HEXA/Beta hexosaminidase subunit alpha Antibody, ALEXA FLUOR 555

Catalog numberbs-11726R-A555
NameHEXA/Beta hexosaminidase subunit alpha Antibody, ALEXA FLUOR 555
Price€ 332.75
Size100ul
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Long nameHEXA/Beta hexosaminidase subunit alpha Antibody, ALEXA FLUOR 555 Conjugated
Also known asAnti-HEXA/Beta hexosaminidase subunit alpha PAb ALEXA FLUOR 555
CategoryConjugated Primary Antibodies
Conjugated withALEXA FLUOR® 555
Host OrganismRabbit (Oryctolagus cuniculus)
Target AntigenHEXA/Beta hexosaminidase subunit alpha
SpecificityThis is a highly specific antibody against HEXA/Beta hexosaminidase subunit alpha.
ModificationUnmodified
Modification SiteNone
ClonalityPolyclonal
ClonePolyclonal antibody
Concentration1ug per 1ul
SourceThis antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha
Tested applicationsIF(IHC-P)
Recommended dilutionsIF(IHC-P)(1:50-200)
CrossreactivityHuman, Mouse, Rat
Cross-reactive species detailsDue to limited amount of testing and knowledge, not every possible cross-reactivity is known.
Background of the antigenHexosaminidase 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.
PurificationPurified by Protein A.
Storage conditionsStore 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.
Excitation emission553nm/568nm
SynonymsBeta 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.
PropertiesFor facs or microscopy Alexa 1 conjugate.Very high photo stable ALEXA conjugate.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
ConjugationAlexa Fluor,ALEXA FLUOR 555
DescriptionThe HEXA/Beta hexosaminidase subunit alpha Antibody, is a α- or alpha protein sometimes glycoprotein present in blood.
French translationanticorps