• There are two types: alpha-Mannosidase beta-Mannosidase A deficiency is associated with mannosidosis. (wikipedia.org)
  • Årsaken til betamannosidose er mutasjoner i genet MANBA, som koder for enzymet lysosomal beta-mannosidase A (også kalt mannase, 3,4). (frambu.no)
  • Lysosomal alpha-mannosidase with acidic pH optimum is ubiquitous in human tissues where is expressed in two major forms, A and B that are the product of a single gene located on chromosome 19. (nih.gov)
  • LAMZEDE is recombinant human lysosomal alpha-mannosidase indicated for the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients. (nih.gov)
  • the corresponding enzymes are, respectively, a putative lysosomal alpha-mannosidase and a Co(II)-activated alpha-mannosidase. (boku.ac.at)
  • Far less well understood is the observation that many bacterial species, predominantly but not exclusively pathogens and symbionts, also possess putative GH38 alpha-mannosidases whose activity and specificity is unknown. (rcsb.org)
  • Årsaken til alfamannosidose er arvelige forandringer (mutasjoner) i genet MAN2B1, som koder for enzymet lysosomal alfa-mannosidase (LAMAN). (frambu.no)
  • Mutations in the gene encoding for alpha-mannosidase cause alpha- mannosidosis, an autosomal recessive disease, resulting in the accumulation of unprocessed mannose containing oligosaccharide material. (nih.gov)
  • 2006): A deletion in the golgi alpha-mannosidase II gene of Caenorhabditis elegans results in unexpected non-wild-type N-glycan structures. (boku.ac.at)
  • Tolerant patients displayed an expansion of peripheral blood B and NK lymphocytes, fewer activated CD4+ T cells, a lack of donor-specific antibodies, donor-specific hyporesponsiveness of CD4+ T cells, and a high ratio of forkhead box P3 to α-1,2-mannosidase gene expression. (jci.org)
  • 11. A novel ER alpha-mannosidase-like protein accelerates ER-associated degradation. (nih.gov)
  • 13. ER-resident protein 46 (ERp46) triggers the mannose-trimming activity of ER degradation-enhancing α-mannosidase-like protein 3 (EDEM3). (nih.gov)
  • The most extensively studied of these enzymes is the Drosophila GH38 alpha-mannosidase II, which has been shown to be a retaining alpha-mannosidase that targets both alpha-1,3 and alpha-1,6 mannosyl linkages, an activity that enables the enzyme to process GlcNAc(Man)(5)(GlcNAc)(2) hybrid N-glycans to GlcNAc(Man)(3)(GlcNAc)(2). (rcsb.org)
  • β-Mannosidase had maximum activity at 55 °C but this dropped significantly at 70 °C. Specific cleavage of β-mannoside linkages by the purified β-mannosidase was demonstrated using a β-(1-4)mannosylmannose as substrate. (utmb.edu)
  • 19. EDEM1's mannosidase-like domain binds ERAD client proteins in a redox-sensitive manner and possesses catalytic activity. (nih.gov)
  • The analysis of the N-glycan structures of an aman- 2 mutant strain demonstrates that the absence of alpha-mannosidase II activity results in a shift to structures not seen in wild-type worms (e. g. (boku.ac.at)
  • Paucimannosidic glycans are almost absent from aman-2 worms, indicative also of a general lack of alpha-mannosidase III activity. (boku.ac.at)
  • β-Mannosidase (β-mannoside mannohydrolase, EC 3.2.1.25) was extracted and purified about 100-fold from malted barley. (utmb.edu)
  • Mannosidase is an enzyme which hydrolyses mannose. (wikipedia.org)
  • After the transfer of a GlcNAc residue to Asn- bound Man(5)GlcNAc(2) by N-acetylglucosaminyl-transferase I, an alpha-mannosidase (EC 3.2.1.114) removes one alpha 1,3- linked and one alpha 1,6- linked mannose residue. (boku.ac.at)
  • Mitchell, Earl D. / Purification and properties of β-mannosidase from malted barley . (utmb.edu)
  • This is usually accomplished by a complex system of mannosidases in which those from class I have an outstanding role, commonly involved in the early. (researchgate.net)
  • 17. Family 47 alpha-mannosidases in N-glycan processing. (nih.gov)
  • The purified preparation was free of α-mannosidase, β-N-acetylhexosaminidase, α-galactosidase and β-glucosidase. (utmb.edu)
  • Misfolded glycoproteins are deglycosylated in the cytosol and the resultant free oligosaccharide can be further hydrolysed by alpha-mannosidase 2C1 (MAN2C1). (reactome.org)
  • 16. Trimming of glucosylated N-glycans by human ER α1,2-mannosidase I. (nih.gov)
  • Conclusions: Our results suggest that some genes previously identified as influencing lung function are independently associated with emphysema rather than lung function, and that genes related to a-mannosidase may influence risk of emphysema. (cdc.gov)