I-Glycylglycine Cas: 556-50-3 99% Impuphu emhlophe
| Inombolo Yekhathalogi | XD90282 |
| Igama Lomkhiqizo | I-Glycylglycine |
| I-CAS | 556-50-3 |
| I-Molecular Formula | I-C4H8N2O3 |
| Isisindo samangqamuzana | 132.11792 |
| Imininingwane Yesitoreji | I-ambient |
| Ikhodi Yentela Evumelanisiwe | 29241900 |
Ukucaciswa Komkhiqizo
| Ukulahlekelwa Ekumisweni | <0.2% |
| I-sulfate | <0.02% |
| Isivivinyo | 99% |
| Amanye ama-amino acid | Ayibonakali |
| Ukudlulisa | >95% |
| I-Chloride | <0.02% |
| Ukubukeka | Impushana emhlophe ukuya kokumhlophe |
| Insimbi (Fe) | <30ppm |
| I-Heavy Metal (Pb) | <10ppm |
| I-Ammonium (NH4) | <0.02% |
| I-Arsenic (as2O3) | <1ppm |
| Izinsalela ekushiseni (njenge-sulphate) | <0.15% |
Ukushoda kwe-Carbamoyl phosphate synthetase 1 (CPS1) ngenxa yokuguqulwa kwe-CPS1 ukushoda kwe-autosomal-recessive urea cycle disorder okubangela i-hyperammonemia engaholela ekufeni noma ukukhubazeka okukhulu kwemizwa.I-CPS1 ivuselela ukwakheka kwe-carbamoyl phosphate kusuka ku-ammonia, i-bicarbonate nama-molecule amabili e-ATP, futhi idinga isishoshovu se-allosteric i-N-acetyl-L-glutamate.Ukuguqulwa komtholampilo kwenzeka kuso sonke isifunda sekhodi se-CPS1, kodwa ikakhulukazi emindenini eyodwa, kunokuphindaphinda okuncane.Siphawule lapha ukuphela kwenguquko ye-CPS1 eyaziwa njengamanje, p.Val1013del, etholakala ezigulini eziyishumi nanye ezingahlobene zozalo lwaseTurkey zisebenzisa uhlobo lwasendle olumakiwe kabusha noma i-CPS1 eguquguqukayo evezwa kusistimu yeseli ye-baculovirus/yezinambuzane.Ukusabela kwe-CPS1 yomhlaba wonke kanye ne-ATPase kanye ne-ATP synthesis ingxenye yokusabela okubonisa, ngokulandelana, izinyathelo ze-bicarbonate kanye ne-carbamate phosphorylation, zahlolwa.Sithole ukuthi uhlobo lwasendle lwe-CPS1 kanye ne-V1013del mutant ibonise amazinga enkulumo afanayo nobumsulwa kodwa i-CPS1 eguquguqukayo ayizange ibonise imisebenzi ebalulekile eyinsalela.Kumodeli yesakhiwo se-CPS1, i-V1013 iyingxenye ye-β-strand ene-hydrophobic kakhulu phakathi kwe-β-sheet emaphakathi yesizinda se-A sesizinda se-carbamate phosphorylation futhi iseduze nomhubhe we-carbamate obikezelwe oxhumanisa zombili izingosi ze-phosphorylation.Ucwaningo lwe-Haplotype luphakamise ukuthi i-p.Val1013del iwukuguqulwa komsunguli.Sengiphetha, i-mutation p.V1013del yenza i-CPS1 isebenze kodwa ayinikezi i-enzyme ngendlela engazinzi neze noma ingancibiliki.Ukuphindeka kwalokhu kuguqulwa okuthile ezigulini zaseTurkey kungenzeka kungenxa yomphumela womsunguli, ohambisana nokuhlangana okuvamile okubonwa kubantu abathintekile.
