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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
a) mild disorder affecting peripheral & CNS myelin
b) lysosomal beta-mannosidosis
General
beta-mannosidase
glycoprotein
Properties
SIZE: entity length = 879 aa
MW = 101 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-17}
N-glycosylation site {N28}
N-glycosylation site {N35}
N-glycosylation site {N77}
N-glycosylation site {N280}
N-glycosylation site {N284}
N-glycosylation site {N297}
N-glycosylation site {N302}
glutamate residue {E457}
N-glycosylation site {N763}
Database Correlations
OMIM correlations
UniProt O00462
Pfam PF02836
Entrez Gene 4126
Kegg hsa:4126
ENZYME 3.2.1.25
References
- UniProt :accession O00462
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=MANBA