Reactome | HEXA cleaves the terminal GalNAc from DS

HEXA cleaves the terminal GalNAc from DS

Stable Identifier

R-HSA-2105001

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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HEXA cleaves the terminal GalNAc from DS

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Beta-hexosaminidase (HEX) cleaves the terminal N-acetyl glucosamine (GlcNAc) from glucosaminoglycans (GAGs) and any other molecules containing a terminal GlcNAc. 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