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SDS-PAGE

Hexosaminidase A / HEXA Recombinant Protein | HEXA recombinant protein

Recombinant Human Hexosaminidase A / HEXA Protein (Subunit A, His tag)

Gene Names
HEXA; TSD
Purity
> 95 % as determined by SDS-PAGE
Synonyms
Hexosaminidase A / HEXA; Recombinant Human Hexosaminidase A / HEXA Protein (Subunit A; His tag); MGC99608; TSD; HEXA recombinant protein
Ordering
For Research Use Only!
Host
Baculovirus-Insect Cells
Purity/Purification
> 95 % as determined by SDS-PAGE
Form/Format
Lyophilized from sterile 20mM Tris, 500mM NaCl, pH 7.4
Sequence Length
529
Application Notes
The recombinant human HEXA consists of 517 amino acids and predicts a molecular mass of 59.7 kDa. It migrates as an approximately 64 kDa band in SDS-PAGE under reducing conditions.
Predicted N Terminal
Leu 23
Endotoxin
< 1.0 EU per mug of the protein as determined by the LAL method
Preparation and Storage
Samples are stable for up to twelve months from date of receipt at -70 degree C

SDS-PAGE

SDS-PAGE
Related Product Information for HEXA recombinant protein
A DNA sequence encoding the human HEXA (AAD13932.1) (Met 1-Thr 529) was fused with a polyhistidine tag at the C-terminus.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
Molecular Weight
19,326 Da
NCBI Official Full Name
lysosomal enzyme beta-N-acetylhexosaminidase A
NCBI Official Synonym Full Names
hexosaminidase subunit alpha
NCBI Official Symbol
HEXA
NCBI Official Synonym Symbols
TSD
NCBI Protein Information
beta-hexosaminidase subunit alpha
UniProt Protein Name
Beta-hexosaminidase subunit alpha
Protein Family
UniProt Gene Name
HEXA
UniProt Synonym Gene Names
Hexosaminidase subunit A

NCBI Description

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 Description

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. Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1); 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). Belongs to the glycosyl hydrolase 20 family.

Protein type: Carbohydrate Metabolism - amino sugar and nucleotide sugar; EC 3.2.1.52; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - other glycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 15q23

Cellular Component: azurophil granule; lysosomal lumen; membrane

Molecular Function: acetylglucosaminyltransferase activity; beta-N-acetylhexosaminidase activity; protein heterodimerization activity

Biological Process: chondroitin sulfate catabolic process; glycosaminoglycan biosynthetic process; glycosphingolipid metabolic process; hyaluronan catabolic process; keratan sulfate catabolic process

Disease: Tay-sachs Disease

Research Articles on HEXA

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Product Notes

The HEXA hexa (Catalog #AAA2545596) is a Recombinant Protein produced from Baculovirus-Insect Cells and is intended for research purposes only. The product is available for immediate purchase. The recombinant human HEXA consists of 517 amino acids and predicts a molecular mass of 59.7 kDa. It migrates as an approximately 64 kDa band in SDS-PAGE under reducing conditions. Researchers should empirically determine the suitability of the HEXA hexa for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Hexosaminidase A / HEXA, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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