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HEXB

hexosaminidase B (beta polypeptide)

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.

Gene Name: hexosaminidase B (beta polypeptide)
Synonyms: HEXB, Beta-hexosanimidase, Beta-N-acetylhexosaminidase, Beta-hexosaminidase beta chain, HCC-7, Hexosaminidase subunit B, N-acetyl-beta- glucosaminidase, ENC-1AS
Target Sequences: NM_000521 NP_000512.1 P07686

Publications (1)

1
Novel bicistronic lentiviral vectors correct ß-Hexosaminidase deficiency in neural and hematopoietic stem cells and progeny: implications for in vivo and ex vivo gene therapy of GM2 gangliosidosis. Francesca Ornaghi, Davide Sala, Fabiana Tedeschi, Maria Chiara Maffia, Martina Bazzucchi, Francesco Morena, Manuela Valsecchi, Massimo Aureli, Sabata Martino, Angela Gritti. Neurobiology of disease. 2020 February;134:104667. [Full Text Article] [PubMed:31682993] Related Antibodies: LS-C354001.

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Proteins (10)
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HEXB (10)
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Myc-DDK (Flag) (3)
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Human Cells (1)
Wheat Germ Extract (1)
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293T Cells
Myc-DDK (Flag)
62.9 kDa
20 µg/$150
HEXB Protein - Recombinant Hexosaminidase B Beta By SDS-PAGE
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E. coli
His
22.9 kDa
10 µg/$285; 50 µg/$470; 100 µg/$750; 200 µg/$937; 1 mg/$2,063; 500 µg/$1,594; 5 mg/$3,277; 2 mg/$2,252
HEXB Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
62.9 kDa
100 µg/$494
HEXB Protein - Recombinant Hexosaminidase B Beta By SDS-PAGE
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E. coli
His-T7
17.1 kDa
10 µg/$292; 50 µg/$512; 100 µg/$817; 200 µg/$1,018; 1 mg/$2,168; 500 µg/$1,690; 5 mg/$3,571; 2 mg/$2,359
HEXB Protein - 12.5% SDS-PAGE of human HEXB stained with Coomassie Blue
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Wheat Germ Extract
GST
89.5 kDa
2 µg/$439
HEXB Protein - Purified recombinant protein HEXB was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
62.9 kDa
20 µg/$1,107
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Human Cells
His
Less than 1.0 EU/µg protein (determined by LAL method).
59.4 kDa
100 µg/$843
HEXB Protein - Recombinant Hexosaminidase B Beta By SDS-PAGE
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E. coli
His
17.8 kDa
10 µg/$285; 50 µg/$470; 100 µg/$750; 200 µg/$937; 1 mg/$2,063; 500 µg/$1,594; 5 mg/$3,277; 2 mg/$2,252
HEXB Protein - Recombinant Hexosaminidase B Beta By SDS-PAGE
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E. coli
His
14.7 kDa
10 µg/$287; 50 µg/$482; 100 µg/$770; 200 µg/$962; 1 mg/$2,094; 500 µg/$1,623; 5 mg/$3,361; 2 mg/$2,284
HEXB Protein - Recombinant Hexosaminidase B Beta By SDS-PAGE
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E. coli
His
17.9 kDa
10 µg/$287; 50 µg/$482; 100 µg/$770; 200 µg/$962; 1 mg/$2,094; 500 µg/$1,623; 5 mg/$3,361; 2 mg/$2,284
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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).