mannozidáz

Mannozidase is an enzyme that catalyzes the hydrolysis of mannose residues from glycoproteins and other mannosylated molecules. It belongs to the glycoside hydrolase family. Mannosidases play a crucial role in various biological processes, including protein folding, quality control, and cellular signaling. There are several types of mannosidases, distinguished by their substrate specificity and the type of glycosidic bond they cleave. For instance, alpha-mannosidase cleaves alpha-mannose linkages, while beta-mannosidase acts on beta-mannose linkages.

In humans and other mammals, mannosidases are involved in the lysosomal degradation of glycoproteins. Defects in these enzymes can lead to a group of rare genetic disorders known as lysosomal storage diseases, specifically mannosidosis. Mannosidosis is characterized by the accumulation of mannose-rich oligosaccharides in various tissues, leading to a range of symptoms including intellectual disability, coarse facial features, and hearing loss. Research into mannosidase function is ongoing, aiming to understand its role in health and disease and to explore potential therapeutic strategies for mannosidosis and other related conditions.