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

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Target: GUSB

Host: Rabbit

Antibody Isotype: IgG

Specificity: Human

Clone: CBLG1-2152

Application*: WB, P, F

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

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Target: GUSB

Host: Mouse

Antibody Isotype: IgG1, κ

Specificity: Human

Clone: CBLG1-2149

Application*: E, IP, IF, WB, P

Rat Gusb ELISA Kit (V2LY-0626-LY912)

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Target: Gusb

Sensitivity: 0.02 ng/mL

Detection Range: 0.05-20 ng/mL

Sample Type: Serum, Plasma, cell culture supernates

Specificity: Rat

Assay Type: Sandwich

Reactivity: Rat

Mouse Gusb ELISA Kit (V2LY-0626-LY764)

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Target: Gusb

Sensitivity: 0.024 ng/mL

Detection Range: 0.05-20 ng/mL

Sample Type: Serum, Plasma, cell culture supernates

Specificity: Mouse

Assay Type: Sandwich

Reactivity: Mouse

Human GUSB ELISA Kit (V2LY-0626-LY1116)

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Target: GUSB

Sensitivity: 0.22 ng/mL

Detection Range: 0.5-60 ng/mL

Sample Type: Serum, Plasma, cell culture supernates

Specificity: Human

Assay Type: Sandwich

Reactivity: Human

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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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