Glycylglycine Cas: 556-50-3 99% funfun lulú
| Nọmba katalogi | XD90282 |
| Orukọ ọja | Glycylglycine |
| CAS | 556-50-3 |
| Ilana molikula | C4H8N2O3 |
| Òṣuwọn Molikula | 132.11792 |
| Awọn alaye ipamọ | Ibaramu |
| Ti irẹpọ Owo idiyele koodu | 29241900 |
Ọja Specification
| Isonu lori Gbigbe | <0.2% |
| Sulfate | <0.02% |
| Ayẹwo | 99% |
| Awọn amino acids miiran | Ko ṣee wa-ri |
| Gbigbe | > 95% |
| Kloride | <0.02% |
| Ifarahan | Funfun si pa-funfun lulú |
| Irin (Fe) | <30ppm |
| Irin Heavy (Pb) | <10ppm |
| Ammonium (NH4) | <0.02% |
| Arsenic (bii As2O3) | <1ppm |
| Aloku lori ina (gẹgẹbi sulphate) | <0.15% |
Aipe Carbamoyl fosifeti synthetase 1 (CPS1) nitori awọn iyipada CPS1 jẹ iṣọn-ẹjẹ autosomal-recessive urea ti o ṣọwọn ti o nfa hyperammonemia ti o le ja si iku tabi ailagbara iṣan.CPS1 ṣe itọsi idasile carbamoyl fosifeti lati amonia, bicarbonate ati awọn moleku meji ti ATP, ati pe o nilo oluṣiṣẹ allosteric N-acetyl-L-glutamate.Awọn iyipada ile-iwosan waye ni gbogbo agbegbe ifaminsi CPS1, ṣugbọn ni pataki ni awọn idile ẹyọkan, pẹlu atunwi diẹ.A ṣe afihan nibi nikan ti a mọ lọwọlọwọ loorekoore CPS1 iyipada, p.Val1013del, ti a rii ni awọn alaisan mọkanla ti ko ni ibatan ti iran Tọki nipa lilo iru egan ti a samisi recombinant tabi mutant CPS1 ti a fihan ni baculovirus/eto sẹẹli kokoro.Idahun CPS1 agbaye ati ATPase ati ATP kolaginni awọn aati apa kan ti o ṣe afihan, lẹsẹsẹ, bicarbonate ati awọn igbesẹ phosphorylation carbamate, ni a ṣe ayẹwo.A rii pe iru egan CPS1 ati V1013del mutant ṣe afihan awọn ipele ikosile ti o jọra ati mimọ ṣugbọn mutant CPS1 ko ṣe afihan awọn iṣẹku pataki.Ninu awoṣe igbekalẹ CPS1, V1013 jẹ ti okun hydrophobic β-strand ti o ga ni aarin aarin β-sheet ti A subdomain ti agbegbe phosphorylation carbamate ati pe o sunmọ oju eefin carbamate ti asọtẹlẹ ti o sopọ mọ awọn aaye phosphorylation mejeeji.Awọn ijinlẹ Haplotype daba pe p.Val1013del jẹ iyipada oludasilẹ.Ni ipari, iyipada p.V1013del ṣe aiṣiṣẹ CPS1 ṣugbọn ko jẹ ki enzymu naa jẹ riru pupọ tabi airotẹlẹ.Ipadabọ ti iyipada pato yii ni awọn alaisan Tọki ṣee ṣe nitori ipa olupilẹṣẹ, eyiti o ni ibamu pẹlu ifọkanbalẹ loorekoore ti a ṣe akiyesi ni olugbe ti o kan.
