Human Beta-hexosaminidase subunit beta (HEXB) ELISA Kit | MyBioSource MBS9318384 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Beta-hexosaminidase subunit beta (HEXB) ELISA Kit

catalog :

MBS9318384

quantity :

48-Strip-Wells

price :

435 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS9318384

products type :

ELISA Kit

products full name :

Human Beta-hexosaminidase subunit beta (HEXB) ELISA Kit

products short name :

Beta-hexosaminidase subunit beta (HEXB)

products name syn :

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

other names :

beta-hexosaminidase subunit beta preproprotein; Beta-hexosaminidase subunit beta; 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; hexosaminidase B (beta polypeptide); Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B; Cervical cancer proto-oncogene 7 protein; HCC-7; N-acetyl-beta-glucosaminidase subunit beta

products gene name :

HEXB

other gene names :

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

uniprot entry name :

HEXB_HUMAN

reactivity :

Human

specificity :

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

storage stability :

Store all reagents at 2-8 degree C

other info1 :

Samples: Serum, Plasma and Tissue Homogenate. Assay Type: Sandwich. Detection Range: 3.12 ug/ml - 100 ug/ml. Sensitivity: 1.0 ug/ml.

other info2 :

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].

products description :

Background: 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 this analyte) in undiluted original Human serum, plasma and tissue homogenate samples.

ncbi gi num :

4504373

ncbi acc num :

NP_000512.1

ncbi gb acc num :

NM_000521.3

uniprot acc num :

P07686

ncbi mol weight :

63,111 Da

ncbi pathways :

Amino Sugar And Nucleotide Sugar Metabolism Pathway (82979); Amino Sugar And Nucleotide Sugar Metabolism Pathway (350); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosphingolipid Biosynthesis - Ganglio Series Pathway (82997); Glycosphingolipid Biosynthesis - Ganglio Series Pathway (372); Glycosphingolipid Biosynthesis - Globo Series Pathway (82996); Glycosphingolipid Biosynthesis - Globo Series Pathway (371); Glycosphingolipid Metabolism Pathway (530751); Keratan Sulfate Degradation Pathway (413378)

ncbi summary :

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]

uniprot summary :

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

size1 :

48-Strip-Wells

price1 :

435 USD

size2 :

96-Strip-Wells

price2 :

600

more info or order :

MyBioSource product webpage