Description:

    Size: 100 microliters

    Catalog no.: GENTObs-11726R-A594

    Price: 489 EUR

    Product details

    Subcellular locations

    N/A

    Gene ID

    N/A

    Swiss Prot

    N/A

    Modification site

    None

    Applications

    IF(IHC-P)

    Concentration

    1ug per 1ul

    Excitation emission

    590nm/617nm

    Conjugated

    Alexa conjugate 1

    Conjugated with

    ALEXA FLUOR® 594

    Applications with corresponding dilutions

    IF(IHC-P)(1:50-200)

    Clonality

    Polyclonal Antibody

    Clone

    Polyclonal Antibodies

    Purification method

    Purified by Protein A.

    Type

    Conjugated Primary Antibody

    Conjugation

    Alexa Fluor,ALEXA FLUOR® 594

    Host organism

    Rabbit (Oryctolagus cuniculus)

    Target Protein/Peptide

    HEXA/Beta hexosaminidase subunit alpha

    Properties

    For facs or microscopy Alexa 1 conjugate.

    Other name

    Anti-HEXA/Beta hexosaminidase subunit alpha

    Also known as

    HEXA/Beta hexosaminidase subunit alpha Antibody

    Modification

    No modification has been applied to this antibody

    Cross reactive species

    Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus)

    Specificity

    This antibody reacts specifically with HEXA/Beta hexosaminidase subunit alpha

    Antigen Source

    KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha

    Storage

    Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.

    Cross Reactive Species details

    No significant cross reactivity has been observed for this antibody for the tested species. However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species.

    Description

    The Anti-HEXA/Beta hexosaminidase subunit alpha is a α- or alpha protein sometimes glycoprotein present in blood.This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided.

    Advisory

    Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody, thus reducing its reactivity, specificity and sensitivity. For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube. Prior to use briefly centrifuge the vial to gather all the solution on the bottom.

    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 information

    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.