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Conditions of optimal GUSB Antibody performance should be determined experimentally by the investigator.
Price/availability/specifications subject to change without notice. Unless otherwise indicated, our catalog and customized products are for research use only and not intended for human or animal diagnostic or therapeutic use.
Phone: 1-617-401-8149
Fax: 1-617-606-5019
Email: message@sydlabs.com
Or leave a message with a formal purchase
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Introduction
GUSB(Beta-Glucuronidase) is an enzyme that in humans is encoded by the GUSB gene. The GUSB gene encodes beta-glucuronidase, a lysosomal hydrolase involved in the stepwise degradation of glucuronic acid-containing glycosaminoglycans. It is a tetrameric glycoprotein composed of identical subunits. The GUSB gene is mutated in mucopolysaccharidosis type VII. Miller et al. (1990) reported that the GUSB gene is 21 kb long and contains 12 exons. The GUSB gene is mapped on 7q11.21. Beta-glucuronic acid acts naturally to cleave glucuronides from conjugated bilirubin to produce unconjugated bilirubin, which is then absorbed into the bloodstream.Kreamer et al. (2001) postulated that the beta-glucuronidase inhibitor L-aspartic acid can thus block enterohepatic circulation of bilirubin. Tomatsu et al. (2002) stated that more than 45 different mutations in the GUSB gene had been identified in human patients with MPS VII, approximately 90% of which were point mutations.
PA006292-PA1916: GUSB Polyclonal Antibody
Rabbit Polyclonal Antibody.
Applications: WB 0.1-0.5 ug/ml.
Reactivity: Human.
Immunogen: A synthetic peptide corresponding to a sequence at the N-terminal of human GUSB.
Purification: Immunogen affinity purified.
Form of Antibody: Lyophilized.
Isotype: Rabbit IgG.
Storage/Stability: Stable for 1 year at -20°C and 3 months at 4°C. For maximum recovery of the product, centrifuge the original vial after thawing and before removing the cap. Aliquot to avoid repeated freezing and thawing.
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