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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 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)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Hu01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Hu01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Hu01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Hu01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Hu01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Mu01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Mu01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Mu01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Mu01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Mu01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Ra01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Ra01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Ra01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Ra01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Ra01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Ca01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Ca01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Ca01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Ca01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Ca01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Po01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Po01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Po01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Po01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Po01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Bo01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Bo01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Bo01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Bo01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Bo01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Rb01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Rb01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Rb01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Rb01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Rb01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW174Mo01 Recombinant Hexosaminidase B Beta (HEXb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW174Mo01 Monoclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IP.
PAW174Mo01 Polyclonal Antibody to Hexosaminidase B Beta (HEXb) WB; IHC; ICC; IF.
Assay Kits CLW174Mo01 CLIA Kit for Hexosaminidase B Beta (HEXb) CLIA assay for Antigen Detection
ELW174Mo01 ELISA Kit for Hexosaminidase B Beta (HEXb) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

"Isolation of cDNA clones coding for the alpha-subunit of human beta-hexosaminidase. Extensive homology between the alpha- and beta-subunits and studies on Tay-Sachs disease."
J. Biol. Chem. 261:8407-8413(1986) [PubMed] [Europe PMC] [Abstract]

"Characterization of the human HEXB gene encoding lysosomal beta-hexosaminidase."
Genomics 3:279-286(1988) [PubMed] [Europe PMC] [Abstract]

"Gene encoding the human beta-hexosaminidase beta chain: extensive homology of intron placement in the alpha- and beta-chain genes."
Proc. Natl. Acad. Sci. U.S.A. 85:1883-1887(1988) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and comparative analysis of human chromosome 5."
Nature 431:268-274(2004) [PubMed] [Europe PMC] [Abstract]

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Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Synthesis and assembly of a catalytically active lysosomal enzyme, beta-hexosaminidase B, in a cell-free system."
J. Biol. Chem. 263:13463-13469(1988) [PubMed] [Europe PMC] [Abstract]

"Translation initiation in the HEXB gene encoding the beta-subunit of human beta-hexosaminidase."
J. Biol. Chem. 265:20799-20806(1990) [PubMed] [Europe PMC] [Abstract]

"Localization of the pro-sequence within the total deduced primary structure of human beta-hexosaminidase B."
FEBS Lett. 231:47-50(1988) [PubMed] [Europe PMC] [Abstract]

"Characterization of human placental beta-hexosaminidase I2. Proteolytic processing intermediates of hexosaminidase A."
J. Biol. Chem. 265:6794-6799(1990) [PubMed] [Europe PMC] [Abstract]

"The amino-terminal sequences in the pro-alpha and -beta polypeptides of human lysosomal beta-hexosaminidase A and B are retained in the mature isozymes."
FEBS Lett. 249:316-320(1989) [PubMed] [Europe PMC] [Abstract]

"Proteolytic processing of pro-alpha and pro-beta precursors from human beta-hexosaminidase. Generation of the mature alpha and beta a beta b subunits."
J. Biol. Chem. 263:4612-4618(1988) [PubMed] [Europe PMC] [Abstract]

"Isolation of cDNA clones coding for the beta subunit of human beta-hexosaminidase."
Proc. Natl. Acad. Sci. U.S.A. 82:1184-1188(1985) [PubMed] [Europe PMC] [Abstract]

"Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase."
Biochemistry 27:5216-5226(1988) [PubMed] [Europe PMC] [Abstract]

"Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry."
Nat. Biotechnol. 21:660-666(2003) [PubMed] [Europe PMC] [Abstract]

"Complete analysis of the glycosylation and disulfide bond pattern of human beta-hexosaminidase B by MALDI-MS."
Glycobiology 11:549-556(2001) [PubMed] [Europe PMC] [Abstract]

"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]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"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]

"The biochemistry of HEXA and HEXB gene mutations causing GM2 gangliosidosis."
Biochim. Biophys. Acta 1096:87-94(1991) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"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]

"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]

"A common beta hexosaminidase gene mutation in adult Sandhoff disease patients."
Hum. Genet. 96:417-422(1995) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"Molecular basis of heat labile hexosaminidase B among Jews and Arabs."
Hum. Mutat. 10:424-429(1997) [PubMed] [Europe PMC] [Abstract]

"Two mutations remote from an exon/intron junction in the beta-hexosaminidase beta-subunit gene affect 3'-splice site selection and cause Sandhoff disease."
Hum. Genet. 103:462-469(1998) [PubMed] [Europe PMC] [Abstract]

"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]