Rabbit GUSB Polyclonal Antibody | anti-GUSB antibody

GUSB Polyclonal Antibody

Cat. Num. AAA3007488

• Gene Names: GUSB; BG; MPS7
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >96% (by SDS-PAGE).
• Synonyms: GUSB; Polyclonal Antibody; GUSB Polyclonal Antibody; asd; Beta G1; Beta glucuronidase; Beta-G1; Beta-glucuronidase; BG; BGLR; BGLR_HUMAN; FLJ39445; Glucuronidase beta; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; Gut; MPS7; anti-GUSB antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: GUSB polyclonal antibody detects endogenous levels of GUSB protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >96% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 651

• Applicable Applications for anti-GUSB antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:1000
• Immunogen: Synthetic peptide, corresponding to amino acids 324-368 of Human GUSB.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of GUSB polyclonal antibody at 1:500 dilutionLane1:HepG2 whole cell lysate (40ug) Lane2:The liver tissue lysate of Fetal Mouse (40ug) Lane3:The lliver tissue lysate of Fetal Rat (40ug))

Related Product Information for anti-GUSB antibody

The enzyme beta-glucuronidase catalyzes the conversion of beta-D-glucuronoside and water to an alcohol and D-glucuronate. Deficiency of beta-glucuronidase is the cause of the human lysosomal storage disorder mucopolysaccharidosis type VII (MPS VII). Specifically, two residues appear important for catalytic activity: Glu 451 and Glu 540. Mutations at these sites affect the overall structure of the protein, which normally consists of a homotetramer with each promoter including a jelly roll barrel, an immunoglobulin constant domain and a TIM barrel. Regulation of beta-glucuronidase activity may play a role in tumorigenesis and the invasiveness of a number of cancers, and is also an important factor in the development of functional prodrugs that require the cleavage of an active cytostatic by endogenous enzymes for antitumor activity.

NCBI and Uniprot Product Information

• NCBI GI #: 268834192
• NCBI GeneID: 2990
• NCBI Accession #: NP_000172.2
• NCBI GenBank Nucleotide #: NP_000172.2
• UniProt Accession #: P08236
• Molecular Weight: ~ 74kDa
• NCBI Official Full Name: beta-glucuronidase isoform 1
• NCBI Official Synonym Full Names: glucuronidase beta
• NCBI Official Symbol: GUSB
• NCBI Official Synonym Symbols: BG; MPS7
• NCBI Protein Information: beta-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. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, May 2014]

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