Recombinant Human HEXA protein is a Human Full Length protein, in the 1 to 529 aa range, expressed in Baculovirus infected Sf9, with >=90% purity and suitable for SDS-PAGE.
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Key facts
- Purity
- >=90% SDS-PAGE
- Expression system
- Baculovirus infected Sf9 cells
- Tags
- His-DDDDK tag C-Terminus
- Applications
- SDS-PAGE
- Biologically active
- No
Amino acid sequence
M T S S R L W F S L L L A A A F A G R A T A L W P W P Q N F Q T S D Q R Y V L Y P N N F Q F Q Y D V S S A A Q P G C S V L D E A F Q R Y R D L L F G S G S W P R P Y L T G K R H T L E K N V L V V S V V T P G C N Q L P T L E S V E N Y T L T I N D D Q C L L L S E T V W G A L R G L E T F S Q L V W K S A E G T F F I N K T E I E D F P R F P H R G L L L D T S R H Y L P L S S I L D T L D V M A Y N K L N V F H W H L V D D P S F P Y E S F T F P E L M R K G S Y N P V T H I Y T A Q D V K E V I E Y A R L R G I R V L A E F D T P G H T L S W G P G I P G L L T P C Y S G S E P S G T F G P V N P S L N N T Y E F M S T F F L E V S S V F P D F Y L H L G G D E V D F T C W K S N P E I Q D F M R K K G F G E D F K Q L E S F Y I Q T L L D I V S S Y G K G Y V V W Q E V F D N K V K I Q P D T I I Q V W R E D I P V N Y M K E L E L V T K A G F R A L L S A P W Y L N R I S Y G P D W K D F Y I V E P L A F E G T P E Q K A L V I G G E A C M W G E Y V D N T N L V P R L W P R A G A V A E R L W S N K L T S D L T F A Y E R L S H F R C E L L R R G V Q A Q P L N V G F C E Q E F E Q T D Y K D D D D K H H H H H H
Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
Associated Products
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Target data
Function
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).
Alternative names
Beta-hexosaminidase subunit alpha, Beta-N-acetylhexosaminidase subunit alpha, N-acetyl-beta-glucosaminidase subunit alpha, Hexosaminidase subunit A, HEXA
Recommended products
Recombinant Human HEXA protein is a Human Full Length protein, in the 1 to 529 aa range, expressed in Baculovirus infected Sf9, with >=90% purity and suitable for SDS-PAGE.
Key facts
- Purity
- >=90% SDS-PAGE
- Expression system
- Baculovirus infected Sf9 cells
- Tags
- His-DDDDK tag C-Terminus
- Applications
- SDS-PAGE
- Biologically active
- No
- Accession
- P06865-1
- Animal free
- No
- Species
- Human
- Concentration
- Storage buffer
pH: 8
Preservative: 1.02% Imidazole
Constituents: 20% Glycerol (glycerin, glycerine), 0.64% Sodium chloride, 0.63% Tris HCl, 0.04% Sorbitan monolaurate, ethoxylated, 0.02% Potassium chloride
Sequence info
Amino acid sequence
- M T S S R L W F S L L L A A A F A G R A T A L W P W P Q N F Q T S D Q R Y V L Y P N N F Q F Q Y D V S S A A Q P G C S V L D E A F Q R Y R D L L F G S G S W P R P Y L T G K R H T L E K N V L V V S V V T P G C N Q L P T L E S V E N Y T L T I N D D Q C L L L S E T V W G A L R G L E T F S Q L V W K S A E G T F F I N K T E I E D F P R F P H R G L L L D T S R H Y L P L S S I L D T L D V M A Y N K L N V F H W H L V D D P S F P Y E S F T F P E L M R K G S Y N P V T H I Y T A Q D V K E V I E Y A R L R G I R V L A E F D T P G H T L S W G P G I P G L L T P C Y S G S E P S G T F G P V N P S L N N T Y E F M S T F F L E V S S V F P D F Y L H L G G D E V D F T C W K S N P E I Q D F M R K K G F G E D F K Q L E S F Y I Q T L L D I V S S Y G K G Y V V W Q E V F D N K V K I Q P D T I I Q V W R E D I P V N Y M K E L E L V T K A G F R A L L S A P W Y L N R I S Y G P D W K D F Y I V E P L A F E G T P E Q K A L V I G G E A C M W G E Y V D N T N L V P R L W P R A G A V A E R L W S N K L T S D L T F A Y E R L S H F R C E L L R R G V Q A Q P L N V G F C E Q E F E Q T D Y K D D D D K H H H H H H
- Accession
- P06865
- Protein length
- Full Length
- Predicted molecular weight
- 62.5 kDa
- Amino acids
- 1 to 529
- Nature
- Recombinant
- Tags
- His-DDDDK tag C-Terminus
Specifications
- Form
- Liquid
- Additional notes
Affinity purified.
General info
- Function
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).
- Sequence similarities
Belongs to the glycosyl hydrolase 20 family.
- Post-translational modifications
N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2) (Probable) (PubMed:1533633, PubMed:16698036, PubMed:19159218). N-linked glycan at Asn-157 consists of either GlcNAc or GlcNAc(2)-Man(7-9). N-linked glycan at Asn-295 consists of either GlcNAc, GlcNAc-Fuc, or GlcNAc(2)-Man(4) (Probable).
- Subcellular localisation
- Lysosome
Storage
- Shipped at conditions
- Dry Ice
- Appropriate short-term storage conditions
- -80°C
- Appropriate long-term storage conditions
- -80°C
- Storage information
- Avoid freeze / thaw cycle
Notes
ab198482 is useful for the study of enzyme kinetics, screening inhibitors, and selectivity profiling.
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
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.
- Associated diseases and disorders
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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SDS-PAGE - Recombinant Human HEXA protein (ab198482)
4-20% SDS PAGE analysis of ab198482 (2.8 μg).
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