GUSB

This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.
Full Name
glucuronidase, beta
Function
Plays an important role in the degradation of dermatan and keratan sulfates.
Biological Process
Carbohydrate metabolic process Source: ProtInc
Glucuronoside catabolic process Source: GO_Central
Glycosaminoglycan catabolic process Source: ProtInc
Cellular Location
Lysosome
Involvement in disease
Mucopolysaccharidosis 7 (MPS7):
An autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment.
PTM
N-linked glycosylated with 3 to 4 oligosaccharide chains.
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Anti-GUSB antibodies

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Mouse Anti-GUSB Recombinant Antibody (CBLG1-2153) (CBMAB-G5817-LY)

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Target: GUSB
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-2153
Application*: WB
Datasheet SDS

Mouse Anti-GUSB Recombinant Antibody (CBLG1-2152) (CBMAB-G5816-LY)

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Target: GUSB
Host: Mouse
Antibody Isotype: IgG
Specificity: Rat
Clone: CBLG1-2152
Application*: WB
Datasheet SDS

Mouse Anti-GUSB Recombinant Antibody (CBLG1-2149) (CBMAB-G5813-LY)

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Target: GUSB
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-2149
Application*: E, WB
Datasheet SDS

Mouse Anti-GUSB Recombinant Antibody (105) (CBMAB-G5810-LY)

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Target: GUSB
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 105
Application*: E, C, WB
Datasheet SDS

Mouse Anti-GUSB Recombinant Antibody (CBLG1-283) (CBMAB-G0940-LY)

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Target: GUSB
Host: Mouse
Antibody Isotype: IgM
Specificity: E. coli
Clone: CBLG1-283
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
  • 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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