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    HEXA/Beta hexosaminidase subunit alpha Antibody, ALEXA FLUOR® 555 Conjugated

    Overview
    Catalog # bs-11726R-A555
    Product Name HEXA/Beta hexosaminidase subunit alpha Antibody, ALEXA FLUOR® 555 Conjugated
    Applications IF(IHC-P)
    Reactivity Human, Mouse, Rat
    Specifications
    Conjugation ALEXA FLUOR® 555
    Host Rabbit
    Source KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha
    Clonality Polyclonal
    Isotype IgG
    Concentration 1ug/ul
    Purification Purified by Protein A.
    Storage Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
    Target
    Synonyms 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.
    Background Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one å 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 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.