Full Product Name
HEXA Polyclonal Antibody
Product Gene Name
anti-HEXA antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P06865
Other Notes
Small volumes of anti-HEXA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
NCBI/Uniprot data below describe general gene information for HEXA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000511.2
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NCBI GenBank Nucleotide #
NM_000520.5
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UniProt Primary Accession #
P06865
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UniProt Secondary Accession #
Q53HS8; Q6AI32; B4DKE7; E7ENH7[Other Products]
UniProt Related Accession #
P06865[Other Products]
Molecular Weight
19,326 Da
NCBI Official Full Name
beta-hexosaminidase subunit alpha isoform 2 preproprotein
NCBI Official Synonym Full Names
hexosaminidase subunit alpha
NCBI Official Symbol
HEXA [Similar Products]
NCBI Official Synonym Symbols
TSD
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NCBI Protein Information
beta-hexosaminidase subunit alpha
UniProt Protein Name
Beta-hexosaminidase subunit alpha
UniProt Synonym Protein Names
Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha
UniProt Gene Name
HEXA [Similar Products]
UniProt Synonym Gene Names
Hexosaminidase subunit A [Similar Products]
NCBI Summary for 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]
UniProt Comments for HEXA
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.
Research Articles on HEXA
1. The alpha mutants E482K and G269S are defective in enzymatic activity, unprocessed by lysosomal proteases, and exhibit altered folding pathways compared with wild-type alpha. E482K is more severely misfolded than G269S, as observed by its aggregation and inability to associate with the HexA beta chain. Importantly, both mutants are retrotranslocated from the endoplasmic reticulum to the cytosol and are degraded by the ...
Precautions
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