I-L-Phenylalanine CAS: 63-91-2 99% Impuphu emhlophe
| Inombolo Yekhathalogi | XD90317 |
| Igama Lomkhiqizo | L-Cysteine hydrochloride anhydrous |
| I-CAS | 63-91-2 |
| I-Molecular Formula | I-C9H11NO2 |
| Isisindo samangqamuzana | 165.19 |
| Imininingwane Yesitoreji | I-ambient |
| Ikhodi Yentela Evumelanisiwe | 29224985 |
Ukucaciswa Komkhiqizo
| Ukubukeka | I-crystalline powder emhlophe |
| Isivivinyo | 99% |
| Ukuzungezisa okuqondile | -20.4 kuya ku-35.2 |
| Izinsimbi ezinzima | <0.002% |
| Ukuhola | <10mg/kg |
| pH | 5.4 - 6 |
| Ukulahlekelwa Ekumisweni | <0.40% |
| Izinsalela ekushiseni | <0.10% |
Kusifo se-maple syrup urine disease (MSUD), i-branched-chain L-amino (BCAA) kanye ne-2-oxo acids (BCOA) anqwabelana oketshezini lomzimba ngenxa yokuntuleka okuzuzwa njengefa lomsebenzi oyinkimbinkimbi we-branched-chain 2-oxo acid dehydrogenase.Ku-MSUD, ulwazi oluncane luyatholakala ngokubaluleka kokulahlwa komchamo kwezinhlanganisela ze-branched-chain.Sihlole ukucaciswa kwezinso kwe-leucine, i-valine, isoleucine ne-alloisoleucine, kanye nama-2-oxo acid ahambisana nawo 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S) - (S- KMV), kanye (R) -3-methyl-2-oxopentanoate (R-KMV), besebenzisa amasampula e-plasma nomchamo (n = 63) kusuka kuziguli ze-10 ezine-MSUD yakudala.Ukukhishwa kwe-fractional renal ye-BCAA yamahhala bekusebangeni elijwayelekile (<0.5%) futhi kuzimele ekugxilweni kwe-plasma.I-excretion of bound (N-acylated) BCAA yayijwayelekile futhi ingancikile kakhulu ekugxilweni kwe-plasma ye-BCAA.Ukukhishwa kwezinso okuyingxenyana kwe-BCOA bekukuhlelo lwe-KIC << KIV < R-KMV < noma = S-KMV (ububanzi (%): KIC 0.1-25; KI V 0.14-21.3; S-KMV 0.26-24.6; R- I-KMV 0.1-35.9), ehlotshaniswa kakhulu nokugxilwa kwe-plasma ye-KIC, futhi ngokuvamile iphakeme kunaleyo ye-BCAA ehlobene.Imiphumela ibonisa ukuthi ukukhishwa kwezinso kwe-BCAA yamahhala kanye nokuphuma kwe-acylated kuyinto encane.Ukukhishwa kwezinso kwe-BCOA, nokho, ngokwezinga elithile kumelana nokwenyuka kokugxila kwe-BCAA futhi ngaleyo ndlela kunomthelela ekwehliseni inani lamachibi e-BCAA ku-MSUD.
