ALG3
Description
The ALG3 (ALG3 alpha-1,3- mannosyltransferase) is a protein-coding gene located on chromosome 3.
ALG3 (Dolichyl-P-Man:Man(5)GlcNAc(2)-PP-dolichyl mannosyltransferase) is a human gene that encodes an enzyme crucial for proper N-glycosylation. This enzyme adds the first mannose derived from dolichyl-beta-D-mannosyl phosphate to the growing oligosaccharide chain in an alpha-1,3 linkage to Man(5)GlcNAc(2)-PP-dolichol. Defects in this gene are associated with congenital disorder of glycosylation type Id (CDG-Id), a condition characterized by abnormal N-glycosylation.
ALG3 catalyzes the addition of the first dolichyl-beta-D-mannosyl phosphate derived mannose in an alpha-1,3 linkage to Man(5)GlcNAc(2)-PP-dolichol, producing Man(6)GlcNAc(2)-PP-dolichol.
ALG3 is also known as CDG1D, CDGS4, CDGS6, D16Ertd36e, NOT56L, Not56, not.
