I-Glycylglycine Cas: 556-50-3 99% Umgubo omhlophe
| Inombolo Yekhathalogu | XD90282 |
| Igama lemveliso | Glycylglycine |
| CAS | 556-50-3 |
| Ifomula yeemolekyuli | C4H8N2O3 |
| Ubunzima beMolekyuli | 132.11792 |
| Iinkcukacha zokuGcina | I-Ambient |
| IKhowudi yoMrhumo eHarmonized | 29241900 |
Ukucaciswa kweMveliso
| Ilahleko ekomisweni | <0.2% |
| Sulfate | <0.02% |
| Isivavanyi | 99% |
| Ezinye ii-amino acids | Ayibonakali |
| Ugqithiso | >95% |
| I-Chloride | <0.02% |
| Imbonakalo | Umgubo omhlophe ukuya kumhlophe |
| Intsimbi (Fe) | <30ppm |
| Intsimbi enzima (Pb) | <10ppm |
| I-Ammonium (NH4) | <0.02% |
| IArsenic (njenge-As2O3) | <1ppm |
| Intsalela ekutshisweni (njengesulphate) | <0.15% |
Ukunqongophala kweCarbamoyl phosphate synthetase 1 (CPS1) ngenxa yeenguqu ze-CPS1 yingxaki enqabileyo ye-autosomal-recessive urea cycle disorder ebangela i-hyperammonemia enokukhokelela ekufeni okanye ukuphazamiseka okukhulu kwe-neurological.CPS1 catalyzes carbamoyl phosphate formation from ammonia, bicarbonate and two molecules of ATP, kwaye ifuna i-allosteric activator N-acetyl-L-glutamate.Utshintsho lwezonyango lwenzeka kuyo yonke ingingqi ye-CPS1 yekhowudi, kodwa ikakhulu kwiintsapho ezingatshatanga, kunye nokuphindaphinda okuncinci.Siye sabonisa apha ekuphela kwento eyaziwayo ngoku ye-CPS1 yokuguqulwa, i-p.Val1013del, ifunyenwe kwizigulane ezilishumi elinanye ezinganxulumananga nenzala yaseTurkey zisebenzisa i-recombinant Uhlobo lwasendle oluphawulweyo okanye i-CPS1 eguqukileyo echazwe kwi-baculovirus/isinambuzane inkqubo yeseli.Ukusabela kwe-CPS1 yehlabathi jikelele kunye ne-ATPase kunye ne-ATP synthesis partial reactions ezibonisa, ngokulandelanayo, i-bicarbonate kunye ne-carbamate phosphorylation amanyathelo, ziye zavavanywa.Sifumene ukuba uhlobo lwasendle lwe-CPS1 kunye ne-V1013del mutant ibonise amanqanaba okuthetha athelekisekayo kunye nokusulungeka kodwa i-CPS1 eguqukayo ayibonisi misebenzi ibalulekileyo yentsalela.Kwimodeli yesakhiwo se-CPS1, i-V1013 iyingxenye ye-hydrophobic β-strand ephakathi kwe-β-sheet ephakathi ye-A subdomain ye-carbamate phosphorylation domain kwaye isondele kwi-tunnel ye-carbamate eqikelelweyo edibanisa zombini iindawo ze-phosphorylation.Uphononongo lweHaplotype lucebise ukuba i-p.Val1013del yinguqu yomseki.Ukuqukumbela, i-mutation p.V1013del yenza i-CPS1 isebenze kodwa ayinikezeli i-enzyme ngokungagungqiyo okanye enganyibilikiyo.Ukuphindaphinda kolu tshintsho oluthile kwizigulana zaseTurkey kusenokwenzeka ukuba kungenxa yesiphumo somseki, esihambelana nokubonwa rhoqo kwabantu abachaphazelekayo.
