Entity Details

Primary name GALNS_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP34059
EntryNameGALNS_HUMAN
FullNameN-acetylgalactosamine-6-sulfatase
TaxID9606
Evidenceevidence at protein level
Length522
SequenceStatuscomplete
DateCreated1994-02-01
DateModified2021-06-02

Ontological Relatives

GenesGALNS

GO terms

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GOName
GO:0003943 N-acetylgalactosamine-4-sulfatase activity
GO:0004065 arylsulfatase activity
GO:0005576 extracellular region
GO:0008484 sulfuric ester hydrolase activity
GO:0035578 azurophil granule lumen
GO:0042340 keratan sulfate catabolic process
GO:0043202 lysosomal lumen
GO:0043312 neutrophil degranulation
GO:0043890 N-acetylgalactosamine-6-sulfatase activity
GO:0046872 metal ion binding
GO:0070062 extracellular exosome

Subcellular Location

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Subcellular Location
Lysosome

Domains

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DomainNameCategoryType
IPR000917 Sulfatase, N-terminalDomainDomain
IPR017850 Alkaline-phosphatase-like, core domain superfamilyFamilyHomologous superfamily
IPR024607 Sulfatase, conserved siteSiteConserved site
IPR035626 N-acetylgalactosamine-6-sulfataseFamilyFamily

Diseases

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Disease IDSourceNameDescription
253000 OMIMMucopolysaccharidosis 4A (MPS4A)A form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB09301 Chondroitin sulfateDrugbanksmall molecule

Interactions

2 interactions

InteractorPartnerSourcesPublicationsLink
GALNS_HUMANMTR1A_HUMANBioGRID, IntAct26514267 details
GALNS_HUMANNEUR1_HUMANBioGRID8910459 details