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Details on Person UniProt:Q9NP73 ALG13

Class:IdReferenceGeneProduct:218399
_chainChangeLogchain:1-1137 added on Fri February 6 2015
_displayNameUniProt:Q9NP73 ALG13
_timestamp2025-02-21 18:54:06
chainchain:1-1137
checksum4E56437BA2609589
commentFUNCTION Catalytic subunit of the UDP-N-acetylglucosamine transferase complex 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. On the cytoplasmic face of the endoplasmic reticulum, the dimeric ALG13/ALG14 complex catalyzes the second step of dolichol pyrophosphate biosynthesis, transferring a beta1,4-linked N-acetylglucosamine (GlcNAc) from UDP-GlcNAc to GlcNAc-pyrophosphatedolichol (Gn-PDol) to produce N,N'-diacetylchitobiosyl diphosphodolichol. N,N'-diacetylchitobiosyl diphosphodolichol is a substrate for ALG1, the following enzyme in the biosynthetic pathway.FUNCTION Catalytic subunit of the UDP-N-acetylglucosamine transferase complex 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. On the cytoplasmic face of the endoplasmic reticulum, the dimeric ALG13/ALG14 complex catalyzes the second step of dolichol pyrophosphate biosynthesis, transferring a beta1,4-linked N-acetylglucosamine (GlcNAc) from UDP-GlcNAc to GlcNAc-pyrophosphatedolichol (Gn-PDol) to produce N,N'-diacetylchitobiosyl diphosphodolichol. N,N'-diacetylchitobiosyl diphosphodolichol is a substrate for ALG1, the following enzyme in the biosynthetic pathway.FUNCTION No glycosyltransferase or deubiquitinase activity is detected for this potential multifunctional enzyme.CATALYTIC ACTIVITY an N-acetyl-alpha-D-glucosaminyl-diphospho-di-trans,poly-cis-dolichol + UDP-N-acetyl-alpha-D-glucosamine = an N,N'-diacetylchitobiosyl-diphospho-di-trans,poly-cis-dolichol + UDP + H(+)BIOPHYSICOCHEMICAL PROPERTIES Optimum pH is 7.0 (at 37 degrees Celsius).PATHWAY Protein modification; protein glycosylation.SUBUNIT Forms with ALG14 the active heterodimeric UDP-N-acetylglucosamine transferase complex.SUBUNIT Not able to interact with ALG14 to form an active UDP-N-acetylglucosamine transferase complex.INTERACTION Recruited to the cytosolic face of the endoplasmic reticulum membrane through its interaction with ALG14.ALTERNATIVE PRODUCTS The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the glycosyltransferase 28 family.CAUTION Contains 1 OTU domain with intact active sites. However, no deubiquitinase activity is detected in vitro with this domain compared to other isolated OTU domains.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated C-terminus.
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: fullName evidence="19"UDP-N-acetylglucosamine transferase subunit ALG13 ecNumber evidence="6 13"2.4.1.141 alternativeName: fullName evidence="20"Asparagine-linked glycosylation 13 homolog alternativeName: fullName evidence="20"Glycosyltransferase 28 domain-containing protein 1
geneNameALG13
CXorf45
GLT28D1
MDS031
identifierQ9NP73
isSequenceChangedFALSE
keywordAlternative splicing
Congenital disorder of glycosylation
Disease variant
Endoplasmic reticulum
Epilepsy
Glycosyltransferase
Hydrolase
Membrane
Multifunctional enzyme
Protease
Proteomics identification
Reference proteome
Thiol protease
Transferase
Ubl conjugation pathway
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
nameALG13
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8996410] ENSEMBL:ENSG00000101901 ALG13 [Homo sapiens]
secondaryIdentifierALG13_HUMAN
B1AKD6
B1AKM1
B2R5L5
B7Z6J0
B7Z804
B7Z847
B7Z9A8
B7ZAJ1
B7ZB57
Q17RC3
Q5JXY9
Q9H5U8
sequenceLength1137
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8970379] UniProt:Q9NP73-1 ALG13 [Homo sapiens]
[ReferenceIsoform:8970380] UniProt:Q9NP73-2 ALG13 [Homo sapiens]
[ReferenceIsoform:8970381] UniProt:Q9NP73-3 ALG13 [Homo sapiens]
[ReferenceIsoform:8970382] UniProt:Q9NP73-4 ALG13 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:449329] ALG13(1-1137) [endoplasmic reticulum membrane] [Homo sapiens]
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