I-3-Hydroxy-4,5-bis (hydroxymethyl) -2-methylpyridine Cas: 65-23-6 powder emhlophe
| Inombolo Yekhathalogi | XD90442 |
| Igama Lomkhiqizo | I-3-Hydroxy-4,5-bis(hydroxymethyl) -2-methylpyridine |
| I-CAS | 65-23-6 |
| I-Molecular Formula | I-C8H11NO3 |
| Isisindo samangqamuzana | 169.18 |
| Imininingwane Yesitoreji | I-ambient |
| Ikhodi Yentela Evumelanisiwe | 29362500 |
Ukucaciswa Komkhiqizo
| Ukubukeka | Impushana emhlophe |
| Isivivinyo | >99% |
| Ukuminyana | Isikhathi sokugcina esifanayo |
| Izinsalela ku-ingnition | <0.5% |
| Ukuhlonza | Ukusabela okuhle |
| Ukulahlekelwa Ekumisweni | <0.5% |
| I-Acidity | 2.6 |
Ukuguqulwa okubili kofuzo lwe-cystathionine beta-synthase (CBS) kwatholwa kuzelamani ezimbili zaseJapane ezine-pyridoxine engaphenduli i-homocystinuria eyayinamazinga e-methionine ahlukene egazini lazo ngesikhathi sokuzala.Zombili lezi ziguli zazingama-heterozygote ahlanganisiwe wama-allele amabili aguquguqukayo: enye yayinoguquko lwe-A-to-G ku-nucleotide 194 (A194 G) eyabangela ukufakwa esikhundleni kwe-histidine-to-arginine endaweni engu-65 yeprotheni (H65R), kanti enye ine- Ukuguqulwa kwe-G-to-A ku-nucleotide 346 (G346A) okuholele ekufakweni kwe-glycine-to-arginine endaweni ye-116 yeprotheni (G116R).Amaprotheni amabili aguquguqukayo avezwa ngokuhlukana ku-Escherichia coli, futhi ayentula ngokuphelele umsebenzi okhuthazayo.Ngaphandle kwama-genotypes afanayo kanye nokudla amaprotheni acishe alingane, lezi zelamani zabonisa amazinga ahlukene e-methionine yegazi ngesikhathi sosana, okuphakamisa ukuthi izinga le-methionine egazini alinqunywa kuphela ukukhubazeka kwezakhi zofuzo ze-CBS kanye nokudla kwamaprotheni, kodwa futhi umsebenzi wamanye ama-enzyme abandakanyeka ku-methionine ne-homocysteine metabolism, ikakhulukazi ngesikhathi sokuzalwa kwengane.Ngakho-ke, izingane ezisanda kuzalwa ezisengozini enkulu ezinezelamani ezine-homocystinuria, ngisho noma izinga le-methionine egazini lazo livamile ekuhlolweni kwenqwaba yezingane ezisanda kuzalwa, kufanele zilandelwe futhi zitholwe ngokuhlolwa komsebenzi we-enzyme noma ukuhlaziywa kofuzo ukuze ukwelashwa kungenziwa. waqala ngokushesha ngangokunokwenzeka ukuvimbela ukuthuthukiswa kwezimpawu zomtholampilo.Ukwengeza, indlela entsha, ebucayi kakhulu yokuhlolwa kwenqwaba yokuntuleka kwe-CBS ezinganeni ezisanda kuzalwa kufanele kuthuthukiswe.
