3-Hydroxy-4,5-bis (hydroxymethyl) -2-methylpyridine Cas: 65-23-6 ufa woyera
| Nambala ya Catalog | XD90442 |
| Dzina lazogulitsa | 3-Hydroxy-4,5-bis (hydroxymethyl) -2-methylpyridine |
| CAS | 65-23-6 |
| Molecular Formula | C8H11NO3 |
| Kulemera kwa Maselo | 169.18 |
| Zambiri Zosungira | Wozungulira |
| Harmonize Tariff Code | 29362500 |
Mafotokozedwe a Zamalonda
| Maonekedwe | White ufa |
| Kuyesa | > 99% |
| Kuchulukana | Nthawi yosungira yomweyi |
| Zotsalira pa ingnition | <0.5% |
| Chizindikiritso | Kuchita bwino |
| Kutaya pa Kuyanika | <0.5% |
| Acidity | 2.6 |
Kusintha kuwiri mu jini ya cystathionine beta-synthase (CBS) idapezeka mwa abale awiri aku Japan omwe ali ndi pyridoxine osayankha homocystinuria omwe anali ndi milingo yosiyana ya methionine m'magazi awo panthawi yaukhanda.Odwala onsewa anali ma heterozygotes amitundu iwiri yosinthika: m'modzi anali ndi kusintha kwa A-to-G pa nucleotide 194 (A194 G) yomwe idayambitsa m'malo mwa histidine-to-arginine pamalo 65 a protein (H65R), pomwe winayo anali ndi Kusintha kwa G-to-A pa nucleotide 346 (G346A) yomwe inachititsa kuti glycine-to-arginine ilowe m'malo mwa 116 ya mapuloteni (G116R).Mapuloteni awiri osinthikawo adawonetsedwa mosiyana mu Escherichia coli, ndipo analibe ntchito yothandiza.Ngakhale kuti ali ndi ma genotypes ofanana komanso amadya mapuloteni pafupifupi ofanana, abalewa adawonetsa milingo yosiyanasiyana ya methionine m'magazi a mwana wakhanda, zomwe zikuwonetsa kuti kuchuluka kwa methionine m'magazi sikudziwika kokha chifukwa cha kuwonongeka kwa jini ya CBS ndi ma protein, komanso ndi kuchuluka kwa methionine m'magazi. ntchito ya michere ina yomwe imakhudzidwa ndi methionine ndi homocysteine metabolism, makamaka pa nthawi ya ukhanda.Chifukwa chake, makanda omwe ali pachiwopsezo chachikulu omwe ali ndi abale ndi alongo omwe ali ndi homocystinuria, ngakhale mulingo wa methionine m'magazi awo ndi wabwinobwino pakuwunika kwa mwana wakhanda, ayenera kutsatiridwa ndikuzindikiridwa ndi kuyesa kwa enzyme kapena kuwunika kwa majini kuti chithandizo chitheke. anayamba posachedwapa kuteteza chitukuko cha matenda zizindikiro.Kuphatikiza apo, njira yatsopano, yovuta kwambiri yowunika kuchuluka kwa CBS kwa ana akhanda iyenera kupangidwa.
