HEXA, Monoclonal Antibody

Mouse monoclonal HEXA antibody; Monoclonal HEXA antibody; Anti HEXA antibody; beta-hexosaminidase subunit alpha antibody; TSD antibody; hexosaminidase A antibody

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2a lambda

AT20F1

Mouse

Purified by Protein A affinity chromatography

Liquid form in Phosphate-Buffered Saline (pH 7.4) containing 10% Glycerol,0.02% Sodium Azide

1 mg/ml (lot specific)

Small volumes of anti-HEXA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

HEXA (Hexosaminidase A), also designated beta-Hexosaminidase A, is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. A mutation in the a subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.

Signal Transduction

ELISA (EIA), Western Blot (WB)

ELISA: 1:3000
WB: 1:3000

The cell lysates of MCF7 (40 ug) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with HEXA antibody (1:3000)

19,326 Da

hexosaminidase subunit alpha

beta-hexosaminidase subunit alpha

Beta-hexosaminidase subunit alpha

Hexosaminidase subunit A  [Similar Products]

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]

Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.

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