Recombinant Human Endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase (MAN1B1), Truncated

Uniprot Id

Q9UKM7

Target Species

Human

Target Name

MAN1B1

Target Full Name

Endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase

Target Function

Involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man(9)GlcNAc(2) to produce Man(8)GlcNAc(2), but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man(5-6)GlcNAc(2).

Target Involvement

Mental retardation, autosomal recessive 15 (MRT15)

Target Subcellular Location

Endoplasmic reticulum membrane; Single-pass type II membrane protein.

Target Protein Families

Glycosyl hydrolase 47 family

Target Tissue Specificity

Widely expressed.

Target Synonyms

MAN1B1; UNQ747/PRO1477Endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase; EC 3.2.1.113; ER alpha-1,2-mannosidase; ER mannosidase 1; ERMan1; Man9GlcNAc2-specific-processing alpha-mannosidase; Mannosidase alpha class 1B member 1

Target Background

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.