Mannosidosis is a deficiency in
mannosidase Mannosidase is an enzyme which hydrolyses mannose.
There are two types:
* alpha-Mannosidase
* beta-Mannosidase
Beta-mannosidase (, ''mannanase'', ''mannase'', ''beta-D-mannosidase'', ''beta-mannoside mannohydrolase'', ''exo-beta-D-mannanase'' ...
, an
enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...
.
There are two types:
*
Alpha-mannosidosis
Alpha-mannosidosis is a lysosomal storage disorder, first described by Swedish physician Okerman in 1967.''Malm D, Nilssen O (2008). "Alpha-mannosidosis". Orphanet J Rare Dis. 3 (1): 21. doi:10.1186/1750-1172-3-21. PMC 2515294 . PMID 1865197 ...
*
Beta-mannosidosis
Beta-mannosidosis, also called lysosomal beta-mannosidase deficiency, is a disorder of oligosaccharide metabolism caused by decreased activity of the enzyme beta-mannosidase. This enzyme is coded for by the gene '' MANBA'', located at 4q22-25. Be ...
See also
*
Swainsonine
Swainsonine is an indolizidine alkaloid. It is a potent inhibitor of Golgi alpha-mannosidase II, an immunomodulator, and a potential chemotherapy drug. As a toxin in locoweed (likely its primary toxin) it also is a significant cause of economi ...
References
{{Glycoproteinoses
Glycoprotein metabolism disorders