N-acetyl-beta-D-galactosaminidase activity
Definition
Target type: molecularfunction
Catalysis of the reaction: H2O + an N-acetyl-beta-D-galactosalaminyl-[glycan] = N-acetyl-beta-D-galactosamine + a glycan. [EC:3.2.1.52, GOC:pz]
N-acetyl-beta-D-galactosaminidase activity is a hydrolase enzyme activity that specifically cleaves the glycosidic bond between N-acetyl-beta-D-galactosamine and another molecule, such as a protein or carbohydrate. This enzymatic activity is essential for the breakdown of various complex carbohydrates and glycoproteins, playing a vital role in diverse cellular processes.
The enzyme catalyzes the hydrolysis of the terminal non-reducing N-acetyl-beta-D-galactosamine residue from substrates, releasing the free sugar and leaving the remaining molecule intact. This hydrolysis reaction is often crucial for the degradation of glycoproteins, glycolipids, and other complex carbohydrates.
N-acetyl-beta-D-galactosaminidase activity is involved in various biological processes, including:
* **Glycoprotein degradation:** By cleaving N-acetyl-beta-D-galactosamine residues from glycoproteins, this enzyme contributes to their degradation and recycling of sugars.
* **Lysosomal function:** N-acetyl-beta-D-galactosaminidase is found in lysosomes, organelles responsible for cellular waste degradation.
* **Cellular signaling:** N-acetyl-beta-D-galactosaminidase activity can influence cell signaling pathways by altering the composition of glycoconjugates on the cell surface.
* **Development and differentiation:** The enzyme has been implicated in developmental processes and cell differentiation, particularly in the nervous system.
Deficiencies in N-acetyl-beta-D-galactosaminidase activity can lead to various genetic disorders, such as:
* **Morquio syndrome:** A lysosomal storage disorder characterized by skeletal abnormalities, corneal clouding, and heart valve defects.
* **MPS VI (Maroteaux-Lamy syndrome):** A mucopolysaccharidosis characterized by skeletal deformities, hepatosplenomegaly, and corneal clouding.
These disorders highlight the critical role of N-acetyl-beta-D-galactosaminidase activity in maintaining proper cellular function and overall health.'
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Proteins (2)
| Protein | Definition | Taxonomy |
|---|---|---|
| Beta-hexosaminidase subunit beta | A beta-hexosaminidase subunit beta that is encoded in the genome of human. [PRO:DNx, UniProtKB:P07686] | Homo sapiens (human) |
| Beta-hexosaminidase subunit alpha | A beta-hexosaminidase subunit alpha that is encoded in the genome of human. [PRO:DNx, UniProtKB:P06865] | Homo sapiens (human) |
Compounds (6)
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| pyrimethamine | Maloprim: contains above 2 cpds | aminopyrimidine; monochlorobenzenes | antimalarial; antiprotozoal drug; EC 1.5.1.3 (dihydrofolate reductase) inhibitor |
| naphthalimides | Naphthalimides: 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-glucitol | 2-acetamido-1,5-imino-1,2,5-trideoxy-D-glucitol: structure given in first source | ||
| 2-(2-oxolanylmethyl)benzo[de]isoquinoline-1,3-dione | isoquinolines | ||
| n-acetylglucosamine thiazoline | N-acetylglucosamine thiazoline: an analog of the oxazolinium bicyclic intermediate leading from N-acetylglucosamine to 1,6-anhydro-N-acetylmuramic acid | ||
| thiamet g |