Rabbit GUSB Polyclonal Antibody | anti-GUSB antibody

GUSB Rabbit Polyclonal

Cat. Num. AAA769965

• Gene Names: GUSB; BG; MPS7
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: GUSB; Polyclonal Antibody; GUSB Rabbit Polyclonal; anti-GUSB antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 651

• Applicable Applications for anti-GUSB antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Glucuronidase, beta
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human colon cancer using MBS769965 (GUSB antibody) at dilution of 1:50)

Western Blot (WB)

(mouse liver tissue were subjected to SDS PAGE followed by western blot with MBS769965 (GUSB antibody) at dilution of 1:500)

NCBI and Uniprot Product Information

• NCBI GI #: 823671294
• NCBI GeneID: 2990
• Molecular Weight: 58,345 Da
• NCBI Official Full Name: GUSB, partial
• 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 - pentose and glucuronate interconversions; Carbohydrate Metabolism - starch and sucrose; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 3.2.1.31; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; Xenobiotic Metabolism - drug metabolism - other enzymes

Chromosomal Location of Human Ortholog: 7q21.11

Cellular Component: extracellular space; intracellular membrane-bound organelle; lysosomal lumen; membrane

Molecular Function: beta-glucuronidase activity; protein domain specific binding; receptor binding

Biological Process: glycosaminoglycan catabolic process; hyaluronan catabolic process

Disease: Mucopolysaccharidosis, Type Vii