HEXA cleaves the terminal GalNAc from small HA fragments

Stable Identifier
R-HSA-2162225
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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

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

Biochemistry 1988
Participants
Participates
Catalyst Activity

beta-N-acetylhexosaminidase activity of HEXA [lysosomal lumen]

Orthologous Events
Authored
Reviewed
Created
Cite Us!