Arginase-1 deficiency yog ib qho tsis tshua muaj tshwm sim los ntawm kev ua tiav lossis ib nrab tsis muaj enzyme arginase hauv lub siab thiab cov qe ntshav liab. Arginase yog ib qho ntawm 6 lub enzymes uas ua lub luag haujlwm hauv kev sib tawg thiab tshem tawm cov nitrogen ntawm lub cev, txheej txheem hu ua urea voj voog.
arginase tuaj yeem pom qhov twg?
Nyob rau hauv cov tsiaj txhu feem ntau, muaj ob lub isozymes ntawm cov enzyme no; thawj, Arginase I, ua haujlwm hauv lub voj voog urea, thiab nyob hauv feem ntau hauv cytoplasm ntawm lub siab. Qhov thib ob isozyme, Arginase II, tau cuam tshuam rau hauv kev tswj hwm ntawm arginine / ornithine concentrations hauv cell.
Yuav ua li cas hauv arginase deficiency?
Arginase deficiency yog ib qho kev mob tshwm sim uas ua rau cov amino acid arginine (ib lub tsev thaiv ntawm cov proteins) thiab ammonia kom maj mam nce hauv cov ntshav. Ammonia, uas yog tsim thaum cov proteins tawg hauv lub cev, yog tshuaj lom yog tias qib siab dhau lawm.
Yuav ua li cas nrog arginine deficiency?
Cov tsos mob muaj xws li muaj teeb meem noj, ntuav, kev loj hlob tsis zoo, qaug dab peg, thiab cov leeg nqaij tawv nrog nce siab(spasticity). Cov neeg uas tsis muaj arginase kuj tseem tuaj yeem ua rau muaj kev loj hlob qeeb, poob ntawm kev loj hlob tseem ceeb, thiab kev tsis txaus ntseeg.
Dab tsi ua rau arginine deficiency?
Hauv paus ntsiab lus, muaj peb yam xwm txheej uas tuaj yeem ua rau arginine tsis txaus:Kev noj zaub mov tsis txaus ntawm arginine los ntawm kev tshaib plab lossis los ntawm kev noj cov zaub mov tsis txaus hauv arginine (txawm tias tom kawg tsis tau pom tias ua rau arginine tsis txaus rau cov neeg laus noj qab haus huv), nce catabolism ntawm arginine, …
