Western Blot (WB)
(Western Blot: Sample: Mouse Testis lysate; Primary Ab: 1ug/ml Rabbit Anti-Mouse HEXa Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
Rabbit anti-Mouse Hexosaminidase A Alpha (HEXa) Polyclonal Antibody | anti-HEXa antibody
Polyclonal Antibody to Hexosaminidase A Alpha (HEXa)
MGSSHHHHHH SSGLVPRGSH MASMTGGQQM GRGSEF- LG GDEVDFTCWK SNPNIQAFMK KKGFTDFKQL ESFYIQTLLD IVSDYDKGYV VWQEVFDNKV KVRPDTIIQV WREEMPVEYM LEMQDITRAG FRALLSAPWY LNRVKYGPDW KDMYKVEPLA FHGTPEQKAL VIGGEACMWG EYVDSTNLVP RLWPRAGAVA ERLWSSNLTT NIDFAFKRLS
Immunocytochemistry in formalin fixed cells: 1:100-500
Immunohistochemistry in formalin fixed frozen section: 1:100-500
Immunohistochemistry in paraffin section: 1:50-200
Enzyme-linked Immunosorbent Assay: 1:100-200
Western Blot (WB)
(Western Blot: Sample: Mouse Testis lysate; Primary Ab: 1ug/ml Rabbit Anti-Mouse HEXa Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
Western Blot (WB)
(Western Blot: Sample: Mouse Testis lysate; Primary Ab: 1ug/ml Rabbit Anti-Mouse HEXa Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin-embedded Liver tissue))
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin-embedded Liver tissue))
NCBI and Uniprot Product Information
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. Mice lacking the encoded protein exhibit accumulation of gangliosides in the brain and membranous cytoplasmic bodies in neurons. Certain mutations in the human ortholog of this gene cause Tay-Sachs disease. [provided by RefSeq, Aug 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: 9 B|9 32.02 cM
Cellular Component: azurophil granule; lysosome; membrane
Molecular Function: acetylglucosaminyltransferase activity; beta-N-acetylhexosaminidase activity; hydrolase activity; hydrolase activity, acting on glycosyl bonds; hydrolase activity, hydrolyzing O-glycosyl compounds; protein heterodimerization activity
Biological Process: adult walking behavior; carbohydrate metabolic process; cell morphogenesis involved in neuron differentiation; ganglioside catabolic process; glycosaminoglycan biosynthetic process; glycosaminoglycan metabolic process; lipid storage; locomotory behavior; lysosome organization and biogenesis; metabolic process; myelination; neuromuscular process controlling balance; neuromuscular process controlling posture; sensory perception of sound; sexual reproduction; skeletal system development
Research Articles on HEXa
Similar Products
Product Notes
The HEXa hexa (Catalog #AAA2016773) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Polyclonal Antibody to Hexosaminidase An Alpha (HEXa) reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's Hexosaminidase An Alpha (HEXa) can be used in a range of immunoassay formats including, but not limited to, Immunocytochemistry (ICC), Immunohistochemistry (IHC) - Formalin/Paraffin, ELISA (EIA), Western Blot (WB). Western blotting: 1:100-400 Immunocytochemistry in formalin fixed cells: 1:100-500 Immunohistochemistry in formalin fixed frozen section: 1:100-500 Immunohistochemistry in paraffin section: 1:50-200 Enzyme-linked Immunosorbent Assay: 1:100-200. 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. The amino acid sequence is listed below: Antigen: The target protein is fused with two N-terminal Tags, His-tag and T7-tag and its sequence is listed below. MGSSHHHHHH SSGLVPRGSH MASMTGGQQM GRGSEF- LG GDEVDFTCWK SNPNIQAFMK KKGFTDFKQL ESFYIQTLLD IVSDYDKGYV VWQEVFDNKV KVRPDTIIQV WREEMPVEYM LEMQDITRAG FRALLSAPWY LNRVKYGPDW KDMYKVEPLA FHGTPEQKAL VIGGEACMWG EYVDSTNLVP RLWPRAGAVA ERLWSSNLTT NIDFAFKRLS. It is sometimes possible for the material contained within the vial of "Hexosaminidase A Alpha (HEXa), Polyclonal Antibody" 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.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
