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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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HEXB (14)
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96-Well Strip Plate (13)
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Adherent Cell Cultures (1)
Cell Culture Supernatants (1)
Plasma (11)
Serum (11)
Tissue Homogenates (8)
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HEXB ELISA Kit
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Sandwich
96-Well Strip Plate
Human
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$764
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Mouse
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$782
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Human
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum
1 Plate/$669
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Mouse
0.313 - 20 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$587
HEXB ELISA Kit
Select
Cell-Based
96-Well Microplate
Human
Colorimetric - 450nm (TMB)
Adherent Cell Cultures
1 Plate/$534
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Rat
1.56 - 100 U/ml
Colorimetric - 450nm (TMB)
Plasma, Serum
1 Plate/$669
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Rat
0.781 - 50 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum
1 Plate/$586
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Human
1.56 - 100 IU/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$579
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Rat
1.56 - 100 IU/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$579
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Rat
0.313 - 20 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$579
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Rat
1.56 - 100 mIU/ml
Colorimetric - 450nm (TMB)
Cell Culture Supernatants, Plasma, Serum, Tissue Homogenates, Urine
1 Plate/$897
HEXB ELISA Kit
Select
Custom
96-Well Strip Plate
Pig
Colorimetric - 450nm (TMB)
1 Plate/$669
HEXB ELISA Kit
Select
Custom
96-Well Strip Plate
Mouse
Colorimetric - 450nm (TMB)
1 Plate/$669
HEXB ELISA Kit
Select
Sandwich
96-Well Strip Plate
Rat
1.56 - 100 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum, Tissue Homogenates
1 Plate/$715
Viewing 1-14 of 14 product results


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If you do not find the reagent or information you require, please contact Customer.Support@LSBio.com to inquire about additional products in development.

PLEASE NOTE

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).