beta-A-mannosidase, lysosomal (MANBA, MANB1)

Function

• exoglycosidase that cleaves the single beta-linked mannose residue from the non-reducing end of all N-linked glycoprotein oligosaccharides
• glycan metabolism; N-glycan degradation
• hydrolysis of terminal, non-reducing beta-D-mannose residues in beta-D-mannosides

Structure

belongs to the glycosyl hydrolase 2 family

Compartment

lysosome

Pathology

• defects in MANBA are the cause of
  • mild disorder affecting peripheral & CNS myelin
  • lysosomal beta-mannosidosis

More general terms

• beta-mannosidase
• glycoprotein

Database

• UniProt: http://www.uniprot.org/uniprot/O00462.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4126
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4126
• OMIM: https://mirror.omim.org/entry/248510
• OMIM: https://mirror.omim.org/entry/609489