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
-90637.jpg)
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).
Organism species: Homo sapiens (Human)
- Recombinant protein RPA637Hu01 Recombinant Hexosaminidase B Beta (HEXb) In Stock
- Recombinant protein RPA637Hu02 Recombinant Hexosaminidase B Beta (HEXb) In Stock
- Recombinant protein RPA637Hu03 Recombinant Hexosaminidase B Beta (HEXb) In Stock
- Polyclonal antibody PAA637Hu01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Monoclonal antibody MAA637Hu22 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Polyclonal antibody PAA637Hu02 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Labelled antibody LAA637Hu71 Biotin-Linked Polyclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Sandwich ELISA SEA637Hu ELISA Kit for Hexosaminidase B Beta (HEXb) In Stock
Organism species: Mus musculus (Mouse)
- Recombinant protein RPA637Mu01 Recombinant Hexosaminidase B Beta (HEXb) In Stock
- Recombinant protein RPA637Mu02 Recombinant Hexosaminidase B Beta (HEXb) In Stock
- Polyclonal antibody PAA637Mu01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Polyclonal antibody PAA637Mu02 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Sandwich ELISA SEA637Mu ELISA Kit for Hexosaminidase B Beta (HEXb) In Stock
Organism species: Rattus norvegicus (Rat)
- Recombinant protein RPA637Ra01 Recombinant Hexosaminidase B Beta (HEXb) In Stock
- Polyclonal antibody PAA637Ra01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Monoclonal antibody MAA637Ra21 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) In Stock
- Sandwich ELISA SEA637Ra ELISA Kit for Hexosaminidase B Beta (HEXb) In Stock