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
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3.2.1.46
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krabbe
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leukodystrophy
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lysosomal
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globoid
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twitcher
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psychosine
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demyelination
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myelin
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infantile
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galactosylsphingosine
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nerve
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neurodegenerative
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oligodendrocyte
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hematopoietic
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sphingolipids
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spastic
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arylsulfatase
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saposins
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metachromatic
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medicine
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presymptomatic
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schwann
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glycosphingolipids
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sphingomyelinase
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galactolipids
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gaucher
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gangliosidosis
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prosaposin
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later-onset
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drug development
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myelin-forming
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late-infantile
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gm1-gangliosidosis
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glucosylceramidase
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glucocerebrosidase
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gm1-ganglioside
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diagnostics
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4-methylumbelliferyl
- 3.2.1.46
- krabbe
- leukodystrophy
- lysosomal
-
globoid
-
twitcher
- psychosine
- demyelination
- myelin
-
infantile
- galactosylsphingosine
- nerve
- neurodegenerative
- oligodendrocyte
-
hematopoietic
- sphingolipids
-
spastic
- arylsulfatase
-
saposins
-
metachromatic
- medicine
-
presymptomatic
-
schwann
- glycosphingolipids
- sphingomyelinase
- galactolipids
- gaucher
- gangliosidosis
- prosaposin
-
later-onset
- drug development
-
myelin-forming
-
late-infantile
- gm1-gangliosidosis
- glucosylceramidase
- glucocerebrosidase
-
gm1-ganglioside
- diagnostics
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4-methylumbelliferyl
Reaction
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
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Specific Activity
Specific Activity on EC 3.2.1.46 - galactosylceramidase
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0.000008
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GALC enzyme activity in the posterior part of the brain of single intracerebroventricular-GALC injected mice
0.0000087
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GALC enzyme activity in the anterior part of the brain of single intracerebroventricular-GALC injected mice
120
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with plasmid encoding GALC-MH transfected 293T cell, GALC units per mg of cell extract
130
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with plasmid encoding unmodified GALC transfected 293T cell, GALC units per mg of cell extract
70
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with plasmid encoding GALC-TMH transfected 293T cell, GALC units per mg of cell extract
additional information
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electrospray ionization mass spectrometry for assay, detection limits are comparable to those from assays using radioactive labels
additional information
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1.65 mU/mg, neural presursor cells, after transduction with lentiviral GALC vectors
additional information
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3.2 mU/mg, monocytic U937 cells, after transduction with lentiviral GALC vector
additional information
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economical and fast histochemical way to distinguish neural cells expressing galactocerebrosidase from thoses that are deficient, using 5Br-3Cl-beta-galactopiranoside in the presence of taurodeoxycholic and oleic acids
additional information
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distribution of recombinant GALC activity in COS-7 cells: GALC-MH, 41.2% activity in cell extract, 58.8% released into cell culture medium, GALC-TMH, 27.5% to 72.5%
additional information
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0.6 mU/mg for hematopoetic stem cells, after transduction with lentiviral GALC vector
additional information
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0.65 mU/mg for hematopoetic stem cells from wild-type mice
additional information
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2.33 mU/mg for neural precursor cells, after transduction with lentiviral GALC vector