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
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globoid
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twitcher
- psychosine
- demyelination
- myelin
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infantile
- galactosylsphingosine
- nerve
- neurodegenerative
- oligodendrocyte
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hematopoietic
- sphingolipids
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spastic
- arylsulfatase
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saposins
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metachromatic
- medicine
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presymptomatic
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schwann
- glycosphingolipids
- sphingomyelinase
- galactolipids
- gaucher
- gangliosidosis
- prosaposin
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later-onset
- drug development
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myelin-forming
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late-infantile
- gm1-gangliosidosis
- glucosylceramidase
- glucocerebrosidase
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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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Substrates Products
Substrates Products on EC 3.2.1.46 - galactosylceramidase
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REACTION DIAGRAM
4-methylumbelliferyl-beta-D-galactopyranoside + H2O
4-methylumbelliferone + beta-D-galactopyranose
6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside + H2O
6-hexadecanoylamino-4-methylumbelliferone + beta-D-galactopyranose
additional information
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the enzyme is required for normal sperm maturation and function, enzyme deficiency leads to degeneration of oligodendrocytes, severe demyelination, and causes sperm abnormalities in the mouse model of human globoid cell leukodystrophy or Krabbe disease, mutant twitcher mice show reduced size of testis and sperm acrosomal membrane which is redundant, detached from the nucleus and folded over
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methylumbelliferone + beta-D-galactose
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4-methylumbelliferyl beta-galactoside + H2O
methylumbelliferone + beta-D-galactose
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?
4-methylumbelliferone + beta-D-galactopyranose
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pH 4.0, 30 min, 37°C
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?
4-methylumbelliferyl-beta-D-galactopyranoside + H2O
4-methylumbelliferone + beta-D-galactopyranose
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pH 4.0, 30 min, 37°C
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?
6-hexadecanoylamino-4-methylumbelliferone + beta-D-galactopyranose
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?
6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside + H2O
6-hexadecanoylamino-4-methylumbelliferone + beta-D-galactopyranose
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fluorogenic substrate
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?
6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside + H2O
6-hexadecanoylamino-4-methylumbelliferone + beta-D-galactopyranose
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fluorogenic substrate
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?
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171506, 171507, 171508, 171509, 171510, 171511, 171512, 171514, 171515, 171516, 171517, 171518, 171519, 171522
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?
D-galactose + N-acylsphingosine
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171506, 171507, 171508, 171509, 171510, 171511, 171512, 171514, 171515, 171516, 171517, 171518, 171519, 171522
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?
D-galactosyl-N-acylsphingosine + H2O
D-galactose + N-acylsphingosine
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?
D-galactosyl-N-acylsphingosine + H2O
D-galactose + N-acylsphingosine
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?
D-galactosyl-N-acylsphingosine + H2O
D-galactose + N-acylsphingosine
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?
D-galactose + N-acylceramide
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171506, 171507, 171508, 171509, 171510, 171511, 171512, 171514, 171515, 171516, 171517, 171518, 171519, 171522
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?
D-galactose + N-acylceramide
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?