Proteins/UNIPROT:Q9H553

ALG2

ProteinUNIPROT:Q9H553

Mannosyltransferase that operates in the biosynthetic pathway of dolichol-linked oligosaccharides, the glycan precursors employed in protein asparagine (N)-glycosylation. The assembly of dolichol-linked oligosaccharides begins on the cytosolic side of the endoplasmic reticulum membrane and finishes in its lumen. The sequential addition of sugars to dolichol pyrophosphate produces dolichol-linked oligosaccharides containing fourteen sugars, including two GlcNAcs, nine mannoses and three glucoses. Once assembled, the oligosaccharide is transferred from the lipid to nascent proteins by oligosaccharyltransferases. Catalyzes, on the cytoplasmic face of the endoplasmic reticulum, the addition of the second and third mannose residues to the dolichol-linked oligosaccharide chain, to produce Man3GlcNAc(2)-PP-dolichol core oligosaccharide. Man3GlcNAc(2)-PP-dolichol is a substrate for ALG11, the following enzyme in the biosynthetic pathway. While both alpha 1,3 and alpha 1,6 linkages are possible, the sequential addition of alpha 1,3 followed by alpha 1,6 is probably the preferred route..

Function from UniProtKB (Q9H553), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

ALG2-congenital disorder of glycosylation has a germline basis in ALG2DiseaseGenCC HGNC:23159|MONDO:0011933Inferred only
congenital myasthenic syndrome 14 has a germline basis in ALG2DiseaseGenCC HGNC:23159|MONDO:0014543Inferred only

Statements

23 statements

23 statements · 3 reference databases · Subject: ALG2 · Sources: Hetionet, Reactome, WikiPathways

Order

ANXA11 interacts with ALG2ProteinHetionet hetionet:interacts:311-85365Inferred only
ANXA7 interacts with ALG2ProteinHetionet hetionet:interacts:310-85365Inferred only
PDCD6IP interacts with ALG2ProteinHetionet hetionet:interacts:10015-85365Inferred only
PLSCR3 interacts with ALG2ProteinHetionet hetionet:interacts:57048-85365Inferred only
PTPN23 interacts with ALG2ProteinHetionet hetionet:interacts:25930-85365Inferred only
SHISA5 interacts with ALG2ProteinHetionet hetionet:interacts:51246-85365Inferred only
SRI interacts with ALG2ProteinHetionet hetionet:interacts:6717-85365Inferred only
TSG101 interacts with ALG2ProteinHetionet hetionet:interacts:7251-85365Inferred only
UBC interacts with ALG2ProteinHetionet hetionet:interacts:7316-85365Inferred only
ALG2 participates in Asparagine N-linked glycosylationPathwayReactome R-HSA-446203Inferred only
ALG2 participates in Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent proteinPathwayReactome R-HSA-446193Inferred only
ALG2 participates in Defective ALG2 causes CDG-1iPathwayReactome R-HSA-4549349Inferred only

Show the remaining 11

ALG2 participates in DiseasePathwayReactome R-HSA-1643685Inferred only
ALG2 participates in Diseases associated with N-glycosylation of proteinsPathwayReactome R-HSA-3781860Inferred only
ALG2 participates in Diseases of glycosylationPathwayReactome R-HSA-3781865Inferred only
ALG2 participates in Diseases of metabolismPathwayReactome R-HSA-5668914Inferred only
ALG2 participates in Glycosylation and related congenital defectsPathwayWikiPathways WP4521Inferred only
ALG2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
ALG2 participates in N glycan biosynthesisPathwayWikiPathways WP5153Inferred only
ALG2 participates in Post-translational protein modificationPathwayReactome R-HSA-597592Inferred only
ALG2 increases the expression of ADRB2ProteinHetionet hetionet:regulates:85365-154Inferred only
ALG2 increases the expression of CPVLProteinHetionet hetionet:regulates:85365-54504Inferred only
ALG2 decreases the expression of NPTX2ProteinHetionet hetionet:regulates:85365-4885Inferred only

Reactions

6 reactions

MAM01390c+MAM01951cMAM01323c+GDP+proton
Catalysed by ALG2
Reaction pageLeft to rightBalancedEC 2.4.1.132cytosol
Human-GEM MAR07267CC BY 4.0Reference data
MAM01323c+MAM01951cMAM01865c+GDP+proton
Catalysed by ALG2
Reaction pageLeft to rightBalancedEC 2.4.1.257cytosol
Human-GEM MAR07268

Other names

4 names

Resolves to: ALG2

Also called: Asparagine-linked glycosylation protein 2 homologGDP-Man:Man(1)GlcNAc(2)-PP-Dol alpha-1,3-mannosyltransferaseGDP-Man:Man(1)GlcNAc(2)-PP-dolichol mannosyltransferaseGDP-Man:Man(2)GlcNAc(2)-PP-Dol alpha-1,6-mannosyltransferase

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