Glycylglycine Cas: 556-50-3 99% phofo e tšoeu
| Nomoro ea lethathamo | XD90282 |
| Lebitso la Sehlahiswa | Glycylglycine |
| CAS | 556-50-3 |
| Foromo ea limolek'hule | C4H8N2O3 |
| Boima ba Molek'hule | 132.11792 |
| Lintlha tsa polokelo | Ambient |
| Khoutu ea Lekhetho e Kopantsoeng | 29241900 |
Tlhaloso ea Sehlahisoa
| Tahlehelo ka ho Omisa | <0.2% |
| Sulfate | <0.02% |
| Tlhahlobo | 99% |
| Li-amino acid tse ling | Ha e bonahale |
| Phetiso | >95% |
| Chloride | <0.02% |
| Ponahalo | Phofo e tšoeu ho isa ho e tšoeu |
| Tshipi (Fe) | <30ppm |
| Heavy Metal (Pb) | <10ppm |
| Ammonium (NH4) | <0.02% |
| Arsenic (joalo ka As2O3) | <1ppm |
| Masalla ha a tuka (joalo ka sulphate) | <0.15% |
Khaello ea Carbamoyl phosphate synthetase 1 (CPS1) ka lebaka la liphetoho tsa CPS1 ke lefu le sa tloaelehang la autosomal-recessive urea cycle le bakang hyperammonemia e ka lebisang lefung kapa ho senyeha ho matla ha methapo ea kutlo.CPS1 e matlafatsa sebopeho sa carbamoyl phosphate ho tsoa ho ammonia, bicarbonate le limolek'hule tse peli tsa ATP, 'me e hloka activator ea allosteric N-acetyl-L-glutamate.Liphetoho tsa kliniki li etsahala sebakeng sohle sa likhoutu tsa CPS1, empa haholo-holo malapeng a le mong, 'me ha ho ipheta hanyane.Re tšoailoe mona feela hona joale e tsejoang hape e le phetoho ea CPS1, p.Val1013del, e fumanoang ho bakuli ba leshome le motso o mong ba sa amaneng ba leloko la Turkey ba sebelisa recombinant Mofuta oa hae oa hlaha kapa CPS1 e fetotsoeng e hlahisitsoeng tsamaisong ea lisele tsa baculovirus/kokoanyana.Karabelo ea lefats'e ea CPS1 le karabelo e sa fellang ea ATPase le ATP e bonts'ang, ka ho latellana, mehato ea bicarbonate le carbamate phosphorylation, li ile tsa lekoa.Re fumane hore mofuta o hlaha oa CPS1 le V1013del mutant o bonts'itse maemo le bohloeki bo bapisoang empa CPS1 e fetotsoeng e ne e sa bonts'e mesebetsi ea bohlokoa e setseng.Moetsong oa sebopeho sa CPS1, V1013 ke ea β-strand e nang le hydrophobic haholo bohareng ba β-sheet e bohareng ea A subdomain ea carbamate phosphorylation domain mme e haufi le kotopo e boletsoeng esale pele ea carbamate e hokahanyang libaka tse peli tsa phosphorylation.Boithuto ba Haplotype bo khothalelitse hore p.Val1013del ke phetoho ea mothehi.Qetellong, phetoho ea p.V1013del e etsa hore CPS1 e sebetse empa ha e etse hore enzyme e se ke ea tsitsa kapa e se ke ea qhibiliha.Ho khutla ha phetoho ena ho bakuli ba Turkey ho ka etsahala hore ebe ka lebaka la phello ea mothehi, e lumellanang le consanguinity e hlokometsoeng khafetsa ho baahi ba amehileng.
