MAN1B1 Rabbit Polyclonal Antibody

This gene encodes an enzyme belonging to the glycosyl hydrolase 47 family. This enzyme functions in N-glycan biosynthesis, and is a class I alpha-1,2-mannosidase that specifically converts Man9GlcNAc to Man8GlcNAc isomer B. It is required for N-glycan trimming to Man5-6GlcNAc2 in the endoplasmic-reticulum-associated degradation pathway. Mutations in this gene cause autosomal-recessive intellectual disability. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 11. [provided by RefSeq, Dec 2011], catalytic activity:Hydrolysis of the terminal (1->2) -linked alpha-D-mannose residues in the oligo-mannose oligosaccharide Man (9) (GlcNAc) (2) ., cofactor:Calcium., enzyme regulation:Inhibited by both 1-deoxymannojirimycin and kifunensine., function:Involved in the maturation of Asn-linked oligosaccharides. Trim a single alpha-1,2-linked mannose residue from Man (9) GlcNAc (2) to produce Man (8) GlcNAc (2) . The only product is the Man (8) GlcNAc (2) isomer B, the form lacking the middle-arm terminal alpha 1,2-mannose. It may be involved in glycoprotein quality control since it is important to target misfolded glycoproteins for degradation., pathway:Protein modification; protein glycosylation., similarity:Belongs to the glycosyl hydrolase 47 family., tissue specificity:Widely expressed.

MAN1B1; Endoplasmic reticulum mannosyl-oligosaccharide 1; 2-alpha-mannosidase; ER alpha-1,2-mannosidase; ER mannosidase 1; ERMan1; Man9GlcNAc2-specific-processing alpha-mannosidase; Mannosidase alpha class 1B member 1