MAN1B1 Protein, Human, Recombinant (His)

Endoplasmic Reticulum Mannosyl-Oligosaccharide 1,2-α-Mannosidase (MAN1B1) belongs to the glycosyl hydrolase 47 family. MAB1B1 is a single-pass type II membrane protein and widely expressed in many tissues. MAB1B1 is involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. MAB1B1 can be inhibited by both 1-deoxymannojirimycin (dMNJ) and kifunensine. Defects in MAN1B1 are the cause of mental retardation autosomal recessive type 15 (MRT15) . Mental retardation is characterized by significantly below average general intellectual functioning, it is also associated with impairments in adaptative behavior and manifested during the developmental period.