Human Beta-hexosaminidase subunit alpha(HEXA) ELISA kit

Code CSB-EL010315HU
Size 96T,5×96T,10×96T How to order?
Trial Size 24T ELISA kits trial application
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Product Details

Description

This Human HEXA ELISA Kit was designed for the quantitative measurement of Human HEXA protein in serum, plasma, tissue homogenates, cell lysates. It is a Sandwich ELISA kit, its detection range is 15.6 pg/mL-1000 pg/mL and the sensitivity is 3.9 pg/mL.

Target Name hexosaminidase A (alpha polypeptide)
Alternative Names Beta hexosaminidase alpha chain precursor ELISA Kit; Beta hexosaminidase subunit alpha ELISA Kit; Beta N acetylhexosaminidase ELISA Kit; Beta N acetylhexosaminidase subunit alpha ELISA Kit; Beta-hexosaminidase A ELISA Kit; Beta-hexosaminidase subunit alpha ELISA Kit; Beta-N-acetylhexosaminidase subunit alpha ELISA Kit; Hexa ELISA Kit; HEXA_HUMAN ELISA Kit; Hexosaminidase A (alpha polypeptide) ELISA Kit; Hexosaminidase A alpha polypeptide ELISA Kit; Hexosaminidase A ELISA Kit; Hexosaminidase subunit A ELISA Kit; MGC99608 ELISA Kit; N acetyl beta glucosaminidase ELISA Kit; N acetyl beta glucosaminidase subunit alpha ELISA Kit; N-acetyl-beta-glucosaminidase subunit alpha ELISA Kit; TSD ELISA Kit
Abbreviation HEXA
Uniprot No. P06865
Species Homo sapiens (Human)
Sample Types serum, plasma, tissue homogenates, cell lysates
Detection Range 15.6 pg/mL-1000 pg/mL
Sensitivity 3.9 pg/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Neuroscience
Assay Principle quantitative
Measurement Sandwich
Precision
Intra-assay Precision (Precision within an assay): CV%<8%        
Three samples of known concentration were tested twenty times on one plate to assess.    
Inter-assay Precision (Precision between assays): CV%<10%        
Three samples of known concentration were tested in twenty assays to assess.      
               
Linearity
To assess the linearity of the assay, samples were spiked with high concentrations of human HEXA in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.  
  Sample Serum(n=4)    
1:1 Average % 101    
Range % 94-106    
1:2 Average % 97    
Range % 91-103    
1:4 Average % 93    
Range % 87-99    
1:8 Average % 88    
Range % 85-92    
Recovery
The recovery of human HEXA spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.  
 
Sample Type Average % Recovery Range    
Serum (n=5) 98 93-102    
EDTA plasma (n=4) 87 80-92    
               
               
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.  
 
pg/ml OD1 OD2 Average Corrected    
1000 2.245 2.164 2.205 2.053    
500 1.407 1.295 1.351 1.199    
250 0.832 0.812 0.822 0.670    
125 0.485 0.499 0.492 0.340    
62.5 0.334 0.316 0.325 0.173    
31.2 0.231 0.226 0.229 0.077    
15.6 0.192 0.199 0.196 0.044    
0 0.152 0.151 0.152      
ELISA Data Analysis Watch ELISA data processing video & download Curve Expert if needed
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 3-5 working days

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Target Background

Function
(From Uniprot)
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. 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. The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide. Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A.
Gene References into Functions
  1. Reported data present, for the first time, reference values for urinary activities of HEX and its isoenzymes HEX A and HEX B in children and adolescent. PMID: 28846871
  2. 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 ... PMID: 27682588
  3. reports a new missense mutation in the HEXA gene in two German siblings with late-onset Tay-Sachs disease and prominent psychiatric symptoms PMID: 25860343
  4. The silencing of the HEXA gene had a stronger immune inhibitory effect, thereby indicating a major involvement of beta-N-acetyl-hexosaminidase A isoenzyme within this mechanism. PMID: 21997228
  5. Human prostate cancer cells are characterised by a significant decrease in HexA activity. PMID: 24389457
  6. DNA reveals novel mutations in Iranian subjects causing Tay-Sachs disease in the alpha and beta subunits of HexA. PMID: 24518553
  7. GM2 gangliosidosis is caused by the gene mutation. (review) PMID: 23370522
  8. Identification of six novel missense mutations in children affected with Tay Sachs disease from India. PMID: 22723944
  9. identified 27 different mutations, 14 of which were novel, in the HEXA gene and 14 different mutations, 8 of which unreported until now, in the HEXB gene, and attempted to correlate these mutations with the clinical presentation of the patients PMID: 22789865
  10. MtsD, MtsF and MtsH are fusion proteins with a methyltransferase domain and a corrinoid-binding domain. PMID: 19732345
  11. HEXA gene in Argentinean patients affected with Tay-Sachs disease, overall 14 different mutations were identified, 8 of them were novel and lead to premature stop codons, drastic residues changes or a splicing defect. PMID: 22441121
  12. Beta-hexosaminidase over-expression affects lysosomal glycohydrolases expression and glycosphingolipid metabolism in mammalian cells. PMID: 22147196
  13. We report the first Jordanian Arab Tay-Sachs disease patient diagnosed by deficient beta-hexosaminidase A activity, mutation analysis revealed homozygosity for a nonsense HEXA mutation, c.78G>A (p.W26X) PMID: 21967858
  14. Two mutations were identified c.1A>G (p.MIV), which obliterated the initiating methionine in codon 1, and c.1177C>T (p.R393X), which predicted a termination codon or nonsense mutation in infantile Tay-Sachs diseae in the Persian population. PMID: 21796138
  15. Down-regulation of beta-N-acetyl-D-glucosaminidase increases Akt1 activity in thyroid anaplastic cancer cells PMID: 21637923
  16. Detected mutations in the HEXA gene through gene sequencing and, by combining the HEXA enzyme assay and the HEXA gene sequencing assay, were able to clarify Tay Sachs carrier status. PMID: 19858779
  17. Eight novel mutations PMID: 12180151
  18. plasma activity of total Hex does not appear to be a reliable marker of erosion and cartilage degradation in rheumatoid arthritis patients; liver function appears to be the major determinant for the plasma Hex activity in these patients PMID: 12413610
  19. second novel HEXA mutation specific to the IJ TDS carriers: a substitution of cytosine 1351 by guanosine (C1351G), resulting in the change of leucine to valine in position 451. PMID: 14648242
  20. the founder effect in a rapidly expanding population arising from a bottleneck provides a robust parsimonious hypothesis explaining the spread of 1278insTATC-linked TSD in Ashkenazi Jews PMID: 14727180
  21. Therefore, our bicistronic lentivector supplying both alpha- and beta-subunits of beta-hexosaminidases may provide a potential therapeutic tool for the treatment of Sandhoff disease. PMID: 15953731
  22. analysis of the HEXA gene in Italian patients with infantile and late onset Tay-Sachs disease PMID: 16088929
  23. The plasma membrane associated beta-hexosaminidase A is fully processed, suggesting its lysosomal origin. PMID: 16212960
  24. the X-ray crystallographic structure of Hex A to 2.8 A resolution. The crystal structure of Hex A reveals an alphabeta heterodimer, with each subunit having a functional active site PMID: 16698036
  25. The highest activities of exoglycosidases were observed in high-grade gliomas, and a positive correlation of enzyme activities and degree of malignancy was noted. PMID: 16710745
  26. isoenzyme composition of N-acetyl-beta-D-hexosaminidase in seminal plasma from patients with secretory azoospermia is significantly different from controls, but this difference does not represent a useful marker of secretory azoospermia PMID: 16776631
  27. Describe strategy for detecting HexA mutations/activity in prenatal diagnosis of Tay-Sachs disease. PMID: 18425478
  28. In the infantile Tay-Sachs group, the number of atoms influenced by a mutation was larger than that in the late-onset disease group. PMID: 18490185
  29. A significantly higher activity of N-acetyl-beta-D-hexosaminidase in all laryngeal cancer specimens compared with that in healthy tissue homogenates, was observed. PMID: 19298806

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Involvement in disease GM2-gangliosidosis 1 (GM2G1)
Subcellular Location Lysosome.
Protein Families Glycosyl hydrolase 20 family
Database Links

HGNC: 4878

OMIM: 272800

KEGG: hsa:3073

STRING: 9606.ENSP00000268097

UniGene: Hs.604479

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