Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase (ALG8)

Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase (ALG8) is a membrane-bound glycosyltransferase enzyme localized in the endoplasmic reticulum. It catalyzes the addition of the second glucose residue from dolichyl-phosphate-glucose to the growing lipid-linked oligosaccharide precursor during the N-linked glycosylation of proteins. This step is essential for the proper folding and maturation of glycoproteins. Mutations in ALG8 disrupt glycan assembly, leading to congenital disorder of glycosylation type Ih, characterized by a spectrum of developmental, neurologic, hepatic, and coagulation abnormalities. ALG8 also plays a role in the maturation and localization of PKD1/Polycystin-1 and has been implicated in polycystic liver disease. No approved drugs are known to specifically target ALG8. It is considered a clinically relevant enzyme in human glycoprotein biosynthesis[1][2][3][4].

Other names

ALG8 alpha-1,3-glucosyltransferaseDolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferaseDolichyl-P-Glc:Glc1Man9GlcNAc2-PP-dolichyl glucosyltransferaseAsparagine-linked glycosylation protein 8 homologMGC2840HUSSY-02CDG1HPCLD3Asparagine-linked glycosylation 8 homolog (S. cerevisiae, alpha-1,3-glucosyltransferase)EC 2.4.1.265

Inhibition of glycosyltransferase activity (theoretical; no specific drugs currently identified)