Product nameAnti-HEXA antibody - C-terminal
See all HEXA primary antibodies
DescriptionRabbit polyclonal to HEXA - C-terminal
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Mouse, Rat, Human
Predicted to work with: Cow, Orangutan
Recombinant fragment corresponding to Human HEXA aa 270-529 (C terminal).
IPGLLTPCYSGSEPSGTFGPVNPSLNNTYEFMSTFFLEVSSVFPDFYLHL GGDEVDFTCWKSNPEIQDFMRKKGFGEDFKQLESFYIQTLLDIVSSYGKG YVVWQEVFDNKVKIQPDTIIQVWREDIPVNYMKELELVTKAGFRALLSAP WYLNRISYGPDWKDFYIVEPLAFEGTPEQKALVIGGEACMWGEYVDNTNL VPRLWPRAGAVAERLWSNKLTSDLTFAYERLSHFRCELLRRGVQAQPLNV GFCEQEFEQT
Database link: P06865
- WB: MCF7, BT474, SW620, HepG2 and HT29 cell lysates; mouse testis and brain tissue lysates.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.3
Preservative: 0.02% Sodium azide
Constituents: 49% PBS, 50% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab189865 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/2000. Predicted molecular weight: 61 kDa.|
FunctionResponsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
Involvement in diseaseDefects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1) [MIM:272800]; also known as Tay-Sachs disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G1 is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. GM2G1 has an increased incidence among Ashkenazi Jews and French Canadians in eastern Quebec. It exists in several forms: infantile (most common and most severe), juvenile and adult (late onset).
Sequence similaritiesBelongs to the glycosyl hydrolase 20 family.
modificationsN-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
- Information by UniProt
- Beta hexosaminidase alpha chain precursor antibody
- Beta hexosaminidase subunit alpha antibody
- Beta N acetylhexosaminidase antibody
All lanes : Anti-HEXA antibody - C-terminal (ab189865) at 1/1000 dilution
Lane 1 : MCF-7 cell lysate
Lane 2 : BT-474 cell lysate
Lane 3 : SW620 cell lysate
Lane 4 : HepG2 cell lysate
Lane 5 : HT-29 cell lysate
Lane 6 : Mouse testis tissue lysate
Lane 7 : Mouse brain tissue lysate
Lysates/proteins at 25 µg per lane.
All lanes : HRP Goat Anti-Rabbit IgG (H+L)
Developed using the ECL technique.
Predicted band size: 61 kDa
Exposure time: 5 seconds
Blocking buffer: 3% nonfat dry milk in TBST.
ab189865 has not yet been referenced specifically in any publications.