Rabbit anti-Human GUSB Polyclonal Antibody | anti-GUSB antibody

GUSB, ID (GUSB, Beta-glucuronidase, Beta-G1) (AP)

Cat. Num. AAA6305163

• Gene Names: GUSB; BG; MPS7
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: GUSB; Polyclonal Antibody; ID (GUSB; Beta-glucuronidase; Beta-G1) (AP); Beta-G1; anti-GUSB antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-GUSB antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:50-100; ELISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: GUSB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 341~371 amino acids from the Center region of human GUSB.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of GUSB Antibody (Center) in WiDr, A2058, K562 cell line lysates (35ug/lane). GUSB (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human colon carcinoma reacted with GUSB Antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(GUSB Antibody (Center) flow cytometric analysis of WiDr cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Related Product Information for anti-GUSB antibody

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).

Product Categories/Family for anti-GUSB antibody

Antibodies; Enzymes

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).

NCBI and Uniprot Product Information

• NCBI GI #: 268834192
• NCBI GeneID: 2990
• NCBI Accession #: NP_000172.2
• NCBI GenBank Nucleotide #: NM_000181.3
• UniProt Accession #: P08236; Q549U0; Q96CL9
• Molecular Weight: 74,732 Da
• NCBI Official Full Name: beta-glucuronidase
• NCBI Official 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
• Protein Family: Beta-glucuronidase
• UniProt Gene Name: GUSB
• UniProt Entry Name: BGLR_HUMAN

NCBI Description

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. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, Nov 2009]

Uniprot Description

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.

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

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