GNS

The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.
Full Name
glucosamine (N-acetyl)-6-sulfatase
Biological Process
Glycosaminoglycan catabolic process Source: ProtInc
Keratan sulfate catabolic process Source: Reactome
Cellular Location
Lysosome
Involvement in disease
Mucopolysaccharidosis 3D (MPS3D):
A form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life.
PTM
The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
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Anti-GNS antibodies

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Rabbit Anti-GNS Recombinant Antibody (CBLG1-1466) (CBMAB-G4248-LY)

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Target: GNS
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1466
Application*: E, WB
Datasheet SDS

Mouse Anti-GNS Recombinant Antibody (CBLG1-1465) (CBMAB-G4247-LY)

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Target: GNS
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBLG1-1465
Application*: E, WB
Datasheet SDS

Rabbit Anti-GNS Recombinant Antibody (CBLG1-1461) (CBMAB-G4243-LY)

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Target: GNS
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1461
Application*: WB
Datasheet SDS

Mouse Anti-GNS Recombinant Antibody (7D5H8H11) (CBMAB-G4240-LY)

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Target: GNS
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 7D5H8H11
Application*: E
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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