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Details on Person UniProt:Q92685-2 ALG3

Class:IdReferenceIsoform:8969530
_chainChangeLogchain:1-438 added on Fri February 17 2017
_displayNameUniProt:Q92685-2 ALG3
_timestamp2025-08-15 22:06:03
chainchain:1-438
checksum687FC8E4A588FD9C
commentFUNCTION Dol-P-Man:Man(5)GlcNAc(2)-PP-Dol alpha-1,3-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. In the lumen of the endoplasmic reticulum, adds the first dolichyl beta-D-mannosyl phosphate derived mannose in an alpha-1,3 linkage to Man(5)GlcNAc(2)-PP-dolichol to produce Man(6)GlcNAc(2)-PP-dolichol (PubMed:10581255). Man(6)GlcNAc(2)-PP-dolichol is a substrate for ALG9, the following enzyme in the biosynthetic pathway (PubMed:10581255).CATALYTIC ACTIVITY an alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-alpha-D-GlcNAc-diphospho-di-trans,poly-cis-dolichol + a di-trans,poly-cis-dolichyl beta-D-mannosyl phosphate = an alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-alpha-D-GlcNAc-diphospho-di-trans,poly-cis-dolichol + a di-trans,poly-cis-dolichyl phosphate + H(+)PATHWAY Protein modification; protein glycosylation.INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the glycosyltransferase ALG3 family.
created[InstanceEdit:8964659] Weiser, JD
descriptionrecommendedName: fullName evidence="6"Dol-P-Man:Man(5)GlcNAc(2)-PP-Dol alpha-1,3-mannosyltransferase ecNumber evidence="2"2.4.1.258 alternativeName: fullName evidence="8"Asparagine-linked glycosylation protein 3 homolog alternativeName: Dol-P-Man-dependent alpha(1-3)-mannosyltransferase alternativeName: Dolichyl-P-Man:Man(5)GlcNAc(2)-PP-dolichyl mannosyltransferase alternativeName: Dolichyl-phosphate-mannose--glycolipid alpha-mannosyltransferase alternativeName: fullName evidence="7"Not56-like protein
geneNameALG3
NOT
NOT56L
identifierQ92685
isoformParent
isSequenceChangedFALSE
keywordAlternative splicing
Congenital disorder of glycosylation
Disease variant
Endoplasmic reticulum
Glycosyltransferase
Membrane
Phosphoprotein
Proteomics identification
Reference proteome
Transferase
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameALG3
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8999902] ENSEMBL:ENSG00000214160 ALG3 [Homo sapiens]
secondaryIdentifierALG3_HUMAN
A8JZZ6
Q9BT71
sequenceLength438
species[Species:48887] Homo sapiens
variantIdentifierQ92685-2
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No pathways have been reviewed or authored by UniProt:Q92685-2 ALG3 (8969530)