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Conditions of optimal HEXA Antibody performance should be determined experimentally by the investigator.
Price/availability/specifications subject to change without notice. Unless otherwise indicated, our catalog and customized products are for research use only and not intended for human or animal diagnostic or therapeutic use.
Phone: 1-617-401-8149
Fax: 1-617-606-5019
Email: message@sydlabs.com
Or leave a message with a formal purchase
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Background
HEXA(hexosaminidase A (alpha polypeptide)) is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines The HEXA gene encodes the alpha subunit of hexosaminidase A , a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit (amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.
PA006533-PA1787: HEXA Polyclonal Antibody
Rabbit Polyclonal Antibody.
Applications: WB: 0.1-0.5 ug/ml IHC-P: 0.5-1 ug/ml
Reactivity: Humsn, Rat, Mouse.
Immunogen: A synthetic peptide corresponding to a sequence in the middle region of human HEXA, different from the related rat and mouse sequences by one amino acid.
Purification: Immunogen affinity purified.
Form of Atibody: lyophilized.
Storage/Stability: Stable for 1 year at -20°C and 3 months at 4°C. For maximum recovery of the product, centrifuge the original vial after thawing and before removing the cap. Aliquot to avoid repeated freezing and thawing.
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