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Details on Person UniProt:Q9H490 PIGU

Class:IdReferenceGeneProduct:61730
_chainChangeLoginitiator methionine:1 added on Sat February 7 2015;chain:2-435 added on Sat February 7 2015;initiator methionine:1 for 61730 removed on Fri Nov 03 2023;initiator methionine: for 61730 added on Fri Nov 03 2023;initiator methionine: for 61730 removed on Fri Aug 15 2025;initiator methionine:1 for 61730 added on Fri Aug 15 2025
_displayNameUniProt:Q9H490 PIGU
_timestamp2025-08-15 21:09:56
chaininitiator methionine:1
chain:2-435
checksum65FDD796AB533027
commentFUNCTION Component of the glycosylphosphatidylinositol-anchor (GPI-anchor) transamidase (GPI-T) complex that catalyzes the formation of the linkage between a proprotein and a GPI-anchor and participates in GPI anchored protein biosynthesis (PubMed:12802054, PubMed:31353022, PubMed:34576938, PubMed:35165458, PubMed:35551457, PubMed:37684232). Binds the lipid portion of GPI-anchor (PubMed:37684232). May act as an organizer in the transmembrane layer to recruit other subunits, and thus is essential for assembly of the complex (PubMed:35165458, PubMed:35551457).PATHWAY Glycolipid biosynthesis; glycosylphosphatidylinositol-anchor biosynthesis.SUBUNIT Heteropentamer (PubMed:35551457). Part of the GPI-anchor transamidase complex, consisting of PIGK, PIGT, PIGS, PIGU and GAA1 (PubMed:12802054, PubMed:34576938, PubMed:35165458, PubMed:35551457, PubMed:37684232).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the PIGU family.
descriptionrecommendedName: fullName evidence="8"GPI-anchor transamidase component PIGU alternativeName: Cell division cycle protein 91-like 1 shortName: Protein CDC91-like 1 alternativeName: GPI transamidase component PIG-U alternativeName: Phosphatidylinositol glycan anchor biosynthesis class U protein
geneNamePIGU
CDC91L1
PSEC0205
UNQ3055/PRO9875
identifierQ9H490
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Direct protein sequencing
Disease variant
Endoplasmic reticulum
Epilepsy
GPI-anchor biosynthesis
Intellectual disability
Membrane
Proteomics identification
Reference proteome
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
namePIGU
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8962473] ENSEMBL:ENSG00000101464 PIGU [Homo sapiens]
secondaryIdentifierPIGU_HUMAN
Q7Z489
Q8N2F2
sequenceLength435
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:152110] UniProt:Q9H490-2 PIGU [Homo sapiens]
[ReferenceIsoform:414239] UniProt:Q9H490-1 PIGU [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:162835] PIGU [endoplasmic reticulum membrane] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q9H490 PIGU (61730)