Reactome | HEXB cleaves the terminal GalNAc from keratan sulfate

HEXB cleaves the terminal GalNAc from keratan sulfate

Stable Identifier

R-HSA-9638078

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

HEXB cleaves the terminal GalNAc from keratan sulfate

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