Page last updated: 2024-10-24

beta-N-acetylhexosaminidase complex

Definition

Target type: cellularcomponent

A protein complex which is capable of beta-N-acetylhexosaminidase activity. [GO_REF:0000088, GOC:bhm, GOC:TermGenie, PMID:6458607]

Beta-N-acetylhexosaminidase, a lysosomal hydrolase, exists as a complex within the cellular compartment known as the lysosome. The lysosome is a membrane-bound organelle found in the cytoplasm of eukaryotic cells. This complex is a tetramer composed of two alpha and two beta subunits. The alpha subunit, encoded by HEXA, is responsible for catalytic activity. The beta subunit, encoded by HEXB, is a non-catalytic subunit that is essential for the stability and activity of the complex. This tetrameric complex localizes to the lysosome, where it plays a critical role in the breakdown of complex carbohydrates, specifically the degradation of the terminal N-acetyl-beta-D-glucosamine residues from various glycoproteins and glycolipids. These complex carbohydrates are transported to the lysosome via endocytosis and autophagy pathways, where they are then processed by the beta-N-acetylhexosaminidase complex.'
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Proteins (2)

ProteinDefinitionTaxonomy
Beta-hexosaminidase subunit betaA beta-hexosaminidase subunit beta that is encoded in the genome of human. [PRO:DNx, UniProtKB:P07686]Homo sapiens (human)
Beta-hexosaminidase subunit alphaA beta-hexosaminidase subunit alpha that is encoded in the genome of human. [PRO:DNx, UniProtKB:P06865]Homo sapiens (human)

Compounds (6)

CompoundDefinitionClassesRoles
pyrimethamineMaloprim: contains above 2 cpdsaminopyrimidine;
monochlorobenzenes
antimalarial;
antiprotozoal drug;
EC 1.5.1.3 (dihydrofolate reductase) inhibitor
naphthalimidesNaphthalimides: Compounds with three fused rings that appear like a naphthalene fused to piperidone or like a benz(de)isoquinoline-1,3-dione (not to be confused with BENZYLISOQUINOLINES which have a methyl separating the naphthyl from the benzyl rings). Members are CYTOTOXINS.
2-acetamido-1,5-imino-1,2,5-trideoxy-d-glucitol2-acetamido-1,5-imino-1,2,5-trideoxy-D-glucitol: structure given in first source
2-(2-oxolanylmethyl)benzo[de]isoquinoline-1,3-dioneisoquinolines
n-acetylglucosamine thiazolineN-acetylglucosamine thiazoline: an analog of the oxazolinium bicyclic intermediate leading from N-acetylglucosamine to 1,6-anhydro-N-acetylmuramic acid
thiamet g