Anti-HEXA Rabbit pAb
- 100ug (NP20314) Please inquiry
Antibody type:Primary antibody
Label:Unconjugated
Modification:Unmodification
Isotype:Rabbit IgG
Host:Rabbit
Application:WB,IHC
Purify method:Affinity purified
Species:Human,Mouse,Rat
Gene Name:HEXA
Synonyms:TSD
Gene Synonyms:
Gene Full Name:hexosaminidase subunit alpha
Gene Infomation:This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]
Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 270-529 of human HEXA (NP_000511.2).
Antigen Synonyms:Beta-hexosaminidase subunit alpha
Clonality:Polyclonal antibody
Source:Human
Reaction:
Form:Liquid
Tested Applications:
- Western blot (1:100 to 1:500)
- Immunofluorescence (1:50 to 1:400)
- Immunohistochemistry (1:200 to 1:500)
- Flow cytometry analysis (1:200 to 1:500)
- Enzyme-linked Immunosorbent Assay (1:100-1:5000)
Clone:
Dilution:WB1:500 -1:2000; IHC1:50 -1:200(Optimal dilutions should be determined by the end user)
Mole Mass:19kDa/60kDa
Location:Lysosome
Concentration:
Sequence Similarity:
Gene Id:
SwissProt ID:P06865
Unigene:3073
Nucleotide Accession:
Tissue specificity:
Storage:Store at -20°C. Avoid freeze / thaw cycles.
Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Background:
Molar Function:Responsible 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.
Western Blot:Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.