L-Phenylalanine CAS:63-91-2 99% Paʻepaʻe paʻepaʻe
| Numera Fa'amaumauga | XD90317 |
| Igoa Oloa | L-Cysteine hydrochloride anhydrous |
| CAS | 63-91-2 |
| Fuafua mole | C9H11NO2 |
| Molecular Weight | 165.19 |
| Fa'amatalaga Teuina | Ambient |
| Fa'atonu Fa'asologa o Fa'asologa | 29224985 |
Fa'amatalaga o oloa
| Fa'aaliga | Pa'epa'e tioata pa'epa'e |
| Su'ega | 99% |
| Ta'amilosaga fa'apitoa | -20.4 i le -35.2 |
| Metala mamafa | <0.002% |
| Ta'ita'i | <10mg/kg |
| pH | 5.4 - 6 |
| Ma'imau ile Fa'amago | <0.40% |
| Toega i luga o le mumu | <0.10% |
I le maple syrup urine disease (MSUD), le filifili lala L-amino (BCAA) ma le 2-oxo acids (BCOA) e fa'aputu i sua o le tino ona o le le atoatoa o le filifili lala 2-oxo acid dehydrogenase gaioiga lavelave.I le MSUD, e itiiti ni fa'amatalaga o lo'o maua i le taua o le lafoaia o le mimi o filifili lala.Na matou suʻesuʻeina le kilia o le fatu o le leucine, valine, isoleucine ma alloisoleucine, ma a latou 2-oxo acids 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S) - (S-). KMV), ma le (R) -3-methyl-2-oxopentanoate (R-KMV), faʻaaogaina taitoalua o plasma ma urine samples (n = 63) mai 10 maʻi ma MSUD masani.Ole vaega ole fa'amama ole BCAA ole fua ole BCAA sa i ai ile tulaga masani (<0.5%) ma e tuto'atasi mai le maualuga ole plasma.O le faʻasalaga o le fusifusia (N-acylated) BCAA sa masani ma e le faʻalagolago tele i le BCAA plasma concentrations.O le fa'asologa o fatuga'o o le BCOA sa i le faasologa KIC << KIV
