Beta-hexosaminidase (IPR025705)

Short name: Beta_hexosaminidase_sua/sub

Overlapping homologous superfamilies

Family relationships



This entry represents beta-hexosaminidase (EC: There are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer, with one alpha, one beta-A and one beta-B chain; hexosaminidase B is a tetramer of two beta-A and two beta-B chains; and hexosaminidase S is a homodimer of alpha chains. The two beta chains are derived from the cleavage of a precursor.

In the brain and other tissues, beta-hexosaminidase A degrades GM2 gangliosides; specifically, the enzyme hydrolyses terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. Mutations in the beta-hexosaminidase beta-chain lead to Sandhoff disease, a lysosomal storage disorder characterised by accumulation of GM2 ganglioside [PMID: 8357844].

Beta-hexosaminidase belongs to the glycoside hydrolase family 20 (GH20).

GO terms

Biological Process

GO:0005975 carbohydrate metabolic process

Molecular Function

GO:0004563 beta-N-acetylhexosaminidase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.