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GUSB Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| IHC-P, FC, WB, E |
|---|---|
| Primary Accession | P08236 |
| Other Accession | Q4FAT7 |
| Reactivity | Human, Mouse |
| Predicted | Pig |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Calculated MW | 74732 Da |
| Antigen Region | 335-362 aa |
| Gene ID | 2990 |
|---|---|
| Other Names | Beta-glucuronidase, Beta-G1, GUSB |
| Target/Specificity | This GUSB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 335-362 amino acids from the Central region of human GUSB. |
| Dilution | WB~~1:4000 IHC-P~~1:100 FC~~1:10~50 |
| Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
| Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | GUSB Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | GUSB |
|---|---|
| Function | Plays an important role in the degradation of dermatan and keratan sulfates. |
| Cellular Location | Lysosome. |
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Provided below are standard protocols that you may find useful for product applications.
Background
The GUSB gene encodes beta-glucuronidase (EC 3.2.1.31), a lysosomal hydrolase involved in the stepwise degradation of glucuronic acid-containing glycosaminoglycans (Shipley et al., 1993 [PubMed 7680524]). It is a tetrameric glycoprotein composed of identical subunits (Oshima et al., 1987 [PubMed 3468507]). The GUSB gene is mutated in mucopolysaccharidosis type VII (MPS7; MIM 253220).
References
Tomatsu,S. Hum. Mutat. 30 (4), 511-519 (2009)
Romanowski,T. Med. Sci. Monit. 14 (7), BR147-BR152 (2008)
Gratz,M. Pharmacogenet. Genomics 15 (12), 875-881 (2005)
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