| Type: | Family | Name: | Beta-hexosaminidase |
| Description: | This entry represents beta-hexosaminidase (). 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 [].Beta-hexosaminidase belongs to the glycoside hydrolase family 20 (). | Short Name: | Beta_hexosaminidase_sua/sub |
