Anti-HEXA antibody (bs-11726R)

Anti-HEXA antibody

DATASHEET

Host:Rabbit

Target Protein:HEXA

IR:Immunogen Range:351-420/529

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:3073

Swiss Prot:P06865

Source:KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha:351-420/529 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C 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:1mg/ml

Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)

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

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