Video: Yuav kho tus kab mob urea cycle li cas?
2024 Tus sau: Michael Samuels | [email protected]. Kawg hloov kho: 2023-12-16 01:44
Kev kho mob nrog Tshuaj
Sodium phenylbutyrate los yog buphenyl yog ammonia scavenger uas tau siv dav rau kho UCDs. Sodium benzoate kuj tseem siv rau kev tshem tawm ammonia tsis tu ncua los ntawm cov ntshav. Qhov ncauj lactulose thiab neosporin tuaj yeem pab tiv thaiv cov tshuaj ammonia los ntawm cov kab mob hauv cov nyuv.
Tsis tas li lo lus nug yog, dab tsi yog qhov teeb meem ntawm urea voj voog?
Nrog rau CPSI thiab NAGS tsis txaus, OTC tsis txaus yog qhov hnyav tshaj plaws ntawm urea cycle tsis meej . Cov neeg mob uas ua tiav qhov tsis txaus OTC sai sai tsim hyperammonemia nyob rau lub sijhawm yug menyuam tshiab. Cov neeg mob uas tau txais txiaj ntsig zoo los ntawm kev kub ntxhov yog qhov pheej hmoo rau kev rov ua dua ntawm hyperammonemia.
Tsis tas li ntawd, vim li cas peb thiaj xav tau lub voj voog urea? Lub hom phiaj tseem ceeb ntawm urea cycle yog kom tshem tawm cov tshuaj ammonia lom los ntawm lub cev. Kwv yees li 10 txog 20 g ntawm ammonia yog tshem tawm ntawm lub cev ntawm cov neeg laus noj qab haus huv txhua hnub. Ib qho tsis ua haujlwm urea voj voog yuav txhais tau tias ntau tshaj ntawm ammonia hauv lub cev, uas tau ua rau hyperammonemia thiab kab mob cuam tshuam.
Qhov thib ob, yuav kuaj tus kab mob urea cycle li cas?
Kev kuaj lub siab tuaj yeem ua tiav kom paub tseeb tias kuaj mob vim nws tuaj yeem qhia qib qis ntawm kev ua haujlwm enzyme. Kev tshuaj ntsuam caj ces kuj tuaj yeem ua tiav los qhia seb puas muaj teeb meem nrog ib qho ntawm cov noob xav tau txhawm rau txhawm rau zom cov protein ntawm cov urea lub voj voog txhawm rau txheeb xyuas hom tshwj xeeb ntawm urea cycle tsis meej.
Dab tsi enzyme hloov urea rau ammonia?
Thawj kauj ruam, uas tseem yog tus nqi txwv, suav nrog hloov dua siab tshiab ntawm CO thiab ammonia mus rau carbamoyl phosphate los ntawm lub enzyme carbamoyl phosphate synthetase I (CPS I). Ammonia yog lub hauv paus ntawm thawj amine pab pawg ntawm urea ua.
Pom zoo:
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