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Hexb (S516) Peptide

Hexb (S516) Peptide

Cat no: BS1917P


Supplier: Bioworld Technology, Inc.
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Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a Hexosaminidase B (HEXB), also designated b-hexosaminidase B, is a tetramer of two b-A and two b-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the b subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.
Catalogue number: BS1917P
Applications: Block/Neutralize/Inhibit
Swiss prot: P07686
Form: 1 mg/ml in DI water.
Purity: Synthetic peptide Hexb (S516). (Note: the amino acid sequence is proprietary). The purity is > 98%.
Storage temp: Store at 4 degree C short term. Aliquot and store at -20 degree C long term. Avoid freeze-thaw cycles.
Alternative names: ENC 1AS; HCC 7; HCC-7; HCC7; HEX B; Hexb; HEXB; Hexosaminidase B (beta polypeptide); Hexosaminidase B; Hexosaminidase subunit B; HexosaminidaseB;
Additional info: This peptide can be used with studies using BS1917 Hexb (S516) pAb.

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