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Literature summary extracted from

  • 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 (2009), Clin. Chem., 55, 541-548.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.46 medicine diagnosis of Krabbe disease Mus musculus
3.2.1.46 medicine diagnosis of Krabbe disease Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.2.1.46 expression in lentiviral GALC vectors Homo sapiens
3.2.1.46 exression in lentiviral GALC vectors Mus musculus

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.2.1.23 AgNO3 competitive inhibitor Mus musculus
3.2.1.23 FeCl3
-
Mus musculus
3.2.1.23 HgCl2
-
Mus musculus
3.2.1.23 ZnCl2
-
Mus musculus

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.2.1.23 0.363
-
4-methylumbelliferyl-beta-D-galactopyranoside without inhibitor Mus musculus
3.2.1.23 4.94
-
4-methylumbelliferyl-beta-D-galactopyranoside in presence of inhibitor Mus musculus
3.2.1.46 0.47
-
4-methylumbelliferyl-beta-D-galactopyranoside
-
Mus musculus
3.2.1.46 0.47
-
4-methylumbelliferyl-beta-D-galactopyranoside
-
Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.2.1.23 lysosome
-
Mus musculus 5764
-
3.2.1.46 lysosome
-
Mus musculus 5764
-
3.2.1.46 lysosome
-
Homo sapiens 5764
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.23 120000
-
subunit C, gel filtration chromatography Mus musculus
3.2.1.23 400000
-
subunit B, gel filtration chromatography Mus musculus
3.2.1.23 520000
-
subunit A, gel filtration chromatography Mus musculus
3.2.1.46 640000
-
gel filtration chromatography Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.23 Mus musculus
-
twi +/i C57BL6 mice
-
3.2.1.46 Homo sapiens
-
-
-
3.2.1.46 Mus musculus
-
twi +/i C57BL6 mice and wild-type mice
-

Purification (Commentary)

EC Number Purification (Comment) Organism
3.2.1.46
-
Mus musculus
3.2.1.46
-
Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.23 hematopoietic stem cell
-
Mus musculus
-
3.2.1.23 neural stem cell precursor cell Mus musculus
-
3.2.1.46 fibroblast
-
Homo sapiens
-
3.2.1.46 hematopoietic stem cell
-
Mus musculus
-
3.2.1.46 lymphocyte
-
Homo sapiens
-
3.2.1.46 neural stem cell precursor cell Mus musculus
-
3.2.1.46 neural stem cell precursor cell Homo sapiens
-
3.2.1.46 U-937 cell
-
Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.2.1.23 additional information
-
30.3 mU/mg, hematopoetic stem cells Mus musculus
3.2.1.23 additional information
-
8.75 mU/mg, neural presursor cells Mus musculus
3.2.1.46 additional information
-
0.14 mU/mg, from Krabbe fibroblasts Homo sapiens
3.2.1.46 additional information
-
0.31 mU/mg Mus musculus
3.2.1.46 additional information
-
0.31 mU/mg Homo sapiens
3.2.1.46 additional information
-
0.6 mU/mg for hematopoetic stem cells, after transduction with lentiviral GALC vector Mus musculus
3.2.1.46 additional information
-
0.65 mU/mg for hematopoetic stem cells from wild-type mice Mus musculus
3.2.1.46 additional information
-
0.884 mU/mg, from Krabbe lymphocytes Homo sapiens
3.2.1.46 additional information
-
1.65 mU/mg, neural presursor cells, after transduction with lentiviral GALC vectors Homo sapiens
3.2.1.46 additional information
-
2.33 mU/mg for neural precursor cells, after transduction with lentiviral GALC vector Mus musculus
3.2.1.46 additional information
-
3.2 mU/mg, monocytic U937 cells, after transduction with lentiviral GALC vector Homo sapiens
3.2.1.46 additional information
-
4.32 mU/mg, from healthy fibroblasts Homo sapiens
3.2.1.46 additional information
-
5.24 mU/mg, from healthy lymphocytes Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.23 4-methylumbelliferyl-beta-D-galactopyranoside + H2O pH 4.0, 30 min, 37°C Mus musculus 4-methylumbelliferone + beta-D-galactopyranose
-
?
3.2.1.46 4-methylumbelliferyl-beta-D-galactopyranoside + H2O pH 4.0, 30 min, 37°C Mus musculus 4-methylumbelliferone + beta-D-galactopyranose
-
?
3.2.1.46 4-methylumbelliferyl-beta-D-galactopyranoside + H2O pH 4.0, 30 min, 37°C Homo sapiens 4-methylumbelliferone + beta-D-galactopyranose
-
?

Synonyms

EC Number Synonyms Comment Organism
3.2.1.23 beta-gal
-
Mus musculus
3.2.1.23 beta-galactosidase
-
Mus musculus
3.2.1.46 beta-galactocerebrosidase
-
Mus musculus
3.2.1.46 beta-galactocerebrosidase
-
Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.2.1.46 37
-
pH 4.0 Mus musculus
3.2.1.46 37
-
pH 4.0 Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.2.1.46 4
-
37°C Mus musculus
3.2.1.46 4
-
37°C Homo sapiens

pH Range

EC Number pH Minimum pH Maximum Comment Organism
3.2.1.46 2.5 5.5
-
Mus musculus
3.2.1.46 2.5 5.5
-
Homo sapiens

Ki Value [mM]

EC Number Ki Value [mM] Ki Value maximum [mM] Inhibitor Comment Organism Structure
3.2.1.23 0.00012
-
AgNO3
-
Mus musculus