Anti-beta glucuronidase (GUSB) Antibody (CAB19248)

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SKU:
CAB19248
Product type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
GUSB
Synonyms:
BG
Synonyms:
MPS7
Synonyms:
beta-glucuronidase
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Description

Product Name:beta glucuronidase (GUSB) Rabbit mAb
Product Code:CAB19248
Size:50uL, 100uL
Synonyms:GUSB, BG, MPS7, beta-glucuronidase
Applications:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant protein of human beta glucuronidase (beta glucuronidase (GUSB)) .
Applications:WB
Recommended Dilutions:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:HepG2, A-549, HL-60
Immunogen:Recombinant protein of human beta glucuronidase (beta glucuronidase (GUSB)) .
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:2990
Uniprot:P08236
Cellular Location:Lysosome
Calculated MW:78kDa
Observed MW:75KDa
UniProt Protein Function:GUSB: Plays an important role in the degradation of dermatan and keratan sulfates. Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7); also known as Sly syndrome. MPS7 is 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. Mucopolysaccharidosis type 7 is associated with non- immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Belongs to the glycosyl hydrolase 2 family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Glycan Metabolism - glycosaminoglycan degradation; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Hydrolase; Carbohydrate Metabolism - starch and sucrose; Carbohydrate Metabolism - pentose and glucuronate interconversions; Xenobiotic Metabolism - drug metabolism - other enzymes; EC 3.2.1.31

Chromosomal Location of Human Ortholog: 7q21.11

Cellular Component: lysosomal lumen; membrane; intracellular membrane-bound organelle

Molecular Function:protein domain specific binding; beta-glucuronidase activity; receptor binding

Biological Process: glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; hyaluronan metabolic process; hyaluronan catabolic process

Disease: Mucopolysaccharidosis, Type Vii

NCBI Summary: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.[provided by RefSeq, May 2014]
UniProt Code:P08236
NCBI GenInfo Identifier:146345377
NCBI Gene ID:2990
NCBI Accession:P08236.2
UniProt Secondary Accession:P08236,Q549U0, Q96CL9, B4E1F6, E9PCV0,
UniProt Related Accession:P08236
Molecular Weight:651
NCBI Full Name:Beta-glucuronidase
NCBI Synonym Full Names:glucuronidase, beta
NCBI Official Symbol:GUSB  
NCBI Official Synonym Symbols:BG; MPS7  
NCBI Protein Information:beta-glucuronidase; beta-G1; beta-D-glucuronidase
UniProt Protein Name:Beta-glucuronidase
UniProt Synonym Protein Names:Beta-G1
Protein Family:Beta-glucuronidase
UniProt Gene Name:GUSB  
UniProt Entry Name:BGLR_HUMAN
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