L-Phenylalanine CAS: 63-91-2 99% Paila keʻokeʻo
| Helu Helu | XD90317 |
| inoa mea kūʻai | L-Cysteine hydrochloride anhydrous |
| CAS | 63-91-2 |
| ʻĀpana Molekala | C9H11NO2 |
| Kaumaha Molecular | 165.19 |
| Nā kikoʻī mālama | Ambient |
| Kānāwai Kūʻai Kūlike | 29224985 |
Huahana Huahana
| Ka nana aku | ʻO ka pauka kristal keʻokeʻo |
| Hoʻāʻo | 99% |
| Kahuli kiko'ī | -20.4 a i -35.2 |
| Nā metala kaumaha | <0.002% |
| Ke alakaʻi | <10mg/kg |
| pH | 5.4 - 6 |
| Nalo ma ka maloo | <0.40% |
| Koena ma ka Ignition | <0.10% |
I loko o ka maʻi maple syrup urine (MSUD), nā lālā L-amino (BCAA) a me 2-oxo acids (BCOA) e hōʻiliʻili i loko o nā wai kino ma muli o kahi hemahema hoʻoilina o ka hana paʻakikī 2-oxo acid dehydrogenase.Ma MSUD, loaʻa ka ʻike liʻiliʻi e pili ana i ke ʻano o ka hoʻolei ʻana i ka mimi o nā pūhui kaulahao lālā.Ua nānā mākou i ka hoʻomaʻemaʻe renal o leucine, valine, isoleucine a me alloisoleucine, a me kā lākou 2-oxo acids 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S)-(S- KMV), a me (R) -3-methyl-2-oxopentanoate (R-KMV), me ka hoʻohana ʻana i nā pālua o ka plasma a me ka mimi (n = 63) mai nā maʻi 10 me MSUD maʻamau.ʻO ka wehe ʻana o ka renal fractional o ka BCAA manuahi ma ke ʻano maʻamau (< 0.5%) a kūʻokoʻa i ka ʻike plasma.ʻO ka excretion o BCAA paʻa (N-acylated) he mea maʻamau a ʻaʻole hilinaʻi nui i ka BCAA plasma concentrations.ʻO ka haʻihaʻi ʻana o ka renal excretion o BCOA aia ma ke kauoha KIC << KIV < R-KMV < a i ʻole = S-KMV (ka palena (%): KIC 0.1-25; KI V 0.14-21.3; S-KMV 0.26-24.6; R- KMV 0.1-35.9), pili nui me ka KIC plasma concentrations, a maʻamau kiʻekiʻe ma mua o ka BCAA pili.Hōʻike nā hopena i ka haʻalele ʻana o ka renal o ka BCAA manuahi a me nā derivatives acylated he mea ʻole.ʻO ka hoʻokuʻu ʻana o ka renal o BCOA, akā naʻe, i kekahi mau mea e kūʻē i ka piʻi ʻana o ka nui o ka BCAA a no laila ke kōkua nei i ka hoʻohaʻahaʻa ʻana i ka nui o nā loko BCAA ma MSUD.
