L-Phenylalanine CAS: 63-91-2 99% Pulvis albus
| Catalogue Number | XD90317 |
| Product Name | L-Cysteine hydrochloride anhydrous |
| CAS | 63-91-2 |
| Formulae hypotheticae | C9H11NO2 |
| M. Pondus | 165.19 |
| Repono Details | Ambiens |
| Tariff Code harmonized | 29224985 |
Product Specification
| Aspectus | Album pulveris crystallini |
| Assay | 99% |
| Imprimis rotationis | -20.4 ad -35.2 |
| Metalla gravis | <0.002% |
| Plumbum | <10mg/kg |
| pH | 5.4 - 6 |
| Damnum in Siccatio | <0.40% |
| Residere in Ignition | <0.10% |
In syrupus acernus morbus urinae (MSUD), catella ramosus L-aminus (BCAA) et acida 2-oxo (BCOA) cumulant humorum corporis propter defectum hereditatis catenae 2-oxo-acidi dehydrogenasi complexi operationis.In MSUD, parum informationis praesto est de significatione dispositionis urinariae mixtorum catellae ramosae.Inspeximus renum alvi leucinium, valinae, isoleucinae et alloisoleucinae, earumque acida 2-oxo correspondentia 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S)-(S. KMV), et (R) -3-methyl-2-oxopentanoate (R-KMV), utens paribus speciminibus plasmatis et urinae (n = 63) ex 10 aegris cum MSUD classicis.Renalis excretio fracti liberae BCAA erat in range normali (<0.5%) et independens a concentratione plasmatis.Excretio devinctum BCAA normale fuit et non signanter in concentratione plasma BCAA dependens.Excretio renalis BCOA fractorum erat in ordine KIC << KIV < R-KMV < seu = S-KMV (%: KIC 0.1-25; KI V 0.14-21.3, S-KMV 0.26-24.6 R- KMV 0.135.9), signanter connectuntur cum concentratione KIC plasma, et plerumque altior quam in actis BCAA.Eventus ostendunt excretionem renum liberae BCAA necnon derivatorum acylatorum neglegebilem esse.Attamen renum excretio BCOA aliquatenus contraria in concentrationes BCAA crescit et sic ad lacus totalis BCAA in MSUD demissionem confert.
