hexosaminidase B (beta polypeptide); ENC-1AS; N-acetyl-beta-glucosaminidase|hexosaminidase B

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

Chromosome: 5; NC_000005.9 (73980969..74017113). Location: 5q13

No significant cross-reactivity or interference between this analyte and analogues is observed.

Store all reagents at 2-8 degree C

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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.

MBS9318384 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 MBS9318384 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(%).

Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of HEXB (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

63,111 Da

hexosaminidase B (beta polypeptide)

beta-hexosaminidase subunit beta; HCC-7; hexosaminidase subunit B; beta-N-acetylhexosaminidase subunit beta; cervical cancer proto-oncogene 7 protein; N-acetyl-beta-glucosaminidase 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). [provided by RefSeq, Jul 2008]

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

Chromosomal Location of Human Ortholog: 5q13

Cellular Component: lysosomal lumen; membrane; acrosome

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

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

Disease: Sandhoff Disease

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