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'', ''lysosomal beta A mannosidase'') is an enzyme with List of enzymes, systematic name ''beta-D-mannoside mannoh ... A deficiency is associated with mannosidosis. A family of mannosidases are also responsible for processing newly formed glycoproteins in the endoplasmic reticulum into mature glycoproteins containing highly heterogeneous complex-type glycans. References EC 3.2.1 {{3.2-enzyme-stub ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Alpha-Mannosidase
alpha-Mannosidase (, ''alpha-D-mannosidase'', ''p-nitrophenyl-alpha-mannosidase'', ''alpha-D-mannopyranosidase'', ''1,2-alpha-mannosidase'', ''1,2-alpha-D-mannosidase'', ''exo-alpha-mannosidase'') is an enzyme involved in the cleavage of the alpha form of mannose. Its systematic name is ''alpha-D-mannoside mannohydrolase''. Isozymes Humans express the following three alpha-mannosidase isozymes: Applications It can be utilized in experiments that determine the effects of the presence or absence of mannose on specific molecules, such as recombinant proteins that are used in vaccine development. Pathology A deficiency can lead to 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 186519 .... References External links GeneReviews/NCBI/NIH/UW entry on Alpha-Manno ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Mannose
Mannose is a sugar monomer of the aldohexose series of carbohydrates. It is a C-2 epimer of glucose. Mannose is important in human metabolism, especially in the glycosylation of certain proteins. Several congenital disorders of glycosylation are associated with mutations in enzymes involved in mannose metabolism. Mannose is not an essential nutrient; it can be produced in the human body from glucose, or converted into glucose. Mannose provides 2–5 kcal/g. It is partially excreted in the urine. Etymology The root of both "mannose" and "mannitol" is manna, which the Bible describes as the food supplied to the Israelites during their journey in the region of Sinai. Several trees and shrubs can produce a substance called manna, such as the "manna tree" ('' Fraxinus ornus'') from whose secretions mannitol was originally isolated. Structure Mannose commonly exists as two different-sized rings, the pyranose (six-membered) form and the furanose (five-membered) form. Eac ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Beta-Mannosidase
Beta-mannosidase (, ''mannanase'', ''mannase'', ''beta-D-mannosidase'', ''beta-mannoside mannohydrolase'', ''exo-beta-D-mannanase'', ''lysosomal beta A mannosidase'') is an enzyme with List of enzymes, systematic name ''beta-D-mannoside mannohydrolase'', which is in humans encoded by the ''MANBA'' gene. This enzyme catalysis, catalyses the following chemical reaction : Hydrolysis of terminal, non-reducing beta-D-mannose residues in beta-D-mannosides This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement. References Further reading * * * * * * * * * * * * * * * * * * * External links * * EC 3.2.1 {{gene-4-stub ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Mannosidosis
Mannosidosis is a deficiency in mannosidase, an enzyme. There are two types: * Alpha-mannosidosis * Beta-mannosidosis 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 ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |