Reactome | ALG3 transfers Man to N-glycan precursor (GlcNAc)2 (Man)5 (PP-Dol)1

ALG3 transfers Man to N-glycan precursor (GlcNAc)2 (Man)5 (PP-Dol)1

Stable Identifier

R-HSA-446188

Type

Reaction [transition]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen

Synonyms

Addition of the sixth mannose to the N-glycan precursor by ALG3

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

ALG3 transfers Man to N-glycan precursor (GlcNAc)2 (Man)5 (PP-Dol)1

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

The sixth mannose is added to the N-glycan precursor. This reaction occurs in the ER lumen and uses a different mannose donor (dolichyl-phosphate-mannose) than the previous steps. It has been proposed that ALG3, along with all the mannosyl- and glucosyltransferases in the N-glycan biosynthesis pathway that use dolichyl-phosphate-mannose or dolichyl-phosphate-glucose as donor, derive from duplications of a common ancestral enzyme (Oriol et al. 2002). Defects in ALG3 are associated with Congenital Disorder of Glycosylation 1D (CDG1D) (Sun et al. 2005).

Literature References

PubMed ID	Title	Journal	Year
12200473	Common origin and evolution of glycosyltransferases using Dol-P-monosaccharides as donor substrate Mollicone, R, Codogno, P, Oriol, R, Chantret, I, Martinez-Duncker, I	Mol Biol Evol	2002
15840742	Congenital disorder of glycosylation id presenting with hyperinsulinemic hypoglycemia and islet cell hyperplasia Cohen, J, Chung, WK, Freeze, HH, Wang, C, Eklund, EA, Sun, L	J Clin Endocrinol Metab	2005