GUSB Antibody

Code CSB-PA010064GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
GUSB
Alternative Names
Ac2-223 antibody; asd antibody; Beta G1 antibody; Beta glucuronidase antibody; Beta-G1 antibody; Beta-glucuronidase antibody; BG antibody; BGLR antibody; BGLR_HUMAN antibody; FLJ39445 antibody; Glucuronidase beta antibody; Gur antibody; Gus antibody; Gus-r antibody; Gus-s antibody; Gus-t antibody; Gus-u antibody; GUSB antibody; Gut antibody; MPS7 antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human GUSB
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB,IHC
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Plays an important role in the degradation of dermatan and keratan sulfates.
Gene References into Functions
  1. Results show that GUS mRNA changes in blood are significantly correlated with gait step length variability in premutation (PM) females patients with fragile X tremor ataxia syndrome . PMID: 27387142
  2. We produced GUS from a CHO cell line grown in suspension in a 15 L perfused bioreactor and developed a three step purification procedure that yields approximately 99% pure enzyme with a recovery of more than 40%. PMID: 28734840
  3. Selective and amplified bG expression together with the prodrug DOXGA3 had an increased antitumor effect, showing great potential for prostate cancer therapy. PMID: 26648021
  4. the use of in silico approaches provided a useful understanding of the effect of single point mutations on the structure-function relationship of GUSBp PMID: 26415878
  5. the efficacy of a helper-dependent (HD) canine adenovirus (CAV-2) vector harboring a human GUSB expression cassette (HD-RIGIE) in the MPS VII dog brain was tested PMID: 24343103
  6. This assessment demonstrated that although butyrate dramatically increased beta-glucuronidase production in bioreactors, it adversely impacted the mannose-6-phosphorylation of this lysosomal storage diseasestherapeutic enzyme PMID: 24033810
  7. expression of CES2, UGTA1A1, and GUSB varies in colorectal pathology tissues and that the expression of CES2 is somewhat related to tumor staging. PMID: 24195516
  8. GUSB and ATP2B4 have been validated as a reliable gene combination for Cystic Fibrosis Transmembrane Conductance Regulator gene qPCR data normalization. PMID: 22525089
  9. Liver disease affects the expression of common HKGs; beta-glucuronidase and splicing factor arginine/serine-rich 4 are the most stable HKGs for studies of gene expression in HCV-infected human liver. PMID: 21073651
  10. beta-glucuronidase mutations are associated with mucopolysaccharidosis type VII PMID: 12522561
  11. Recombinant adeno-associated virus encoding human GUSB injected into the vitreous humor of young adult MPS VII mice increased GUSB activity and reduced lysosomal distension in regions of the thalamus and tectum PMID: 12716937
  12. beta glucuronidase present in the synovial fluid of rheumatoid arthritis patients, may contribute to the depletion of glycosaminoglycans from cartilage allowing invasion of synovial cells. PMID: 12905469
  13. Over-expression of beta-glucuronidase was related to the degree of cancer differentiation, but not to lymph node metastasis. PMID: 14612292
  14. These results indicate that beta-glucuronidase transport into brain parenchyma in early postnatal life is mediated by the mannose 6-phosphate/insulin-like growth factor II receptor. PMID: 15314220
  15. The genes GUS and PMM1 are recommended for normalization purposes in gene expression studies of liver tissue from patients with chronic hepatitis. PMID: 18591914
  16. Study summarized information on the 49 unique, disease-causing mutations determined so far in the GUS gene. PMID: 19224584

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Involvement in disease
Mucopolysaccharidosis 7 (MPS7)
Subcellular Location
Lysosome.
Protein Families
Glycosyl hydrolase 2 family
Database Links

HGNC: 4696

OMIM: 253220

KEGG: hsa:2990

STRING: 9606.ENSP00000302728

UniGene: Hs.255230

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