Proteins/UNIPROT:Q8NBL1

POGLUT1

ProteinUNIPROT:Q8NBL1

Dual specificity glycosyltransferase that catalyzes the transfer of glucose and xylose from UDP-glucose and UDP-xylose, respectively, to a serine residue found in the consensus sequence of C-X-S-X-P-C. Specifically targets extracellular EGF repeats of protein such as CRB2, F7, F9 and NOTCH2. Acts as a positive regulator of Notch signaling by mediating O-glucosylation of Notch, leading to regulate muscle development. Notch glucosylation does not affect Notch ligand binding. Required during early development to promote gastrulation: acts by mediating O-glucosylation of CRB2, which is required for CRB2 localization to the cell membrane (By similarity)..

Function from UniProtKB (Q8NBL1), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

4 rows

autosomal recessive limb-girdle muscular dystrophy has a germline basis in POGLUT1DiseaseGenCC HGNC:22954|MONDO:0015152Inferred only
autosomal recessive limb-girdle muscular dystrophy type 2R1 has a germline basis in POGLUT1DiseaseGenCC HGNC:22954|MONDO:0014977Inferred only
Dowling-Degos disease has a germline basis in POGLUT1DiseaseGenCC HGNC:22954|MONDO:0008371Inferred only
Dowling-Degos disease 4 has a germline basis in POGLUT1DiseaseGenCC HGNC:22954|MONDO:0014307Inferred only

Statements

15 statements

15 statements · 4 reference databases · Subject: POGLUT1 · Sources: gene ontology, Hetionet, Reactome, Rhea

Order

OR1D5 covaries with POGLUT1ProteinHetionet hetionet:covaries:56983-8386Inferred only
RPP25L covaries with POGLUT1ProteinHetionet hetionet:covaries:138716-56983Inferred only
SLC25A53 covaries with POGLUT1ProteinHetionet hetionet:covaries:401612-56983Inferred only
POGLUT1 covaries with SLC38A1ProteinHetionet hetionet:covaries:56983-81539Inferred only
POGLUT1 covaries with SPRED3ProteinHetionet hetionet:covaries:399473-56983Inferred only
POGLUT1 participates in Pre-NOTCH Expression and ProcessingPathwayReactome R-HSA-1912422Inferred only
POGLUT1 participates in Pre-NOTCH Processing in the Endoplasmic ReticulumPathwayReactome R-HSA-1912399Inferred only
POGLUT1 participates in Signal TransductionPathwayReactome R-HSA-162582Inferred only
POGLUT1 participates in Signaling by NOTCHPathwayReactome R-HSA-157118Inferred only
POGLUT1 regulates positive regulation of Notch signaling pathwayBiological processgene ontology Q8NBL1|GO:0045747Inferred only
POGLUT1 regulates regulation of gastrulationBiological processgene ontology Q8NBL1|GO:0010470Inferred only
EBF1 increases the expression of POGLUT1ProteinHetionet hetionet:regulates:1879-56983Inferred only

Show the remaining 3

RPS19 increases the expression of POGLUT1ProteinHetionet hetionet:regulates:6223-56983Inferred only
UDP-alpha-D-glucose is a substrate of POGLUT1CompoundRhea RHEA:58116Inferred only
UDP-alpha-D-xylose is a substrate of POGLUT1CompoundRhea RHEA:62016Inferred only

Reactions

2 reactions

L-seryl-[EGF-like domain protein]+UDP-alpha-D-glucose3-O-(beta-D-glucosyl)-L-seryl-[EGF-like domain protein]+UDP+hydron
Catalysed by POGLUT2 or POGLUT3 or POGLUT1
Reaction pageDirection not assertedBalance undeterminable
Rhea RHEA:58116CC BY 4.0Reference data
L-seryl-[EGF-like domain protein]+UDP-alpha-D-xylose3-O-(beta-D-xylosyl)-L-seryl-[EGF-like domain protein]+UDP+hydron

Other names

12 names

Resolves to: POGLUT1

Also called: C3orf9CAP10-like 46 kDa proteinCLP46hCLP46hRumiKTEL motif-containing protein 1KTELC1MDSRPMyelodysplastic syndromes relative proteinO-glucosyltransferase Rumi homologO-xylosyltransferase POGLUT1Protein O-xylosyltransferase POGLUT1

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