L-Phenylalanine CAS: 63-91-2 99% Dawb hmoov
| Catalog Number | xwm 90317 |
| Khoom npe | L-Cysteine hydrochloride anhydrous |
| CAS | 63-91-2 |
| Molecular Formula | C9H11NO2 |
| Molecular Luj | 165.19 Nws |
| Cov ntaub ntawv khaws cia | Ambient |
| Harmonized tariff Code | 29224985 ib |
Khoom Specification
| Qhov tshwm sim | Dawb crystalline hmoov |
| Assay | 99% |
| Kev sib hloov tshwj xeeb | -20.4 - 35.2 |
| Cov hlau hnyav | <0.002% |
| Coj | <10mg/kg |
| pH | 5.4-6 |
| Poob rau ziab | <0.40% |
| Residue ntawm Ignition | <0.10% |
Nyob rau hauv maple syrup urine disease (MSUD), branched-chain L-amino (BCAA) thiab 2-oxo acids (BCOA) tsub kom nyob rau hauv lub cev kua vim yog ib tug tsis muaj peev xwm ntawm branched saw 2-oxo acid dehydrogenase complex kev ua.Hauv MSUD, cov ntaub ntawv me me muaj nyob rau ntawm qhov tseem ceeb ntawm kev tso zis tso tawm ntawm cov kab txuas txuas txuas.Peb tau tshuaj xyuas lub raum tshem tawm ntawm leucine, valine, isoleucine thiab alloisoleucine, thiab lawv cov 2-oxo acids 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S)-(S- KMV), thiab (R)-3-methyl-2-oxopentanoate (R-KMV), siv ib khub ntawm cov ntshav plasma thiab cov zis kuaj (n = 63) los ntawm 10 cov neeg mob nrog classical MSUD.Lub raum fractional excretion ntawm BCAA dawb yog nyob rau hauv ib txwm muaj (<0.5%) thiab ywj siab ntawm cov ntshav plasma concentrations.Lub excretion ntawm bound (N-acylated) BCAA yog qhov qub thiab tsis yog nyob ntawm BCAA plasma concentrations.fractional renal excretion of BCOA is in order KIC << KIV < R-KMV < or = S-KMV (range (%): KIC 0.1-25; KI V 0.14-21.3; S-KMV 0.26-24.6; R- KMV 0.1-35.9), muaj feem cuam tshuam nrog KIC plasma concentrations, thiab feem ntau siab dua li ntawm BCAA cuam tshuam.Cov txiaj ntsig tau pom tias lub raum tso tawm dawb BCAA nrog rau cov acylated derivatives yog qhov tsis txaus ntseeg.Lub raum excretion ntawm BCOA, txawm li cas los xij, rau qee qhov kev tawm tsam nce hauv BCAA concentrations thiab yog li ua rau txo qis ntawm tag nrho BCAA pas dej hauv MSUD.
