LMPD Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
LMP000141

UniProt Annotations

Entry Information
Gene Namehexosaminidase A
Protein EntryHEXA_MOUSE
UniProt IDP29416
SpeciesMouse
Comments
Comment typeDescription
Catalytic ActivityHydrolysis of terminal non-reducing N-acetyl- D-hexosamine residues in N-acetyl-beta-D-hexosaminides.
FunctionResponsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.
SimilarityBelongs to the glycosyl hydrolase 20 family. {ECO:0000305}.
Subcellular LocationLysosome.
SubunitThere are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer composed of one alpha chain, one beta-A chain and one beta-B chain; hexosaminidase B is a tetramer of two beta-A and two beta-B chains; hexosaminidase S is a homodimer of two alpha chains. The two beta chains are derived from the cleavage of a precursor chain (By similarity). {ECO:0000250}.
Tissue SpecificityUbiquitous. Most abundant in testis, adrenal, epididymis and heart. Low levels seen in the liver. {ECO:0000269|PubMed:7896264}.