carbamoil fasfate synthetase I

carbamoil fasfate synthetase I anedi mitochondrialo unna oka ligase enjaim, idhi euria utpattilo paalgontundi. carbamoil fasfate synthetase i (cps1 ledhaa cpsi) oka ammonia anuvunu bicarbonate anuvuku badilee chestundi, idhi ATP anuvu dwara phosphorylate cheyabadindi. phalitamgaa vachhe carbamate appudu maroka etipi anuvutho phosphorylate cheyabadutundi. carbamoil fasfate yokka phalitamgaa vachhe anuvu enjaimnu vadilivestundi.
nirmaanam.
ii. loe. kolai anedi cpsi mariyu cpsi vidhulanu nirvahinche oke cps, idhi oka chinna upaghatakam mariyu sumaru 382 mariyu 1073 amaino aamlam avasheshaalatho pedda upaghatakamtho kuudina heterodymer, ayithe ksheeradaalalo (mariyu itara sakasherukaalalo) cpsi protein oke janyuvu dwara encode cheyabadutundi. chinna upabhaagamlo ammonia mariyu glutamatenu tayaaru cheyadaaniki glutamine yokka bandham mariyu demination kosam oka kriyaaseela pradaesam untundi. pedda upabhaagamlo rendu kriyaaseela pradaesaalu unnaayi, okati carboxifasfate utpatti kosam mariyu marokati carbamoil fasfate utpatti kosam. pedda upabhaagamlo rendu domainlu (bii mariyu sii) unnaayi, okkokkati eah. tii. pii-grap kutumbaaniki chendina kriyaaseela pradaesamgaa unnaayi. rendu upabhaagaalanu anusandhaaninche oka rakamaina sorangam, idhi ammonianu chinna upabhaagamu nundi pedda upabhaagaaniki nirdesistundi.
yantraangam.
cpsilo sambhavinche mottam praticharyah
praticharyah + + − + + + → → + carbamoil fasfate + paie
eee praticharya moodu vibhina dasallo jarugutundani bhaavinchavachchu.
niyantrana.
sii. pii. ess. ii. nii enn-acetylglutemate niyantristundi, idhi sii. pii. ess. 1 yokka tappanisari alosteric activatorga panichestundi. enn. zii, domine domine bandhinchadam dwara, eah-looplo maarpulanu prerepistundi mariyu mariyu idhi domine domine yokka timm-looptho paraspara charyalanu maarchadaaniki daariteestundi, idhi apo roopamlo β-hairpin nundi ligand-bound roopamlo vistruta loopku puurtigaa punarvyavastheekaristundi. eee chivari roopamlo, t′-loop tunnell-loop mariyu l1 domine yokka t-looptho kooda sankarshana chendutundi, tadwara sakriyam chese samacharanni bicarbonate-phosphorylating domainku badilee chestundi. naag thoo eee paraspara charya mariyu nucleotidetho rendava paraspara charya, cpsi yokka kriyaaseela roopaanni sthireekaristundi.
eee ligand yokka avasaram kooda adhika natrajani saandratanu anusandhaanistundi, idhi naag utpatti cheyadaaniki glutamate mariyu arjinin kante ekkuvagaa pratibimbistumdi, eee adanapu tolaginchadaaniki cpsi charyalo perugudalaku.
jeevakriya.
protein mariyu natrajani gvakriyalo sii. pii. ess. ii keelaka paatra poeshistumdi. glutamine ledhaa glutamate dwara ammonianu mitochondrialoki teesukuvacchina tarvaata, carbamoil phosphatenu roopondinchadaaniki fasfate samuuhamtoe paatu bicarbonateku ammonianu jodinchadam cpsi pani. carbamoil phosphatenu euria chakramlo unchi chivariki eurianu srushtistaaru. eurianu tirigi rakta pravaahaaniki mariyu vadapotha kosam moothrapindaalaku mariyu visarjana kosam muutraasayaaniki badilee cheyavachu.
sambandhita aarogya samasyalu.
cpsiki sambandhinchina pradhana samasya janyusaastram aadhaaritamainadi. konnisaarlu janyu sankethamlo utparivartana kaaranamgaa sareeram taginanta cpsini utpatti cheyadu, phalitamgaa proteins mariyu natrajani yokka paelavamaina jeevakriya, alaage sareeramlo adhika sthaayi ammonia erpadutundi. idhi pramaadakaram endukante ammonia sariiraaniki, mukhyamgaa naadii vyavasthaku chaalaa vishapuuritam, mariyu medho vaikalyam mariyu muurchalaku daariteeyavachchu.

