Glycylglycine Cas: 556-50-3 99% Dawb hmoov
| Catalog Number | xwm 90282 |
| Khoom npe | Glycylglycine |
| CAS | 556-50-3 |
| Molecular Formula | C4H8N2O3 |
| Molecular Luj | 132.11792 ib |
| Cov ntaub ntawv khaws cia | Ambient |
| Harmonized tariff Code | 29241900 ib |
Khoom Specification
| Poob rau ziab | <0.2% |
| Sulfate | <0.02% |
| Assay | 99% |
| Lwm cov amino acids | Tsis ntes tau |
| Kev xa tawm | > 95% |
| Chloride | <0.02% |
| Qhov tshwm sim | Dawb mus rau dawb hmoov |
| Hlau (Fe) | <30ppm |
| Heavy Hlau (Pb) | <10ppm |
| Ammonium (NH4) | <0.02% |
| Arsenic (raws li As 2O3) | <1 ppm |
| Residue ntawm ignition (raws li sulphate) | <0.15% |
Carbamoyl phosphate synthetase 1 (CPS1) deficiency vim CPS1 kev hloov pauv yog qhov tsis tshua muaj autosomal-recessive urea cycle disorder ua rau hyperammonemia uas tuaj yeem ua rau tuag lossis mob hlwb tsis zoo.CPS1 catalyzes carbamoyl phosphate tsim los ntawm ammonia, bicarbonate thiab ob molecules ntawm ATP, thiab yuav tsum tau cov allosteric activator N-acetyl-L-glutamate.Kev hloov pauv hauv kev kho mob tshwm sim nyob rau hauv tag nrho CPS1 coding cheeb tsam, tab sis feem ntau yog nyob rau hauv ib tsev neeg, nrog me ntsis rov tshwm sim.Peb tus cwj pwm ntawm no tsuas yog tam sim no paub txog kev hloov pauv CPS1, p.Val1013del, pom nyob rau hauv kaum ib tus neeg mob uas tsis muaj kev cuam tshuam ntawm Turkish qhovntsej thiaj tsis mob uas siv cov hom tsiaj qus los yog cov mutant CPS1 qhia nyob rau hauv baculovirus / kab cell system.Lub ntiaj teb no CPS1 cov tshuaj tiv thaiv thiab ATPase thiab ATP synthesis ib feem kev cuam tshuam uas cuam tshuam, ntsig txog, cov qib bicarbonate thiab carbamate phosphorylation, tau soj ntsuam.Peb pom tias CPS1 hom tsiaj qus thiab V1013del mutant pom qhov sib piv qhia qib thiab purity tab sis cov mutant CPS1 tsis muaj cov dej num tseem ceeb.Hauv CPS1 tus qauv qauv, V1013 belongs rau lub siab hydrophobic β-strand nyob rau hauv nruab nrab ntawm lub hauv paus β-daim ntawv ntawm A subdomain ntawm carbamate phosphorylation domain thiab yog ze rau qhov kwv yees carbamate qhov uas txuas ob qhov chaw phosphorylation.Kev tshawb fawb Haplotype qhia tias p.Val1013del yog tus tsim kev hloov pauv.Hauv kev xaus, qhov kev hloov pauv p.V1013del inactivates CPS1 tab sis tsis ua rau cov enzyme grossly unstable los yog insoluble.Kev rov ua dua ntawm qhov kev hloov pauv tshwj xeeb no hauv cov neeg mob Turkish yuav yog vim muaj kev cuam tshuam tus tsim, uas zoo ib yam nrog kev sib koom ua ke nquag pom hauv cov neeg cuam tshuam.
