Alpha-1,3-mannosyltransferase (ALG3) (ALG3)

Alpha-1,3-mannosyltransferase (ALG3) is an enzyme located in the endoplasmic reticulum membrane, where it plays an essential role in protein N-glycosylation by transferring the sixth of nine mannose residues from dolichol-phosphate mannose to the lipid-linked oligosaccharide precursor. This step is crucial for the correct assembly of the dolichol-linked oligosaccharide, which is later transferred en bloc to nascent proteins. Mutations in the ALG3 gene cause congenital disorder of glycosylation type Id (ALG3-CDG), an autosomal recessive multisystem disorder marked by neurodevelopmental delay, dysmorphic features, visual impairment, and currently without specific treatment. Diagnosis can be supported by specific glycoform and oligosaccharide biomarker patterns, but is confirmed genetically[1][2][3].

Other names

Dolichyl-P-Man:Man(5)GlcNAc(2)-PP-dolichyl mannosyltransferaseDol-P-Man:Man(5)GlcNAc(2)-PP-Dol alpha-1,3-mannosyltransferaseAsparagine-linked glycosylation protein 3 homologNot56-like proteinNOT56LNot56CDGS4D16Ertd36eCarbohydrate deficient glycoprotein syndrome type IVDolichyl-phosphate-mannose--glycolipid alpha-mannosyltransferaseCDG1DCDGS6NOTnotMannosyltransferase 6 deficiencyAsparagine-linked glycosylation 3 homolog (S. cerevisiae, alpha-1,3-mannosyltransferase)EC 2.4.1.258EC 2.4.1.130