Function and Biology

Crystal structure of acid-beta-glucosidase at pH 5.5

Source organism: Homo sapiens

EC 3.2.1.46: Galactosylceramidase

Reaction catalysed:
D-galactosyl-N-acylsphingosine + H(2)O = D-galactose + N-acylsphingosine
Systematic name:
D-galactosyl-N-acylsphingosine galactohydrolase
Alternative Name(s):
  • Beta-galactocerebrosidase
  • Beta-galactosylceramidase
  • Ceramide galactosidase
  • Cerebroside beta-galactosidase
  • Cerebroside galactosidase
  • Galactocerebrosidase
  • Galactocerebroside beta-galactosidase
  • Galactocerebroside galactosidase
  • Galactocerebroside-beta-D-galactosidase
  • Galactocerebroside.beta-galactosidase
  • Galactosylceramidase I
  • Galactosylceramide beta-galactosidase
  • Galactosylceramide.beta-galactosidase
  • Galactosylcerebrosidase
  • Galcerase
  • Lactosylceramidase
  • Lactosylceramidase I

EC 3.2.1.45: Glucosylceramidase

Reaction catalysed:
D-glucosyl-N-acylsphingosine + H(2)O = D-glucose + N-acylsphingosine
Systematic name:
D-glucosyl-N-acylsphingosine glucohydrolase
Alternative Name(s):
  • Acid beta-glucosidase
  • Beta-D-glucocerebrosidase
  • Beta-glucocerebrosidase
  • Beta-glucosylceramidase
  • Ceramide glucosidase
  • GlcCer-beta-glucosidase
  • Glucocerebrosidase
  • Glucosphingosine glucosylhydrolase
  • Glucosylcerebrosidase
  • Glucosylsphingosine beta-D-glucosidase
  • Glucosylsphingosine beta-glucosidase
  • Psychosine hydrolase

GO terms

Biochemical function:
Biological process:
Cellular component:

Sequence families

Pfam Protein families (Pfam)
PF17189
Domain description: Glycosyl hydrolase family 30 beta sandwich domain
Occurring in:
  1. Lysosomal acid glucosylceramidase
The deposited structure of PDB entry 3gxi contains 4 copies of Pfam domain PF17189 (Glycosyl hydrolase family 30 beta sandwich domain) in Lysosomal acid glucosylceramidase. Showing 1 copy in chain A.

PF02055
Domain description: Glycosyl hydrolase family 30 TIM-barrel domain
Occurring in:
  1. Lysosomal acid glucosylceramidase
The deposited structure of PDB entry 3gxi contains 4 copies of Pfam domain PF02055 (Glycosyl hydrolase family 30 TIM-barrel domain) in Lysosomal acid glucosylceramidase. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR001139
Domain description: Glycoside hydrolase family 30
Occurring in:
  1. Lysosomal acid glucosylceramidase
IPR033452
Domain description: Glycosyl hydrolase family 30, beta sandwich domain
Occurring in:
  1. Lysosomal acid glucosylceramidase
IPR017853
Domain description: Glycoside hydrolase superfamily
Occurring in:
  1. Lysosomal acid glucosylceramidase
IPR033453
Domain description: Glycosyl hydrolase family 30, TIM-barrel domain
Occurring in:
  1. Lysosomal acid glucosylceramidase

Structure domains

CATH CATH domains
2.60.40.1180
Class: Mainly Beta
Architecture: Sandwich
Topology: Immunoglobulin-like
Homology: Golgi alpha-mannosidase II
Occurring in:
  1. Lysosomal acid glucosylceramidase
The deposited structure of PDB entry 3gxi contains 4 copies of CATH domain 2.60.40.1180 (Immunoglobulin-like) in Lysosomal acid glucosylceramidase. Showing 1 copy in chain A.
3.20.20.80
Class: Alpha Beta
Architecture: Alpha-Beta Barrel
Topology: TIM Barrel
Homology: Glycosidases
Occurring in:
  1. Lysosomal acid glucosylceramidase
The deposited structure of PDB entry 3gxi contains 4 copies of CATH domain 3.20.20.80 (TIM Barrel) in Lysosomal acid glucosylceramidase. Showing 1 copy in chain A.