Proteins/UNIPROT:Q3T906

GNPTAB

ProteinUNIPROT:Q3T906N-acetylglucosamine-1-phosphotransferase subunits alpha/beta

Catalyzes the formation of mannose 6-phosphate (M6P) markers on high mannose type oligosaccharides in the Golgi apparatus. M6P residues are required to bind to the M6P receptors (MPR), which mediate the vesicular transport of lysosomal enzymes to the endosomal/prelysosomal compartment..

Function from UniProtKB (Q3T906), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

4 rows

GNPTAB-mucolipidosis has a germline basis in GNPTABDiseaseGenCC HGNC:29670|MONDO:0100122Inferred only
mucolipidosis has a germline basis in GNPTABDiseaseGenCC HGNC:29670|MONDO:0019248Inferred only
mucolipidosis type II has a germline basis in GNPTABDiseaseGenCC HGNC:29670|MONDO:0009650Inferred only
mucolipidosis type III, alpha/beta has a germline basis in GNPTABDiseaseGenCC HGNC:29670|MONDO:0018931Inferred only

Statements

22 statements

22 statements · 4 reference databases · Subject: GNPTAB · Sources: Bgee, Hetionet, Human-GEM, Rhea

Order

FBN3 covaries with GNPTABProteinHetionet hetionet:covaries:79158-84467Inferred only
FHDC1 covaries with GNPTABProteinHetionet hetionet:covaries:79158-85462Inferred only
FHL2 covaries with GNPTABProteinHetionet hetionet:covaries:2274-79158Inferred only
FOLR2 covaries with GNPTABProteinHetionet hetionet:covaries:2350-79158Inferred only
MAMDC4 covaries with GNPTABProteinHetionet hetionet:covaries:158056-79158Inferred only
GNPTAB covaries with SHISAL2AProteinHetionet hetionet:covaries:348378-79158Inferred only
GNPTAB covaries with THAP3ProteinHetionet hetionet:covaries:79158-90326Inferred only
GNPTAB is expressed in endothelial cellCell typeBgee ENSG00000111670Inferred only
GNPTAB is expressed in parotid glandAnatomyBgee ENSG00000111670Inferred only
GNPTAB is expressed in sural nerveAnatomyBgee ENSG00000111670Inferred only
GNPTAB is expressed in tibiaAnatomyBgee ENSG00000111670Inferred only
GNPTAB is expressed in urethraAnatomyBgee ENSG00000111670Inferred only

Show the remaining 10

APP interacts with GNPTABProteinHetionet hetionet:interacts:351-79158Inferred only
DISC1 interacts with GNPTABProteinHetionet hetionet:interacts:27185-79158Inferred only
MAPK1 interacts with GNPTABProteinHetionet hetionet:interacts:5594-79158Inferred only
UBC interacts with GNPTABProteinHetionet hetionet:interacts:7316-79158Inferred only
AFF1 decreases the expression of GNPTABProteinHetionet hetionet:regulates:4299-79158Inferred only
E2F5 increases the expression of GNPTABProteinHetionet hetionet:regulates:1875-79158Inferred only
GALE decreases the expression of GNPTABProteinHetionet hetionet:regulates:2582-79158Inferred only
VRK2 increases the expression of GNPTABProteinHetionet hetionet:regulates:7444-79158Inferred only
CHEBI:16264 is a substrate of GNPTABCompoundHuman-GEM MAR09547Inferred only
UDP-N-acetyl-alpha-D-glucosamine is a substrate of GNPTABCompoundRhea RHEA:13581Inferred only

Reactions

2 reactions

MAM02427c+CHEBI:16264MAM02428c+UMP
Catalysed by GNPTAB
Reaction pageReversibleBalance undeterminableEC 2.7.8.17cytosol
Human-GEM MAR09547CC BY 4.0Reference data
N(4)-[alpha-D-mannosyl-(1->2)-alpha-D-mannosyl-(glycan)]-L-asparaginyl-[protein]+UDP-N-acetyl-alpha-D-glucosamineN(4)-[6-(N-acetyl-alpha-D-glucosaminyl-1-phospho)-alpha-D-mannosyl-(1->2)-alpha-D-mannosyl-(glycan)]-L-asparaginyl-[protein]+UMP+hydron
Catalysed by GNPTAB

Other names

5 names

Resolves to: GNPTAB

Also called: GlcNAc-1-phosphotransferase subunits alpha/betaGNPTAKIAA1208Stealth protein GNPTABUDP-N-acetylglucosamine-1-phosphotransferase subunits alpha/beta

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