Human Hexosaminidase A/HEXA antibody

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.

Product Specifications

Product Name Alternative

Beta-hexosaminidase subunit alpha, TSD, hexosaminidase A

Host

Mouse

Antigen Species

Human

Reactivity

Human

Immunogen

Recombinant human HEXA (89-529aa) purified from E. coli

Clonality

Monoclonal

Isotype

IgG2a λ

Clone

AT20F1

Conjugation

Unconjugated

Applications

ELISA, WB, FACS

Purification Method

By protein-A affinity chromatography

Concentration

1 mg/mL (determined by BCA assay)

Additionnal Information

Hexosaminidase A, HEXA, ATGA0259-10 µg, ATGA0259-20 µg, ATGA0259-50 µg, ATGA0259-100 µg, ATGA0259-250 µg, ATGA0259-500 µg, ATGA0259-1 mg, ATGA0259-10, ATGA0259-20, ATGA0259-50, ATGA0259-100, ATGA0259-250, ATGA0259-500, ATGA0259-1

References & Citations

Cabezas. J.A. (1989) Biochem J 261 (3) : 1059-1060.; ; Lemieux. M.J., et al. (2006) J Mol Biol 359 (4) : 913-929.; ; Boles. D.J. and Proia. R.L. (1995) Am J Hum Genet 56 (3) : 716-724.

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol

Applications Notes

The antibody has been tested by ELISA, Western blot and FACS analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Scientific Category

Signal Transduction

NCBI Accession Number

NP_000511.1

Uniprot Accession Number

P06865

WB Description

The cell lysate of MCF7 (40ug) was resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human HEXA antibody (1:3000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system. The cell lysates (10 µg) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human HEXA antibody (1:3000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system.; ; Lane 1.: 293T cell lysate; ; Lane 2.: HEXA Transfected 293T cell lysate {ATGA0259-WB2.jpg}

FACS Description

Flow cytometry analysis of HEXA in A549 cell line, staining at 2-5ug for 1x10^6cells (red line) . The secondary antibody used goat anti-mouse IgG Alexa fluor 488 conjugate. Isotype control antibody was mouse IgG (black line) .