3-Hydroxy-4,5-bis(hydroxymethyl) -2-methylpyridine Cas: 65-23-6 Te paura ma
| Nama Putumōhio | XD90442 |
| Ingoa Hua | 3-Hydroxy-4,5-bis(hydroxymethyl) -2-methylpyridine |
| CAS | 65-23-6 |
| Tātai rāpoi | C8H11NO3 |
| Taumaha rāpoi | 169.18 |
| Nga Taipitopito Rokiroki | Ambient |
| Waehere Rere Whakariterite | 29362500 |
Whakatakotoranga Hua
| Te ahua | Te paura ma |
| Te whakamatautau | >99% |
| Kiato | He rite tonu te wa pupuri |
| Te toenga i runga i te whakaurunga | <0.5% |
| Tautuhinga | Tauhohenga pai |
| Ngaronga i te whakamaroke | <0.5% |
| Kawakawa | 2.6 |
E rua nga huringa i roto i te ira cystathionine beta-synthase (CBS) i kitea i roto i nga teina e rua Hapanihi whai pyridoxine homocystinuria kore-whakautu he rereke nga taumata methionine i roto i o ratau toto i te wa e hapu ana.Ko nga turoro e rua he heterozygotes pūhui o nga allele mutant e rua: ko tetahi he whakawhiti A-ki-G i te nucleotide 194 (A194 G) i puta he whakakapinga histidine-ki-arginine i te waahi 65 o te pūmua (H65R), ko tetahi he he Ko te whakawhitinga G-ki-A i te nucleotide 346 (G346A) i puta he whakakapinga glycine-ki-arginine i te waahi 116 o te pūmua (G116R).Ko nga pūmua mutant e rua i wehe motuhaketia i roto i te Escherichia coli, a karekau he mahi catalytic.Ahakoa te rite o o raatau momo momo me te rite tonu te kai o te pūmua, he rerekee nga taumata o te methionine toto i enei tuākana i te wa o te whanau hou, e kii ana ko te taumata o te methionine i roto i te toto ka whakatauhia ehara i te mea ko te koha o te ira CBS me te whakauru pūmua, engari na te te mahi o etahi atu enzyme e uru ana ki te methionine me te homocysteine whakawhitiwhiti, ina koa i te wa o te wa whanau.No reira, ko nga kohungahunga morearea nui kei a ratou teina he homocystinuria, ahakoa he mea noa te taumata o te methionine i roto i o ratou toto i roto i te tirotiro papatipu whanau hou, me whai me te tirotirohia e te whakamatautau o te mahi enzyme, te tātari ira ranei kia taea ai te maimoatanga. i timata wawe ki te aukati i te whakawhanaketanga o nga tohu haumanu.I tua atu, me whakawhanake he tikanga hou, he maamaa ake mo te tirotiro papatipu mo te ngoikoretanga o te CBS i roto i nga tamariki hou.
