Conjugated Primary Antibodies
Rabbit (Oryctolagus cuniculus)
HEXA/Beta hexosaminidase subunit alpha
This is a highly specific antibody against HEXA/Beta hexosaminidase subunit alpha.
1ug per 1ul
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha
Human, Mouse, Rat
Cross-reactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
Background of the antigen
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.
Purified by Protein A.
Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
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.
Anti-HEXA/Beta hexosaminidase subunit alpha
Immunoglobulin G (IgG)