3-Hydroxy-4,5-bis (hydroxymethyl)-2-methylpyridine Cas: 65-23-6 Dawb hmoov
| Catalog Number | xwm 90442 |
| Khoom npe | 3-Hydroxy-4,5-bis (hydroxymethyl)-2-methylpyridine |
| CAS | 65-23-6 |
| Molecular Formula | C8H11NO3 |
| Molecular Luj | 169.18 Nws |
| Cov ntaub ntawv khaws cia | Ambient |
| Harmonized tariff Code | 29362500 ib |
Khoom Specification
| Qhov tshwm sim | Dawb hmoov |
| Assay | > 99% |
| Qhov ntom | Tib lub sijhawm khaws cia |
| Residue ntawm ingnition | <0.5% |
| Kev txheeb xyuas | Cov tshuaj tiv thaiv zoo |
| Poob rau ziab | <0.5% |
| Acidity | 2.6 |
Ob qhov kev hloov pauv hauv cystathionine beta-synthase (CBS) gene tau pom nyob rau hauv ob tug kwv tij Nyij Pooj nrog pyridoxine tsis teb homocystinuria uas muaj ntau hom methionine hauv lawv cov ntshav thaum lub sij hawm cev xeeb tub.Ob tus neeg mob tau sib xyaw heterozygotes ntawm ob lub mutant alleles: ib qho tau hloov pauv A-rau-G ntawm nucleotide 194 (A194 G) uas ua rau muaj kev hloov pauv histidine-rau-arginine ntawm txoj haujlwm 65 ntawm cov protein (H65R), thaum lwm tus muaj ib qho G-rau-A kev hloov pauv ntawm nucleotide 346 (G346A) uas ua rau muaj kev hloov pauv glycine-rau-arginine ntawm txoj haujlwm 116 ntawm cov protein (G116R).Ob qhov mutant proteins tau cais tawm hauv Escherichia coli, thiab lawv tsis muaj catalytic.Txawm hais tias lawv cov genotypes zoo ib yam thiab yuav luag muaj protein ntau sib npaug, cov kwv tij no tau pom ntau qib ntawm cov ntshav methionine thaum lub sij hawm cev xeeb tub, qhia tias qib ntawm methionine hauv cov ntshav yog txiav txim siab tsis yog los ntawm qhov tsis xws luag hauv CBS noob thiab noj cov protein, tab sis kuj los ntawm cov methionine hauv cov ntshav. Kev ua haujlwm ntawm lwm cov enzymes koom nrog hauv methionine thiab homocysteine metabolism, tshwj xeeb tshaj yog thaum lub sij hawm cev xeeb tub.Yog li ntawd, cov me nyuam mos uas muaj kev pheej hmoo siab uas muaj cov kwv tij nrog homocystinuria, txawm tias theem ntawm methionine hauv lawv cov ntshav yog ib txwm nyob rau hauv kev tshuaj ntsuam xyuas menyuam yaus, yuav tsum tau ua raws thiab kuaj xyuas los ntawm kev soj ntsuam ntawm kev ua enzyme lossis kev tshuaj ntsuam gene kom kho tau. pib sai li sai tau los tiv thaiv kev txhim kho ntawm cov tsos mob.Tsis tas li ntawd, yuav tsum tau tsim ib txoj hauv kev tshiab, rhiab dua rau kev soj ntsuam loj ntawm CBS tsis txaus hauv cov menyuam yug tshiab.
