Asparagine-linked glycosylation protein 11 alpha-1,2-mannosyltransferase (ALG11)

Asparagine-linked glycosylation protein 11 alpha-1,2-mannosyltransferase (ALG11) is an essential glycosyltransferase enzyme localized to the endoplasmic reticulum membrane, where it catalyzes the sequential addition of two alpha-1,2-mannose residues to the growing oligosaccharide chain (Man3GlcNAc2-PP-dolichol), producing Man5GlcNAc2-PP-dolichol, a key precursor in N-linked glycosylation[1][3][6]. This process is critical for proper folding, stability, and function of glycoproteins. Pathogenic mutations in the ALG11 gene result in congenital disorder of glycosylation type Ip (ALG11-CDG), characterized by multi-system involvement, notably neurological symptoms such as developmental delay and seizures[5]. No therapeutic agents directly act on ALG11, but its activity is a determinant in glycoprotein biosynthesis and an important biomarker in diagnosis of glycosylation disorders[1][3][5].

Other names

ALG11GDP-Man:Man(3)GlcNAc(2)-PP-Dol alpha-1,2-mannosyltransferaseAsparagine-linked glycosylation protein 11 homologGlycolipid 2-alpha-mannosyltransferaseCDG1PGT8GDP-Man:Man(3)GlcNAc(2)-PP-Dolichol alpha-1,2-mannosyltransferase

Not applicable: there are no known drugs directly targeting ALG11 enzyme function for therapeutic intervention