Beta-hexosaminidase subunit beta, ELISA Kit

Hexb/ENC-1AS/N-acetyl-beta-glucosaminidase subunit beta/Beta-N-acetylhexosaminidase subunit beta (Hexosaminidase subunit B)/Cervical cancer proto-oncogene 7 protein (HCC-7)/ENC-1AS/HCC-7/HEL-248/beta-hexosaminidase subunit beta/Beta-N-acetylhexosaminidase subunit beta/Cervical cancer proto-oncogene 7 protein/hexosaminidase B (beta polypeptide)/Hexosaminidase subunit B/N-acetyl-beta-glucosaminidase subunit beta

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

Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of Hexb 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.

MBS761479 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Beta-hexosaminidase subunit beta (Hexb) 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 Hexb. The ELISA analytical biochemical technique of the MBS761479 kit is based on Hexb antibody-Hexb antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect Hexb antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, Hexb. 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(%).

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Rat Hexb antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Rat Hexb antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Rat Hexb amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Rat Hexb can be calculated.

63,111 Da

hexosaminidase subunit beta

beta-hexosaminidase subunit beta

Beta-hexosaminidase subunit beta

Hexosaminidase subunit B; HCC-7  [Similar Products]

HEXB_HUMAN

Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2014]

HEXB: Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2); also known as Sandhoff disease. GM2- gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2- gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula. Belongs to the glycosyl hydrolase 20 family.

Protein type: Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.2.1.52; Carbohydrate Metabolism - amino sugar and nucleotide sugar; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - other glycan degradation

Chromosomal Location of Human Ortholog: 5q13

Cellular Component: acrosome; azurophil granule; lysosomal lumen; membrane

Molecular Function: acetylglucosaminyltransferase activity; beta-N-acetylhexosaminidase activity; protein heterodimerization activity; protein homodimerization activity

Biological Process: astrocyte cell migration; carbohydrate metabolic process; cellular calcium ion homeostasis; cellular protein metabolic process; chondroitin sulfate catabolic process; chondroitin sulfate metabolic process; ganglioside catabolic process; glycosaminoglycan metabolic process; glycosphingolipid metabolic process; hyaluronan catabolic process; hyaluronan metabolic process; keratan sulfate catabolic process; keratan sulfate metabolic process; locomotory behavior; lysosome organization and biogenesis; male courtship behavior; myelination; neuromuscular process controlling balance; oligosaccharide catabolic process; oogenesis; penetration of zona pellucida; phospholipid biosynthetic process; positive regulation of transcription from RNA polymerase II promoter; regulation of cell shape; sensory perception of sound; sequestering of lipid; skeletal development; sphingolipid metabolic process

Disease: Sandhoff Disease

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