ENTRY EC 3.2.1.52
NAME beta-N-Acetylhexosaminidase
Hexosaminidase
CLASS Hydrolases
Glycosidases
Hydrolysing O-glycosyl compounds
SYSNAME beta-N-Acetyl-D-hexosaminide N-acetylhexosaminohydrolase
REACTION Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine
residues in N-acetyl-beta-D-hexosaminides
SUBSTRATE N-Acetyl-beta-D-hexosaminide
H2O
N-Acetyl-D-glucosaminide
N-Acetyl-D-galactosaminide
PRODUCT N-Acetyl-D-hexosamine
N-Acetyl-D-glucosamine
N-Acetyl-D-galactosamine
COMMENT Acts on N-acetylglucosides and N-acetylgalactosides.
PATHWAY PATH: MAP00530 Aminosugars metabolism
PATH: MAP00600 Sphingoglycolipid metabolism
DISEASE MIM: 268800 Hexosaminidase B (beta polypeptide); Sandhoff disease,
infantile, juvenile, and adult forms (3); Spinal
muscular atrophy, HEXB-related (3)
MIM: 272800 Hexosaminidase A (alpha polypeptide); Tay-Sachs
disease (3); GM2-gangliosidosis, juvenile, adult (3);
[Hex A pseudodeficiency] (1)
MOTIF PS: PS00013 {DERK}(6)-[LIVMFWSTAG](2)-[LIVMFYSTAGCQ]-[AGS]-C
GENES HSA: HEXA HEXB(Hs.51043)
STRUCTURES PDB: 1QBB 1QBA
DBLINKS University of Geneva ENZYME DATA BANK: 3.2.1.52
WIT (What Is There) Metabolic Reconstruction: 3.2.1.52
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