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MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3

alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc + H2O → alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc + 2 Man

Reactome: R-HSA-8853686
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcCHEBI:91280, C38H65NO31, lysosomal lumen
H2OCHEBI:15377, H2O, lysosomal lumen

Outputs

alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcCHEBI:91279, C26H45NO21, lysosomal lumen
2 ManCHEBI:4208, C6H12O6, lysosomal lumen

Catalysed by

alpha-mannosidase activity of MAN2B1:Zn2+ [lysosomal lumen]EC 3.2.1.24

Literature

Cloning, expression, purification, and characterization of the human broad specificity lysosomal acid alpha-mannosidase J. Biol. Chem., 1996
Purification and characterization of recombinant human lysosomal alpha-mannosidase Mol. Genet. Metab., 2001
Lysosomal alpha-D-mannosidase Biosci. Rep., 1999
amamutdb.no: A relational database for MAN2B1 allelic variants that compiles genotypes, clinical phenotypes, and biochemical and structural data of mutant MAN2B1 in α-mannosidosis Hum. Mutat., 2015
Alpha-mannosidosis - a review of genetic, clinical findings and options of treatment Pediatr Endocrinol Rev, 2014