Rat Beta-Glucuronidase (betaGD) ELISA Kit | betaGD elisa kit

Rat beta-Glucuronidase (betaGD) ELISA Kit

Cat. Num. AAA3807699

• Gene Names: GUSB; BG; MPS7
• Reactivity: Rat
• Synonyms: Beta-Glucuronidase (betaGD); Rat beta-Glucuronidase (betaGD) ELISA Kit; betaGD elisa kit

• Reactivity: Rat
• Sequence Length: 651

• Samples: Serum, plasma, cell culture supernate and other biological fluids
• Assay Type: Sandwich
• Detection Range: 1.5 U/L-24 U/L
• Sensitivity: 0.1 U/L

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for betaGD elisa kit

Intended Use: The Stop Solution changes the color from blue to yellow and the intensity of the color is measured at 450 nm using a spectrophotometer. In order to measure the concentration ofbetaGDin the sample, This betaGDELISA Kit includes a set of calibration standards. The calibration standards are assayed at the same time as the samples and allow the operator to produce a standard curve of Optical Density versusbetaGDconcentration. The concentration ofbetaGDin the samples is then determined by comparing the O.D. of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 268834192
• NCBI GeneID: 2990
• NCBI GenBank Nucleotide #: NP_000172.2
• UniProt Accession #: P08236
• 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
• 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