Beta-Glucuronidase, ELISA Kit

Rabbit b-Glucuronidase (b-GUS) ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C.

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Small volumes of beta-GUS elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS2605664 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Beta-Glucuronidase (beta-GUS) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing beta-GUS. The ELISA analytical biochemical technique of the MBS2605664 kit is based on beta-GUS antibody-beta-GUS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect beta-GUS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, beta-GUS. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

58,345 Da

glucuronidase beta

beta-glucuronidase

Beta-glucuronidase

BGLR_HUMAN

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]

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 *****hood. 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

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