Fumarase yog TCA lub voj voog enzyme uas catalyzes hloov pauv ntawm fumarate rau L-malate hauv mitochondriaRaws li DNA ua rau lub cytosolic echoform ntawm fumarase nyob rau hauv lub nucleus, muaj, Nws cov kev ua enzyme catalyzes qhov rov qab hloov dua siab tshiab ntawm malate rau fumarate, yog li ua rau hauv zos tsub zuj zuj ntawm fumarate.
Fumarase deficiency ua dab tsi?
Fumarase deficiency yog ib qho mob tshwm sim uas cuam tshuam rau lub hlwb thiab lwm qhov ntawm lub paj hlwb. Cov tsos mob thiab cov tsos mob tuaj yeem suav nrog lub taub hau me me (microcephaly), kev loj hlob qeeb, kev noj zaub mov tsis zoo, cov leeg nqaij tsis muaj zog (hypotonia), tsis muaj kev vam meej, qaug dab peg, thiab lub ntsej muag txawv
fumarase siv qhov twg?
Fumarase lossis fumarase hydratase yog siv hauv lub voj voog citric acid los ua cov kauj ruam hloov pauv hauv kev tsim lub zog los ua NADH Nws metabolizes fumarate hauv cytosol, uas dhau los ua cov khoom lag luam. ntawm urea voj voog thiab amino acid catabolism. Nws catalyzes ntxiv dej rau fumarate ua S-malate.
Cov tshuaj tiv thaiv dab tsi yog catalyzed los ntawm fumarase?
Lub enzyme fumarase catalyzes reversible hydration ntawm fumarate rau malate. Cov tshuaj tiv thaiv catalyzed los ntawm fumarase yog qhov tseem ceeb rau cellular energetics raws li ib feem ntawm lub voj voog tricarboxylic acid, uas ua rau txo qis sib npaug rau tsav oxidative ATP synthesis.
Fumarase tswj hwm li cas?
Nyob rau hauv cov poov xab, cytosolic fumarase koom nrog hauv homologous recombination (HR) kho txoj hauv kev, los ntawm nws txoj haujlwm hauv DSB resection txheej txheem. Ib lub hom phiaj ntawm cov kev cai no yog resection enzyme Sae2. Hauv tib neeg lub hlwb, fumarase tau koom nrog hauv txoj kev kho tsis yog homologous kawg koom nrog (NHEJ).
