Analytical Data
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Gene name
Glucosamine (N-acetyl)-6-Sulfatase/GNS
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简介
Glucosamine (N-acetyl)-6-Sulfatase/GNS Protein is a lysosomal enzyme present in all cells. It is involved in the catabolism of heparin, heparin sulfate and keratin sulfate. Deficiency of this enzyme leads to inadequate substrate accumulation and lysosomal storage impairment IIID mucopolysaccharidosis. Glucosamine (N-acetyl) -6-Sulfatase/GNS Protein, Human (HEK293, His) is the recombinant human-derived Glucosamine, expressed by HEK293 , with C-6*His labeled tag.
- Application
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Alternative Names
rHuN-acetylglucosamine-6-sulfatase/GNS, His; N-Acetylglucosamine-6-Sulfatase; Glucosamine-6-Sulfatase; G6S; GNS
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Species
Human
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Source
HEK293
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Tag
C-6*His
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Purity
Greater than 95% as determined by SDS-PAGE.
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Uniprot
P15586-1
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Expression Region
V37-L552
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AA Sequence
VFGVAAGTRRPNVVLLLTDDQDEVLGGMTPLKKTKALIGEMGMTFSSAYVPSALCCPSRASILTGKYPHNHHVVNNTLEGNCSSKSWQKIQEPNTFPAILRSMCGYQTFFAGKYLNEYGAPDAGGLEHVPLGWSYWYALEKNSKYYNYTLSINGKARKHGENYSVDYLTDVLANVSLDFLDYKSNFEPFFMMIATPAPHSPWTAAPQYQKAFQNVFAPRNKNFNIHGTNKHWLIRQAKTPMTNSSIQFLDNAFRKRWQTLLSVDDLVEKLVKRLEFTGELNNTYIFYTSDNGYHTGQFSLPIDKRQLYEFDIKVPLLVRGPGIKPNQTSKMLVANIDLGPTILDIAGYDLNKTQMDGMSLLPILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVSQCFPDCVCEDAYNNTYACVRTMSALWNLQYCEFDDQEVFVEVYNLTADPDQITNIAKTIDPELLGKMNYRLMMLQSCSGPTCRTPGVFDPGYRFDPRLMFSNRGSVRTRRFSKHLL
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Protein Length
Full Length of Isoform-1 Mature Protein
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Molecular Weight
90 kDa
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Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
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Form
Freeze-dried powder
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Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
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Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
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Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
Related Products
Protein Description
Glucosamine (N-acetyl)-6-sulfatase (GNS) is a crucial enzyme involved in the lysosomal degradation of glycosaminoglycans, particularly heparan sulfate and keratan sulfate. Mutations in the GNS gene lead to mucopolysaccharidosis type IIIC (MPS IIIC), a rare lysosomal storage disorder characterized by progressive neurological decline, skeletal abnormalities, and impaired growth. The study of GNS and its recombinant protein form is vital for understanding the enzymatic mechanisms underlying GNS function and pathology. By developing recombinant GNS, researchers aim to explore potential therapeutic approaches, such as enzyme replacement therapy or substrate reduction strategies. These studies can also provide insights into enzyme structure-function relationships, mechanisms of enzyme activation and inhibition, and the development of small molecule drugs targeting lysosomal enzymatic pathways. Consequently, GNS research holds promise not only for elucidating the biochemical pathways associated with lysosomal storage disorders but also for the potential advancement of novel treatment strategies for affected patients. The characterization of recombinant GNS offers a valuable tool for screening compounds that can enhance enzymatic activity or provide alternative therapeutic avenues for MPS IIIC and related disorders.
