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Hexosaminidase B Beta (HEXb)

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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 (HEXb)
CCC A637
HGNC 4879
MGI 96074
PubMed 1967020
RGD 1307607
UniProt P07686
Wiki HEXB

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)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA637Hu01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
RPA637Hu02 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA637Hu01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
MAA637Hu22 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAA637Hu02 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
LAA637Hu71 Biotin-Linked Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC.
Assay Kits SEA637Hu ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA637Mu01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
RPA637Mu02 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA637Mu01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAA637Mu02 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
Assay Kits SEA637Mu ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA637Ra01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA637Ra01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
MAA637Ra21 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
Assay Kits SEA637Ra ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection.
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  16. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
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  19. "Bacterial chitobiase structure provides insight into catalytic mechanism and the basis of Tay-Sachs disease."Nat. Struct. Biol. 3:638-648(1996) [PubMed] [Europe PMC] [Abstract]
  20. "Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease."J. Mol. Biol. 327:1093-1109(2003) [PubMed] [Europe PMC] [Abstract]
  21. "The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease."J. Mol. Biol. 328:669-681(2003) [PubMed] [Europe PMC] [Abstract]
  22. "The biochemistry of HEXA and HEXB gene mutations causing GM2 gangliosidosis."Biochim. Biophys. Acta 1096:87-94(1991) [PubMed] [Europe PMC] [Abstract]
  23. "Molecular basis of an adult form of beta-hexosaminidase B deficiency with motor neuron disease."Biochem. Biophys. Res. Commun. 181:108-115(1991) [PubMed] [Europe PMC] [Abstract]
  24. "A novel exon mutation in the human beta-hexosaminidase beta subunit gene affects 3' splice site selection."J. Biol. Chem. 267:2406-2413(1992) [PubMed] [Europe PMC] [Abstract]
  25. "Molecular basis of an adult form of Sandhoff disease: substitution of glutamine for arginine at position 505 of the beta-chain of beta-hexosaminidase results in a labile enzyme."Biochim. Biophys. Acta 1182:142-146(1993) [PubMed] [Europe PMC] [Abstract]
  26. "A novel missense mutation (C522Y) is present in the beta-hexosaminidase beta-subunit gene of a Japanese patient with infantile Sandhoff disease."Biochem. Biophys. Res. Commun. 212:564-571(1995) [PubMed] [Europe PMC] [Abstract]
  27. "A common beta hexosaminidase gene mutation in adult Sandhoff disease patients."Hum. Genet. 96:417-422(1995) [PubMed] [Europe PMC] [Abstract]
  28. "A second, large deletion in the HEXB gene in a patient with infantile Sandhoff disease."Hum. Mol. Genet. 4:777-780(1995) [PubMed] [Europe PMC] [Abstract]
  29. "Significance of two point mutations present in each HEXB allele of patients with adult GM2 gangliosidosis (Sandhoff disease) homozygosity for the Ile207-->Val substitution is not associated with a clinical or biochemical phenotype."Biochim. Biophys. Acta 1317:127-133(1996) [PubMed] [Europe PMC] [Abstract]
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  32. "A Pro504 --> Ser substitution in the beta-subunit of beta-hexosaminidase A inhibits alpha-subunit hydrolysis of GM2 ganglioside, resulting in chronic Sandhoff disease."J. Biol. Chem. 273:21386-21392(1998) [PubMed] [Europe PMC] [Abstract]