ELISA/assay

Hexosaminidase B Beta (HEXb) ELISA Kit

MBS2022114

48-Strip-Wells

515 USD

ELISA Kit

Hexosaminidase B Beta (HEXb) ELISA Kit

Hexosaminidase B Beta (HEXb)

Hex-B; ENC-1AS; HCC-7; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; N-acetyl-beta-glucosaminidase subunit beta

hexosaminidase B (beta polypeptide), isoform CRA_a; Beta-hexosaminidase subunit beta; beta-hexosaminidase subunit beta; hexosaminidase B (beta polypeptide); Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B

HEXb

HEXB; HEXB; ENC-1AS; HEL-248; Hexosaminidase subunit B; HCC-7

HEXB_HUMAN

Human

556

This assay has high sensitivity and excellent specificity for detection of Hexosaminidase B Beta (HEXb). No significant cross-reactivity or interference between Hexosaminidase B Beta (HEXb) and analogues was observed.

For unopened kit: All the reagents should be kept according to the labels on vials. The Standard, Detection Reagent A, Detection Reagent B and the 96-well strip plate should be stored at -20 degree C upon receipt while the others should be at 4 degree C. For opened kit: When the kit is opened, the remaining reagents still need to be stored according to the above storage condition. Besides, please return the unused wells to the foil pouch containing the desiccant pack, and reseal along entire edge of zip-seal. 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.

Samples: Serum, plasma, tissue homogenates and other biological fluids. Assay Type: Sandwich. Detection Range: 0.156-10ng/mL. Sensitivity: Typically less than 0.055ng/mL.

Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Hexosaminidase B Beta (HEXb) were tested 20 times on one plate, respectively. Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Hexosaminidase B Beta (HEXb) were tested on 3 different plates, 8 replicates in each plate. CV(%) = SD/meanX100 . Intra-Assay: CV<10% . Inter-Assay: CV<12% . Assay Procedure Summary: 1. Prepare all reagents, samples and standards;. 2. Add 100uL standard or sample to each well. Incubate 2 hours at 37 degree C;. 3. Aspirate and add 100uL prepared Detection Reagent A. Incubate 1 hour at 37 degree C;. 4. Aspirate and wash 3 times;. 5. Add 100uL prepared Detection Reagent B. Incubate 30 minutes at 37 degree C;. 6. Aspirate and wash 5 times;. 7. Add 90uL Substrate Solution. Incubate 15-25 minutes at 37 degree C;. 8. Add 50uL Stop Solution. Read at 450nm immediately.

Principle of the Assay: The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Hexosaminidase B Beta (HEXb). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Hexosaminidase B Beta (HEXb). 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 Hexosaminidase B Beta (HEXb), 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 Hexosaminidase B Beta (HEXb) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

119616148

EAW95742.1

P07686

63,111 Da

Amino Sugar And Nucleotide Sugar Metabolism Pathway (82979); Amino Sugar And Nucleotide Sugar Metabolism Pathway (350); CS/DS Degradation Pathway (645311); Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway (645308); Defective B3GAT3 Causes JDSSDHD Pathway (1127588); Defective B4GALT1 Causes B4GALT1-CDG (CDG-2d) Pathway (1127592); Defective B4GALT7 Causes EDS, Progeroid Type Pathway (1127587); Defective CHST14 Causes EDS, Musculocontractural Type Pathway (1127591); Defective CHST3 Causes SEDCJD Pathway (1127590); Defective CHST6 Causes MCDC1 Pathway (1127593)

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: Carbohydrate Metabolism - amino sugar and nucleotide sugar; Glycan Metabolism - other glycan degradation; EC 3.2.1.52; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Hydrolase. 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: oogenesis; male courtship behavior; keratan sulfate metabolic process; myelination; glycosaminoglycan metabolic process; ganglioside catabolic process; pathogenesis; locomotory behavior; hyaluronan catabolic process; regulation of cell shape; sequestering of lipid; oligosaccharide catabolic process; 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; cellular calcium ion homeostasis; chondroitin sulfate metabolic process; cellular protein metabolic process; lysosome organization and biogenesis; carbohydrate metabolic process; glycosphingolipid metabolic process; positive regulation of transcription from RNA polymerase II promoter; hyaluronan metabolic process; astrocyte cell migration. Disease: Sandhoff Disease

48-Strip-Wells

515 USD

96-Strip-Wells

695

5x96-Strip-Wells

2685

10x96-Strip-Wells

4850