L-Phenylalanine CAS: 63-91-2 99% Umgubo omhlophe
| Inombolo Yekhathalogu | XD90317 |
| Igama lemveliso | L-Cysteine hydrochloride anhydrous |
| CAS | 63-91-2 |
| Ifomula yeemolekyuli | C9H11NO2 |
| Ubunzima beMolekyuli | 165.19 |
| Iinkcukacha zokuGcina | I-Ambient |
| IKhowudi yoMrhumo eHarmonized | 29224985 |
Ukucaciswa kweMveliso
| Imbonakalo | Umgubo omhlophe wekristale |
| Isivavanyi | 99% |
| Ukujikeleza okuthe ngqo | -20.4 ukuya -35.2 |
| Iintsimbi ezinzima | <0.002% |
| Inkokeli | <10mg/kg |
| pH | 5.4 - 6 |
| Ilahleko ekomisweni | <0.40% |
| Intsalela kwi-Ignition | <0.10% |
Kwisifo se-maple syrup urine disease (MSUD), i-branched-chain L-amino (BCAA) kunye ne-2-oxo acids (BCOA) ziqokelela kumanzi omzimba ngenxa yokunqongophala okuzuzwe njenge-branched-chain 2-oxo acid dehydrogenase complex activity.Kwi-MSUD, ulwazi oluncinci lukhoyo malunga nokubaluleka kokuchithwa komchamo kwiikhompawundi ze-branched-chain.Siye savavanya ukucocwa kwezintso ze-leucine, i-valine, isoleucine kunye ne-alloisoleucine, kunye ne-2-oxo acids ehambelanayo 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S) - (S- KMV), kunye (R) -3-methyl-2-oxopentanoate (R-KMV), usebenzisa izibini zeplasma kunye neesampuli zomchamo (n = 63) kwizigulane ze-10 ezine-MSUD yeklasi.I-fractional renal excretion ye-BCAA yamahhala yayikuluhlu oluqhelekileyo (<0.5%) kwaye luzimeleyo kwi-plasma concentrations.I-excretion of bound (N-acylated) BCAA yayiqhelekile kwaye ayixhomekeke kakhulu kwi-BCAA ye-plasma concentrations.I-fractional renal excretion ye-BCOA yayiku-oda KIC << KIV
