Glycylglycine Cas: 556-50-3 99% White paura
| Nama Putumōhio | XD90282 |
| Ingoa Hua | Glycylglycine |
| CAS | 556-50-3 |
| Tātai rāpoi | C4H8N2O3 |
| Taumaha rāpoi | 132.11792 |
| Nga Taipitopito Rokiroki | Ambient |
| Waehere Rere Whakariterite | 29241900 |
Whakatakotoranga Hua
| Ngaronga i te whakamaroke | <0.2% |
| pūkawa | <0.02% |
| Te whakamatautau | 99% |
| Ētahi atu waikawa amino | Kaore e kitea |
| Whakawhitinga | >95% |
| pūhaumāota | <0.02% |
| Te ahua | Ma ki te paura ma |
| Rino (Fe) | <30ppm |
| Koura Taumaha (Pb) | <10ppm |
| Haukinia (NH4) | <0.02% |
| Arsenic (hei As2O3) | <1ppm |
| Te toenga i runga i te mura (hei sulphate) | <0.15% |
Ko te ngoikore o te Carbamoyl phosphate synthetase 1 (CPS1) na te rerekee o CPS1 he mate onge te huringa o te huringa Urea autosomal-recessive e hua ai te hyperammonemia ka mate, ka mate kino ranei.Ko te CPS1 te whakakorikori i te hanga carbamoyl phosphate mai i te haukini, te bicarbonate me nga ngota ngota e rua o te ATP, me te tono i te allosteric activator N-acetyl-L-glutamate.Ka puta nga huringa haumanu i roto i te rohe whakawaehere CPS1 katoa, engari ko te nuinga o nga whanau kotahi, he iti te hokinga mai.I tohuhia e matou i konei ko te huringa CPS1 noa e mohiotia ana i tenei wa, p.Val1013del, i kitea i roto i nga turoro kotahi tekau ma tahi o nga uri o Turikina e whakamahi ana i te momo mohoao kua tohuhia e ana, te CPS1 mutant ranei e whakaatuhia ana i roto i te punaha o te baculovirus / pepeke.Ko te tauhohenga CPS1 o te ao me te ATPase me te ATP whakahiato i nga tauhohenga wahanga e whakaata ana, ia, te bicarbonate me nga hikoi phosphorylation carbamate, i whakamatauria.I kitea e matou ko te momo mohoao CPS1 me te mutant V1013del i whakaatu i nga taumata korero me te maa engari ko te CPS1 mutant karekau he mahi toenga nui.I roto i te tauira hangahanga CPS1, ko V1013 no tetahi whenu β-hydrophobic tino nui i waenganui o te rau-β-pokapū o te A subdomain o te rohe phosphorylation carbamate me te tata ki te kauhanga carbamate matapae e hono ana i nga waahi phosphorylation e rua.Ko nga rangahau Haplotype i kii ko te p.Val1013del he rerekee kaiwhakarewa.Hei whakamutunga, ko te irakētanga p.V1013del ka whakakore i te CPS1 engari karekau e tino kore e rewa, e kore e rewa.Ko te hokinga mai o tenei whakarereketanga i roto i nga turoro o Turikina tera pea na te huanga o te kaiwhakarewa, e rite ana ki te whanautanga i kitea i roto i te taupori kua pa.
