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China Factory for Neuroscience - Glycylglycine Cas: 556-50-3 99% White powder – XD BIOCHEM

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Our development depends on the advanced equipment ,excellent talents and continuously strengthened technology forces for 2,3,4,6-Tetra-O-Benzyl-D-Galactopyranose, Intermediates, 117961-20-3, We always hold the philosophy of win-win, and build long-term cooperation relationship with customers from around the world.We believe that our growth base on customer's success, credit is our life.
China Factory for Neuroscience - Glycylglycine Cas: 556-50-3 99% White powder – XD BIOCHEM Detail:

Catalog Number XD90282
Product Name Glycylglycine

CAS

556-50-3

Molecular Formula

C4H8N2O3

Molecular Weight

132.11792
Storage Details Ambient

Harmonized Tariff Code

29241900

 

Product Specification

Loss on Drying <0.2%
Sulfate <0.02%
Assay 99%
Other amino acids Not detectable
Transmittance >95%
Chloride <0.02%
Appearance White to off-white powder
Iron (Fe) <30ppm
Heavy Metal (Pb) <10ppm
Ammonium (NH4) <0.02%
Arsenic (as As2O3) <1ppm
Residue on ignition (as sulphate)

<0.15%

 

Carbamoyl phosphate synthetase 1 (CPS1) deficiency due to CPS1 mutations is a rare autosomal-recessive urea cycle disorder causing hyperammonemia that can lead to death or severe neurological impairment. CPS1 catalyzes carbamoyl phosphate formation from ammonia, bicarbonate and two molecules of ATP, and requires the allosteric activator N-acetyl-L-glutamate. Clinical mutations occur in the entire CPS1 coding region, but mainly in single families, with little recurrence. We characterized here the only currently known recurrent CPS1 mutation, p.Val1013del, found in eleven unrelated patients of Turkish descent using recombinant His-tagged wild type or mutant CPS1 expressed in baculovirus/insect cell system. The global CPS1 reaction and the ATPase and ATP synthesis partial reactions that reflect, respectively, the bicarbonate and the carbamate phosphorylation steps, were assayed. We found that CPS1 wild type and V1013del mutant showed comparable expression levels and purity but the mutant CPS1 exhibited no significant residual activities. In the CPS1 structural model, V1013 belongs to a highly hydrophobic β-strand at the middle of the central β-sheet of the A subdomain of the carbamate phosphorylation domain and is close to the predicted carbamate tunnel that links both phosphorylation sites. Haplotype studies suggested that p.Val1013del is a founder mutation. In conclusion, the mutation p.V1013del inactivates CPS1 but does not render the enzyme grossly unstable or insoluble. Recurrence of this particular mutation in Turkish patients is likely due to a founder effect, which is consistent with the frequent consanguinity observed in the affected population.


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China Factory for Neuroscience - Glycylglycine  Cas: 556-50-3  99%  White powder – XD BIOCHEM detail pictures


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We emphasize progress and introduce new merchandise into the market each and every year for China Factory for Neuroscience - Glycylglycine Cas: 556-50-3 99% White powder – XD BIOCHEM , The product will supply to all over the world, such as: Nepal, Tunisia, Ireland, We offer OEM services and replacement parts to meet the varying needs of our customers. We offer competitive price for quality products and we will make certain your shipment is handled quickly by our logistics department. We sincerely hope to have the opportunity to meet with you and see how we can help you further your own business.
  • After the signing of the contract, we received satisfactory goods in a short term, this is a commendable manufacturer.
    5 Stars By Joanna from Paris - 2017.03.07 13:42
    The company account manager has a wealth of industry knowledge and experience, he could provide appropriate program according our needs and speak English fluently.
    5 Stars By Maria from Austria - 2017.09.09 10:18
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    China Factory for Neuroscience - Glycylglycine Cas: 556-50-3 99% White powder – XD BIOCHEM