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Item number | Size | Datasheet | Manual | SDS | Delivery time | Quantity | Price |
---|---|---|---|---|---|---|---|
ELK-ES3963.50 | 50 µl | - | - |
Request delivery time estimate |
169.00€
|
||
ELK-ES3963.100 | 100 µl | - | - |
Request delivery time estimate |
283.00€
|
If you have any questions, please use our Contact Form.
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded... more
Product information "Anti-HEXA"
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], Protein function: Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides (PubMed:11707436, PubMed:9694901, PubMed:8672428, PubMed:8123671). The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6- sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2 (PubMed:11707436). The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide (PubMed:11707436). Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A (PubMed:9694901, PubMed:8672428, PubMed:8123671). [The UniProt Consortium] Recommended dilutions: Western Blot: 1/500 - 1/2000. IHC-p: 1:100-300 ELISA: 1/20000. Not yet tested in other applications.. Cellular localization: Lysosome.
Keywords: | Anti-Hexosaminidase subunit A, Anti-Beta-hexosaminidase subunit alpha, Anti-Beta-N-acetylhexosaminidase subunit alpha, Anti-N-acetyl-beta-glucosaminidase subunit alpha, HEXA rabbit pAb |
Supplier: | ELK Biotechnology |
Supplier-Nr: | ES3963 |
Properties
Application: | WB, IHC, IF, ELISA |
Antibody Type: | Polyclonal |
Conjugate: | No |
Host: | Rabbit |
Species reactivity: | human, mouse, rat |
Immunogen: | Synthesized peptide derived from HEXA . at AA range: 121-170 |
MW: | 60 kD |
Format: | Purified |
Database Information
KEGG ID : | K12373 | Matching products |
UniProt ID : | P06865 | Matching products |
Gene ID | GeneID 3073 | Matching products |
Handling & Safety
Storage: | -20°C |
Shipping: | +4°C (International: +4°C) |
Caution
Our products are for laboratory research use only: Not for administration to humans!
Our products are for laboratory research use only: Not for administration to humans!
You will get a certificate here
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