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AB313971

Human HEXA ELISA Kit

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Human HEXA ELISA Kit is a Sandwich (quantitative) ELISA for the measurement of Human HEXA in Human in Biofluids, Cell Culture Media samples.

View Alternative Names

Beta-hexosaminidase subunit alpha, Beta-N-acetylhexosaminidase subunit alpha, N-acetyl-beta-glucosaminidase subunit alpha, Hexosaminidase subunit A, HEXA

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Sandwich ELISA - Human HEXA ELISA Kit (AB313971)
  • sELISA

Supplier Data

Sandwich ELISA - Human HEXA ELISA Kit (AB313971)

Example of standard curve using ab313971. This standard curve is for demonstrative purposes only. A standard curve must be run with each assay.

Key facts

Detection method

Colorimetric

Sample types

Serum, Plasma, Cell culture supernatant

Reacts with

Human

Assay type

Sandwich (quantitative)

Sensitivity

= 0.082 ng/mL

Range

0.082 - 20 ng/mL

Assay Platform

Pre-coated microplate (12 x 8 well strips)

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "sELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Product details

Human HEXA ELISA Kit is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of human HEXA in serum, plasma and cell culture supernatants. This assay employs an antibody specific for human HEXA coated on a 96-well plate.
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Recovery

[ { "sample": "Serum", "range": "67 - 105 %", "average": "= 85.6" }, { "sample": "Plasma", "range": "69 - 91 %", "average": "= 79.45" }, { "sample": "Cell culture media", "range": "68 - 84 %", "average": "= 75.64" } ]
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What's included?

{ "values": { "1x96Tests": { "sellingSize": "1 x 96 Tests", "publicAssetCode":"ab313971-1x96Tests", "assetComponentDetails": [ { "size":"1 x 1 Unit", "name":"Human HEXA Antibody-coated ELISA Plate", "number":"AB313971-CMP08", "productcode":"" }, { "size":"1 x 25 mL", "name":"20X Wash Buffer", "number":"AB313971-CMP04", "productcode":"" }, { "size":"1 x 15 mL", "name":"5X Assay Diluent (Item E2)", "number":"AB313971-CMP05", "productcode":"" }, { "size":"2 x 1 Vial", "name":"Biotinylated Human HEXA Detection Antibody", "number":"AB313971-CMP09", "productcode":"" }, { "size":"2 x 1 Vial", "name":"Lyophilized Human HEXA Protein Standard", "number":"AB313971-CMP10", "productcode":"" }, { "size":"1 x 12 mL", "name":"TMB One-Step Substrate Reagent", "number":"AB313971-CMP07", "productcode":"" }, { "size":"1 x 200 µL", "name":"150X HRP-Streptavidin", "number":"AB313971-CMP03", "productcode":"" }, { "size":"1 x 8 mL", "name":"Stop Solution; 0.2M Sulfuric Acid", "number":"AB313971-CMP06", "productcode":"" } ] } } }
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Properties and storage information

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Storage information
-20°C
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

The HEXA gene codes for the enzyme Hexosaminidase A also known as hexosaminidase alpha or HEXA subunit. This enzyme has several subunits with a molecular mass of approximately 58 kDa. HEXA expresses in various tissues but it shows high expression in the brain and other neural tissues. Mechanically Hexosaminidase A is involved in the hydrolysis of GM2 gangliosides into GM3 by removing the N-acetylgalactosamine residue which plays a role in the degradation of glycolipids within lysosomes.
Biological function summary

Hexosaminidase A functions as part of the lysosomal enzyme complex partnering with the GM2 activator protein and another related enzyme Hexosaminidase B. The complex is critical in the catabolism of GM2 gangliosides a type of lipid found in cell membranes specifically in neuronal cell membranes. Efficient function of Hexosaminidase A prevents accumulation of the lipid ensuring cellular health particularly in neurons.

Pathways

Hexosaminidase A is significant within glycolipid metabolism pathways and lysosomal degradation pathways. It cooperates closely with enzymes like Hexosaminidase B and the GM2 activator protein within these processes. These pathways are important for the breakdown of gangliosides which prevents their accumulation and maintains cellular function in neuronal tissues.

HEXA mutations have a direct link to Tay-Sachs disease a severe genetic disorder affecting nerve cells. This disorder results from HEXA mutations causing deficient enzyme activity leading to GM2 ganglioside accumulation. Additionally Sandhoff disease links through similar pathways though its relation more involves Hexosaminidase B deficiencies. Both disorders highlight the importance of a functioning Hexosaminidase A enzyme for normal neurological function.
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Product protocols

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Target data

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 : 8123671, PubMed : 8672428, PubMed : 9694901). 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 : 8123671, PubMed : 8672428, PubMed : 9694901).
See full target information HEXA
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Complementary Products

Primary Antibodies

AB189865

Anti-HEXA antibody - C-terminal

Western blot - Anti-HEXA antibody - C-terminal (AB189865)

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