pyruvet dehydrogenase (lipoamide) alfa 2

pyruvet dehydrogenase (lipoamide) alfa 2, jyala pyruvet dehydrogenase eae1 ghatak subunit alfa mhanoon dekhil olakhale jaate, vrushan-vishisht swaroop, mitochondrial kinwa pii. dii. eae. 1-aii prakar II, heey eka enzyme aahe jeey manavanmadhye "pii. dii. etch. aii. 2" janukadware encode kele jaate.
rachana.
done paripakv pii. etch. aii. prathine done pii. dii. etch. bii. prathinansah ekatra yeuun heterotetrameric eae1 upghatak tayar karatat. sphatik sanrachananna asha pratimanasathi parvanagi aahe jyamadhye utpreran karnyasathi enzyme tyachya heterodimerchya 2-aii shatalsarkhya halchalitun jaato. manvi pii. etch. aii. 2 janukadware encode kelele prathine heey pyruvet dehydrogenase multienzime complexcha bhag aahe. sampoorn manvi sankul 9.5 emm. dii. aii. aakarache aahe aanee tyache varnan 60-american asey kele gele aahe, mhanje sampoorn sankul tayar karnyasathi 60 hoon adhik ghatak ekatra kele jaatat. heey upghatak anek prajatinmadhye sanrakshit kele jaatat, kaaran serve eukariotsasathi aii. tii. pii. nirmitisathi yaa sankulache karya aavashyak aahe.
pratyek ghatak yaa margatil eka tappyachya utpreranasathi jababdar aahe; heey sankul pratyek abhikriyechya madhyavarti ghatakanna pudhil enzyme pathvanyachya uddeshane astitvat aahe, asha prakare abhikriyecha darr mothya pramanat vadhato.
karya.
pyruvet dehydrogenase complexes heey payaruvetchya oxidative decarboxylationsathy jababdar aahe, antim utpadan acetyl coa aahe. ekoon paach abhikriyanna sanklisht utprerit karate, ekoon abhikriya ashee aaheh
pyruvet + see. oo. aii. + enae. dii. + → acetyl-see. oo. aii. + see. oo. 2
yaa sankuladware paar padalya chalavalya 5 tappyanmadhye teen bhinn saha-utsechakanchi aavashyakta asateh thyamine pyrophosphate (tii. pii. pii.), lipoamide aanee saha-utsechak aii. hii payari yukeriyotsdware chalavalya chalavalya madhyavarti chayapachay marganpaiki eka aahe, jyamadhye glukojche auksidikaran karoon carbon dye auxide, paanee aanee aii. tii. pii. tayar kele jaate. eae1 sankul visheshtah payaruvetache calfa-see (= oo) bandh todanyasathi tii. pii. pii. cofactorcha vapar karate aanee nantar acetyl gut tii. pii. pii. coenzyme hastantarit karate, parinami madhyavarti, hydroxylathyl-tii. pii. pii. * eae1 tayar hote aanee see. oo. 2 tayar hote. tii. pii. pii. vareel thyzolium ring carbonyl gatanmadhye jodanyasathi aanee electron sink mhanoon karya karnyasathi aadarsh aahe, kinwa eka gut joou pratikriyetun electron khechu shakato aanee electron-kamatarata madhyavarti sthir karoo shakato.
niyam.
sastan pranyanchya uutinmadhil pii. dii. etch. sankulachi kriya mothya pramanat sankulatil vishisht upaghatakanchya phosphorylationdware nirdharit kelee jaate. asha prakare, mitochondriamadhye vyakt jhalelya site-vishisht kinesses aanee phosphatechya paripoorn pramanancha thet pii. dii. etch. kriyakalapanvar parinam hoto.
heey januk bahutek nishkriy asalyane, vrushan uutinmadhye vagalata, eka methylation yantrana aahe jee heey januk sharirik peshinmadhye nishkriy karate. coding kshetratun methyl gut kadhoon takalyane inn vitro enzyme sakriy jhalyache disun aale aahe.
vaidyakeey mahatva.
pii. etch. etch. 2 janukatil utparivartananmule pyruvet dehydrogenage kamtaratecha eka prakar hoto. pyruvet dehydrogenage kamatarata hii sharirat lactic acid navache rasayan tayar hone aanee vividh prakarchya majjasansthesambandhi samasyanmule darshavili jaate. yaa sthitichi chinhe aanee lakshane sahasa janmanantar lagechach pratham disatat aanee tyaa prabhavit vyaktinmadhye mothya pramanat badaloo shakatat. sarvat samanya vaishishtya mhanje sambhavyapane jivghene asalele lactic acid (lactic acidosis) tayar hone, jyamule malmal, ulatya, shvasanachya gambhir samasya aanee hrudayache thoke asamanya houu shakatat. pyruvet dehydrogenage kamatarata asalelya lokanna samanyatah majjasansthesambandhi samasya dekhil asatat. bahutansh lokanni mansik kshamata aanee basane aanee chalane yasarkhya chalanyachya kaushalyancha vikas karnyas vilamb keela aahe. itar majjasansthesambandhi samasyanmadhye bauddhik apangatva, khilkhilepana, snayuncha kamkuvat rung (hypotonia), kharab samanvay aanee chalanyat adchan yancha samavesh asoo shakato. kahee prabhavit vyaktinmadhye menduchya davya aanee ujavya bhaganna bhaganna uutincha avikasit vikas (corpus calosum), menduchya bahereel bhagacha ksheenata jyala cerebral cortex mhanatat kinwa menduchya kahee bhaganvar kharab jhalelya uutinche (jakhama) patte yasarkhya menduchya asamanya sanrachana asatat. aarogyachya gambhir parinamanmule, pyruvet dehydrogenage kamatarata asalelya anek vyakti mageel balpanapasun jagoo shakat naheet, jaree kahee jann paugandavasthet kinwa praudhavasthet jagoo shakatat. utparivartan pramukhyane pii. dii. etch. aii. 1 janukamadhye disun yete.
mahilanmadhye hii kamatarata olakhane khoop katheen asoo shakate. heey fibroblastmadhye tirkas ex nishkriyata namuna enzyme mapan asanyachi shakyata asalyamule aahe, mhanje enzyme kriyakalap mapan purnapane achook nasel. yaa vikarache vaidyakeey sadarikaran oxidative phosphorylationmadhil kamataratesah mothya pramanat overlap hott asalyane, pyruvet dehydrogenage kamatarata asalyacha sanshay asalelya mahilanmadhil snayunchya biopsiver tapshilvar jaivarasayanik vishleshan karanyachi shifaras kelee jaate, tyanantar pii. etch. aii. 1 janukache aanvik anuvanshik vishleshan kele jaate.
itar janukanmadhil utparivartananmule pii. dii. etch. chee kamatarata asalelyansathi, niyaman mhanoon methylation heey eka upchar mhanoon aashvasak asalyache darshavate, kaaran januk demethylationdware inn vitro sakriy kele gele aahe.

