bs-11726R [Primary Antibody]
HEXA/Beta hexosaminidase subunit alpha Antibody
www.biossusa.com
[email protected]
800.501.7654 [DOMESTIC]
+1.781.569.5821 [INTERNATIONAL]
DATASHEET

Host: Rabbit

Target Protein: HEXA/Beta hexosaminidase subunit alpha

Immunogen Range: 351-420/529


Clonality: Polyclonal

Isotype: IgG

Source: KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.

Background:

Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one chain, one -A chain and one -B chain and is found in the lysosomes of cells. HEXA, along with the cofactor CM2 activator protein, catalyzes the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines in the brain and other tissues. A mutation in the 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.

Size: 100ul

Concentration: 1ug/ul

Applications: ELISA(1:500-1000)
IHC-P(1:200-400)
IHC-F(1:100-500)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)
ICC(1:100-500)

Predicted Molecular Weight: 61, 72


Predicted Cross Reactive Species: Human
Mouse
Rat
Dog
Cow
Sheep
Pig
Horse
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.

PRODUCT SPECIFIC PUBLICATIONS
  • Medini R et al. Expression of β-hexosaminidase in the male reproductive system of the lizard, Eutropis carinata.(Reptilia, Squamata)(Schneider, 1801).Cell Tissue Res. 2018 Nov;374(2):413-421.Read more>>
VALIDATION IMAGES

Paraformaldehyde-fixed, paraffin embedded (human liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (HEXA/Beta) Polyclonal Antibody, Unconjugated (bs-11726R) at 1:500 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.