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Cat. Number:

bs-11726R-PE

Quantity size:

100ul

Concentration:

1mg/ml  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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.

Also known as:

Beta hexosaminidase alpha chain precursor; Beta hexosaminidase subunit alpha; Beta N acetylhexosaminidase; Beta N acetylhexosaminidase subunit alpha; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexa; HEXA_HUMAN; Hexosaminidase A (alpha polypeptide); Hexosaminidase A alpha polypeptide; Hexosaminidase A; Hexosaminidase subunit A; MGC99608; N acetyl beta glucosaminidase; N acetyl beta glucosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha; TSD.

Specificity:

Storage:

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

Application:

Excitation spectrum: 496nm,564nm
Emission spectrum: 578nm
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.

Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.