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Details on Person UniProt:Q92685 ALG3
| Class:Id | ReferenceGeneProduct:49882 |
|---|---|
| _chainChangeLog | chain:1-438 added on Sat February 7 2015 |
| _displayName | UniProt:Q92685 ALG3 |
| _timestamp | 2025-08-15 22:06:02 |
| chain | chain:1-438 |
| checksum | 687FC8E4A588FD9C |
| comment | FUNCTION 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. |
| description | recommendedName: 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 |
| geneName | ALG3 NOT NOT56L |
| identifier | Q92685 |
| isSequenceChanged | FALSE |
| keyword | Alternative 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 |
| name | ALG3 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8999902] ENSEMBL:ENSG00000214160 ALG3 [Homo sapiens] |
| secondaryIdentifier | ALG3_HUMAN A8JZZ6 Q9BT71 |
| sequenceLength | 438 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:8969529] UniProt:Q92685-1 ALG3 [Homo sapiens] [ReferenceIsoform:8969530] UniProt:Q92685-2 ALG3 [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:449287] ALG3 [endoplasmic reticulum membrane] [Homo sapiens] [EntityWithAccessionedSequence:4720466] ALG3 W71R [endoplasmic reticulum membrane] [Homo sapiens] [EntityWithAccessionedSequence:4720468] ALG3 G118D [endoplasmic reticulum membrane] [Homo sapiens] [EntityWithAccessionedSequence:4720470] ALG3 R171Q [endoplasmic reticulum membrane] [Homo sapiens] [EntityWithAccessionedSequence:4720484] ALG3 M157K [endoplasmic reticulum membrane] [Homo sapiens] |
| (referenceSequence) | [ReplacedResidue:4720459] glycine 118 replaced with L-aspartic acid [ReplacedResidue:4720469] L-tryptophan 71 replaced with L-arginine [ReplacedResidue:4720471] L-methionine 157 replaced with L-lysine [ReplacedResidue:4720479] L-arginine 171 replaced with L-glutamine |
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No pathways have been reviewed or authored by UniProt:Q92685 ALG3 (49882)
