Goto, I.; Kuroiwa, Y.
Hydrolysis of galactosylceramide is catalyzed by two genetically distinct acid beta-galactosidases
J. Biol. Chem.
260
14982-14987
1985
Homo sapiens
Manually annotated by BRENDA team
Kato, T.; Suzuki, Y.
Enzymatic determination of galactosylceramide galactosidase in tissues by NAD cycling
Anal. Biochem.
126
44-51
1982
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Ben-Yoseph, Y.; Hungerford, M.; Nadler, H.L.
The interrelations between high- and low-molecular weight forms of normal and mutant (Krabbe-disease) galactocerebrosidase
Biochem. J.
189
9-15
1980
Homo sapiens
Manually annotated by BRENDA team
Hanada, E.; Suzuki, K.
Activation of human brain galactosylceramidase by phosphatidylserine
Biochim. Biophys. Acta
575
410-420
1979
Homo sapiens
Manually annotated by BRENDA team
Ben-Yoseph, Y.; Hungerford, M.; Nadler, H.L.
Galactosylceramide beta-galactosidase in Krabbe disease: partial purification and characterization of the mutant enzyme
Arch. Biochem. Biophys.
196
93-101
1979
Homo sapiens
Manually annotated by BRENDA team
Arora, R.C.; Lin, Y.N.; Radin, N.S.
The inhibitor-sensitive sites of galactosyl ceramide galactosidase
Arch. Biochem. Biophys.
156
77-83
1973
Rattus norvegicus
Manually annotated by BRENDA team
Rushton, A.R.; Dawson, G.
Glycosphinoglipid beta-galactosidases of cultured mammalian cells. Characterization of the enzymes from mouse cell line lmtk and human Lesch-Nyhan fibroblasts
Biochim. Biophys. Acta
388
92-105
1975
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Besley, G.T.N.
Isoelectric focusing of galactosylceramide beta-galactosylceramide beta-galactosidase in cultured skin fibroblasts of patients with Krabbes globoid-cell leucodystrophy
Biochem. Soc. Trans.
3
241-244
1975
Homo sapiens
Manually annotated by BRENDA team
Chiao, Y.B.; Moffitt, K.; Smallwood, Y.; Glew, R.H.
Glyconamides as inhibitors of human beta-glucosidases and beta-galactosidases
Arch. Biochem. Biophys.
192
1-9
1979
Homo sapiens
Manually annotated by BRENDA team
Wiederschain, G.; Raghavan, S.; Kolodny, E.
Characterization of 6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside as fluorogenic substrate of galactocerebrosidase for the diagnosis of Krabbe disease
Clin. Chim. Acta
205
87-96
1992
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Chen, Y.Q.; Wenger, D.A.
Galactocerebrosidase from human urine: purification and partial characterization
Biochim. Biophys. Acta
1170
53-61
1993
Homo sapiens
Manually annotated by BRENDA team
Sakai, N.; Inui, K.; Midorikawa, M.; Okuno, Y.; Ueda, S.; Iwamatsu, A.; Okada, S.
Purification and characterization of galactocerebrosidase from human lymphocytes
J. Biochem.
116
615-620
1994
Homo sapiens
Manually annotated by BRENDA team
Sakai, N.; Inui, K.; Fujii, N.; Fukushima, H.; Nishimoto, J.; Yanagihara, I.; Isegawa, Y.; Iwamatsu, A.; Okada, S.
Krabbe disease: isolation and characterization of a full-length cDNA for human galactocerebrosidase
Biochem. Biophys. Res. Commun.
198
485-491
1994
Homo sapiens
Manually annotated by BRENDA team
Luzi, P.; Rafi, M.A.; Victoria, T.; Baskin, G.B.; Wenger, D.A.
Characterization of the rhesus monkey galactocerebrosidase (GALC) cDNA and gene and identification of the mutation causing globoid cell leukodystrophy (Krabbe disease) in this primate
Genomics
42
319-324
1997
Macaca mulatta (O02791), Macaca mulatta
Manually annotated by BRENDA team
Nagano, S.; Yamada, T.; Shinnoh, N.; Furuya, H.; Taniwaki, T.; Kira, J.
Expression and processing of recombinant human galactosylceramidase
Clin. Chim. Acta
276
53-61
1998
Homo sapiens
Manually annotated by BRENDA team
deGasperi, R.; Raghavan, S.S.; Sosa, M.G.; Kolodny, E.H.; Carrier, C.; Rubenstein, P.; Peters, C.; Wagner, J.; Kurtzberg, J.; Krivit, W.
Measurements from normal umbilical cord blood of four lysosomal enzymatic activities: alpha-L-iduronidase (Hurler), galactocerebrosidase (globoid cell leukodystrophy), arylsulfatase A (metachromatic leukodystrophy), arylsulfatase B (Maroteaux-Lamy)
Bone Marrow Transplant.
25
541-544
2000
Homo sapiens
Manually annotated by BRENDA team
Zhou, X.; Turecek, F.; Scott, C.R.; Gelb, M.H.
Quantification of cellular acid sphingomyelinase and galactocerebroside beta-galactosidase activities by electrospray ionization mass spectrometry
Clin. Chem.
47
874-881
2001
Homo sapiens
Manually annotated by BRENDA team
Dolcetta, D.; Perani, L.; Givogri, M.I.; Galbiati, F.; Orlacchio, A.; Martino, S.; Roncarolo, M.G.; Bongarzone, E.
Analysis of galactocerebrosidase activity in the mouse brain by a new histological staining method
J. Neurosci. Res.
77
462-464
2004
Mus musculus
Manually annotated by BRENDA team
Luddi, A.; Strazza, M.; Carbone, M.; Moretti, E.; Costantino-Ceccarini, E.
Galactosylceramidase deficiency causes sperm abnormalities in the mouse model of globoid cell leukodystrophy
Exp. Cell Res.
304
59-68
2005
Mus musculus
Manually annotated by BRENDA team
Lee, W.C.; Tsoi, Y.K.; Troendle, F.J.; DeLucia, M.W.; Ahmed, Z.; Dicky, C.A.; Dickson, D.W.; Eckman, C.B.
Single-dose intracerebroventricular administration of galactocerebrosidase improves survival in a mouse model of globoid cell leukodystrophy
FASEB J.
21
2520-2527
2007
Mus musculus
Manually annotated by BRENDA team
Zhang, X.Y.; Dinh, A.; Cronin, J.; Li, S.C.; Reiser, J.
Cellular uptake and lysosomal delivery of galactocerebrosidase tagged with the HIV Tat protein transduction domain
J. Neurochem.
104
1055-1064
2008
Mus musculus
Manually annotated by BRENDA team
Martino, S.; Tiribuzi, R.; Tortori, A.; Conti, D.; Visigalli, I.; Lattanzi, A.; Biffi, A.; Gritti, A.; Orlacchio, A.
Specific determination of beta-galactocerebrosidase activity via competitive inhibition of beta-galactosidase
Clin. Chem.
55
541-548
2009
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Visigalli, I.; Ungari, S.; Martino, S.; Park, H.; Cesani, M.; Gentner, B.; Sergi Sergi, L.; Orlacchio, A.; Naldini, L.; Biffi, A.
The galactocerebrosidase enzyme contributes to the maintenance of a functional hematopoietic stem cell niche
Blood
116
1857-1866
2010
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Deane, J.E.; Graham, S.C.; Kim, N.N.; Stein, P.E.; McNair, R.; Cachon-Gonzalez, M.B.; Cox, T.M.; Read, R.J.
Insights into Krabbe disease from structures of galactocerebrosidase
Proc. Natl. Acad. Sci. USA
108
15169-15173
2011
Mus musculus (P54818)
Manually annotated by BRENDA team
Liao, H.C.; Spacil, Z.; Ghomashchi, F.; Escolar, M.L.; Kurtzberg, J.; Orsini, J.J.; Turecek, F.; Scott, C.R.; Gelb, M.H.
Lymphocyte galactocerebrosidase activity by LC-MS/MS for post-newborn screening evaluation of Krabbe disease
Clin. Chem.
63
1363-1369
2017
Homo sapiens (P54803)
Manually annotated by BRENDA team
Scott-Hewitt, N.J.; Folts, C.J.; Hogestyn, J.M.; Piester, G.; Mayer-Proeschel, M.; Noble, M.D.
Heterozygote galactocerebrosidase (GALC) mutants have reduced remyelination and impaired myelin debris clearance following demyelinating injury
Hum. Mol. Genet.
26
2825-2837
2017
Mus musculus (P54818)
Manually annotated by BRENDA team
Saavedra-Matiz, C.A.; Luzi, P.; Nichols, M.; Orsini, J.J.; Caggana, M.; Wenger, D.A.
Expression of individual mutations and haplotypes in the galactocerebrosidase gene identified by the newborn screening program in New York State and in confirmed cases of Krabbes disease
J. Neurosci. Res.
94
1076-1083
2016
Homo sapiens (P54803), Homo sapiens
Manually annotated by BRENDA team
Jang, D.S.; Ye, W.; Guimei, T.; Solomon, M.; Southall, N.; Hu, X.; Marugan, J.; Ferrer, M.; Maegawa, G.H.
Cell-based high-throughput screening identifies galactocerebrosidase enhancers as potential small-molecule therapies for Krabbes disease
J. Neurosci. Res.
94
1231-1245
2016
Homo sapiens (P54803), Homo sapiens
Manually annotated by BRENDA team
Luddi, A.; Crifasi, L.; Capaldo, A.; Piomboni, P.; Costantino-Ceccarini, E.
Suppression of galactocerebrosidase premature termination codon and rescue of galactocerebrosidase activity in twitcher cells
J. Neurosci. Res.
94
1273-1283
2016
Homo sapiens (P54803), Homo sapiens, Mus musculus (P54818), Mus musculus
Manually annotated by BRENDA team