Human Glucuronidase Beta (GUSb) ELISA Kit | GUSb elisa kit

Human Glucuronidase Beta (GUSb) Mini Samples ELISA Kit

Cat. Num. AAA2124076

• Gene Names: GUSB; BG; MPS7
• Reactivity: Human
• Applications: ELISA
• Synonyms: Glucuronidase Beta (GUSb); Human Glucuronidase Beta (GUSb) Mini Samples ELISA Kit; GUS-B; MPS7; Beta-G1; GUSb elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of GUSb. No significant cross-reactivity or interference between GUSb and analogues was observed.

• Applicable Applications for GUSb elisa kit: ELISA (EIA)
• Application Notes: Enzyme-linked Immunosorbent Assay for Antigen Detection.
• Samples: Serum, plasma, tissue homogenates, cell lysates, cell culture supernates and other biological fluids
• Assay Type: Quantitative Sandwich
• Detection Range: 1.56-100ng/mL
• Sensitivity: < 0.67ng/mL
• Intra-assay Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level GUSb were tested 20 times on one plate, respectively. Intra-Assay: CV<10%
• Inter-assay Precision: Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level GUSb were tested on 3 different plates, 8 replicates in each plate. CV(%) = SD/meanX100. Inter-Assay: CV<12%
• Preparation and Storage: The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition.; To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for GUSb elisa kit

Intended Uses: The kit is a sandwich enzyme immunoassay for in vitro quantitative measurement of GUSb in human serum, plasma, tissue homogenates, cell lysates, cell culture supernates and other biological fluids.

Principle of the Assay: The microplate provided in this kit has been pre-coated with an antibody specific to GUSb. Standards or samples are then added to the appropriate microplate wells with a biotin-conjugated antibody specific to GUSb. Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain GUSb, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of GUSb in the samples is then determined by comparing the O.D. of the samples to the standard curve.

Product Categories/Family for GUSb elisa kit

Signal transduction; Enzyme & Kinase; Metabolic pathway

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