HEXA cleaves the terminal GalNAc from keratan sulfate

Stable Identifier
R-HSA-1638053
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Beta-hexosaminidase (HEX) cleaves the terminal N-acetyl galactosamine (GalNAc) from glucosaminoglycans (GAGs) and any other molecules containing a terminal GalNAc. There are two forms of HEX: HEXA and B. The A form is a trimer of the subunits alpha, beta A and beta B. The B form is a tetramer of 2 beta A and 2 beta B subunits (O'Dowd et al. 1988). Defects in the two subunits cause lysosomal storage diseases marked by the accumulation of GM2 gangliosides in neuronal cells.

Literature References
PubMed ID Title Journal Year
2971395 Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase

O'Dowd, BF, Cumming, DA, Gravel, RA, Mahuran, D

Biochemistry 1988
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
beta-N-acetylhexosaminidase activity of HEXA [lysosomal lumen]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created