Glycylglycine Cas: 556-50-3 99% ntụ ntụ ọcha
| Nọmba katalọgụ | XD90282 |
| Aha ngwaahịa | Glycylglycine |
| CAS | 556-50-3 |
| Usoro ihe omimi | C4H8N2O3 |
| Ibu molekụla | 132.11792 |
| Nkọwa nchekwa | Ambient |
| Koodu tarifu akwadoro | 29241900 |
Nkọwapụta ngwaahịa
| Ọnwụ na ihicha | <0.2% |
| Sulfate | <0.02% |
| Nyocha | 99% |
| Amino acid ndị ọzọ | Enweghị ike ịhụ ya |
| nnyefe | > 95% |
| Chloride | <0.02% |
| Ọdịdị | Na-acha ọcha na-acha ọcha ntụ ntụ |
| Ígwè (Fe) | <30ppm |
| Ọla dị arọ (Pb) | <10ppm |
| Ammonium (NH4) | <0.02% |
| Arsenic (dị ka As2O3) | <1pm |
| Ihe fọdụrụ na mgbanye (dị ka sulphate) | <0.15% |
Enweghị ụkọ Carbamoyl phosphate synthetase 1 (CPS1) n'ihi mmụgharị CPS1 bụ ọrịa na-adịghị ahụkebe autosomal-recessive urea okirikiri na-akpata hyperammonemia nke nwere ike ibute ọnwụ ma ọ bụ nnukwu nsogbu akwara ozi.CPS1 na-akwalite nhazi nke carbamoyl phosphate sitere na amonia, bicarbonate na molekul abụọ nke ATP, ma na-achọ onye na-arụ ọrụ allosteric N-acetyl-L-glutamate.Mgbanwe ụlọ ọgwụ na-eme na mpaghara koodu CPS1 dum, mana ọ kachasị n'ime ezinaụlọ na-alụbeghị di, na-enwe obere nlọghachi.Anyị mara ebe a naanị ugbu a mara ugboro ugboro CPS1 mutation, p.Val1013del, hụrụ na iri na otu na-enweghị njikọ na ndị ọrịa nke Turkish na-eji recombinant His-tagged ụdị anụ ọhịa ma ọ bụ mutant CPS1 egosipụtara na baculovirus/ụmụ ahụhụ sistemu.Achọpụtara mmeghachi omume CPS1 zuru ụwa ọnụ yana ATPase na njikọ ATP na-egosipụta mmeghachi omume akụkụ nke na-egosipụta, n'otu n'otu, usoro bicarbonate na carbamate phosphorylation.Anyị chọpụtara na ụdị anụ ọhịa CPS1 na V1013del mutant gosipụtara ọkwa okwu na ịdị ọcha yiri nke ahụ mana mutant CPS1 egosighi ihe ọ bụla dị mkpa fọdụrụnụ.N'ụdị nhazi CPS1, V1013 bụ nke nwere nnukwu hydrophobic β-strand dị n'etiti etiti β-sheet nke A subdomain nke ngalaba phosphorylation carbamate ma dị nso na ọwara carbamate buru amụma nke jikọtara saịtị abụọ phosphorylation.Ọmụmụ ihe ọmụmụ Haplotype tụrụ aro na p.Val1013del bụ ngbanwe nke onye malitere.Na mmechi, mmụgharị p.V1013del na-eme ka CPS1 rụọ ọrụ mana ọ naghị eme ka enzyme ahụ ghara ịkasi obi ma ọ bụ ghara ịsacha ya.Ntughari nke mmụgharị a pụrụ iche na ndị ọrịa Turkey nwere ike ịbụ n'ihi nsonaazụ onye nrụpụta, nke kwekọrọ na nnabata a na-ahụkarị na ndị mmadụ metụtara.
