Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one å chain, one âˆ«-A chain and one âˆ«-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 å 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.
Crossreactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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.
This antibody was purified via Protein A.
Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
Conjugated Primary Antibodies
KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha
This is a highly specific antibody against HEXA/Beta hexosaminidase subunit alpha.
Human, Mouse, Rat
1ug per 1ul
HEXA/Beta hexosaminidase subunit alpha
If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C. Cy5 antibodies are excited by the 650-nanometer wave of an argon laser and detected at a 670-nanometer captor.
These antibodies are excite for emission at 678nm and detected at a 694 nm wavelengths.
Scope note:Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.