
eiii. tii. pii5iii

manushyaril "eiii. tii. pii. 5eph1iii" jeane encode cheiyunna oru ensaimaanu eiii. tii. pii synthase eph1 subunit epsiloon, mitocondrial. atp5eph1iii encode cheytha protein atp sinthesinte oru upavibhagamaanu, ithu complexes vii ennum ariyappedunnu. eee jeeninte vyathiyanangal mitocondrial complexes vii kuravu, nuclear 3 (emsy5dn3), pappillary thyroid cancer ennivayumaayi bandhappettirikkunnu.
pravartthanam.
eee jeane mitocondrial eiii. tii. pii sinthesinte oru upavibhaagathe encode cheiyunnu. auxidative phosphorylation samayat aantharika stharathiludaneelam protonukalude electrochemical gradient upayogichu mitocondrial eiii. tii. pii synthase eiii. tii. pii sinthasisine uthejippikkunnu. atp synthase rund bandhippicha multy-subunit complexukal chernnathaanna layikkunna kaattalatik coor, eph 1, prottoon chaanal ulkkollunna membran-spanning ghadakam, epho. mitocondrial eiii. tii. pii sinthesinte kaattalatik bhaagath 5 vyathyastha subunitukal (alfa, beetta, gaama, deltta, epsiloon) adangiyirikkunnu, 3 alfa, 3 beetta, gaama, deltta, epsiloon ennivayude oronninteyum stoikiyometri. prottoon chanalil moonnu pradhana upavibhagangal (eiii, bii, sii) adangiyirikkunnu. eee jeane kaattalatik korinte epsiloon sabyoonittine encode cheiyunnu. eee jeeninte rund syoodojinukal chromosomukalaaya 4,13 ennivayil sthithicheyyunnu.
ghadana.
eiii. tii. pii. 5eph1iii" jeane, chromosome 20-nte kyu kaikalil <aii. dii. 1> sthaanathu sthithicheyyunnu, ithu 3 exonukal kondu nirmmichathum 3,690 bays jodikalude neelamullathumaanu. 51 amino aasidukal chernna 5.7 kiloda bhaaramullathaan eiii. tii. pii. 5eph1iii protein. 14 nuclear, 2 mitocondrial-encode cheytha subunitukal adangiya complexes vii ennum ariyappedunna eph1phoo atpesinte oru sabyoonittaan protein. ensaiminte naamakaranathinu oru neenda charithramund. eph1 bhinnasankhya athinte peru "bhinnasankhya 1" enna padatthil ninnaan uruthirinjathu, koodaathe epho (subscript aksharam "oo" ennu ezhuthuka, poojyamalla) athinte peru oligomycin enna swaabhaavikamaayi uruthirinja oru tharam antibayottikkinulla binding bhinnasamkhyayil ninnaan uruthirinjathu, ithu eiii. tii. pii sinthaasinte epho yoonittine thadayaan kazhiyum. eph1 kanika valuthaanu, ithu transmission electron microscopyl negatteevu staining vazhi kaanaan kazhiyum. aantharika mitocondrial membranine kurumulak akkunna 9 enm vyaasamulla kanangalaaniva. avaye yadhaarthathil praadhamika kanikakal ennu vilikkukayum mitocondriyante muzhuvan shwasana upakaranavum adangiyirikkumennu karuthukayum cheythirunnuvenkilum, oru neenda pareekshana parambarayiloode, ephraim rackerk addehathinte sahapravarthakarkkum (1961 ll aadyamaayi eph 1 kanika verthiricheduthavar) eee kanika uncupled mitocondriyayile atpes pravarthanavumaayum mitocondriyaye ultraasoundilekku thurannukaattunnathiloode srishtikkappetta sarjantondriyal kanangalile atpes pravarthanavumaayum bandhappettirikkunnave kaanikkan kazhinju. eee atpes pravartthanam pala laborattarikalilum neenda pareekshanangalude oru parambarayiloode eiii. tii. pii srishtikkunnathumaayi kooduthal bandhappettirikkunnu.
pravartthanam.
mitocondrial membran eiii. tii. pii synthase (eph1phoo eiii. tii. pii synthase allengil complexes vii) shwaasakosha shringalayude electron transport complexukal srishtikkunna membraniludaneelam oru prottoon gradiantinte saannidhyathil eiii. dii. piyil ninnu eiii. tii. pii uthpaadippikkunnu. eph-type atpesukalil rund gadanaaparamaaya domainukal adangiyirikkunnu, eph 1-extramembronous kaattalatik coor adangiyirikkunnu, koodaathe epho-membran prottoon chaanal adangiyirikkunnu, ithu oru central stankil ninnum oru peripheral stankil ninnum bandhippichirikkunnu. catalis samayat, eph 1-nte kaattalatik domainile eiii. tii. pii synthesis central staakk sabyoonittukalude oru rottari mechanism vazhi prottoon translocationilekku bandhippikkunnu. sankeernamaaya eph1 domaininteyum sankeernamaaya rottari moolakatthinte bhagamaaya central stankinteyum bhagam. chuttumulla alfa3beetta3 subunitukalodu kendra thandinte bhramanam beetta subunitukalile moonnu vyathyastha kaattalatik saittukalil (samaanathayode) eiii. tii. piyude jalavishleshanathilekku nayikkunnu.
eph1 complexinte thandu mekhalayil sthithi cheiyunna epsiloon subunit, atpes kaattalitik korinte inhibittaraayi pravartthikkunnu. epsiloon sabyoonittin churungiyathum vipuleekarichathumaaya rund roopeekaranangal anumanikkaan kazhiyum, avide randaamathethu eiii. tii. pii jalavishleshanathe thadayunnu. epsiloon sabyoonittinte roopeekaranam nirnnayikkunnath gaama sabyoonittinte bhramanathinte dishayiloodeyum orupakshe eiii. dii. piyude saannidyathiloodeyumaanu. epsilan subunit eiii. dii. piyude saannidhyathil vipuleekarikkappedumenna karuthappedunnu, athuvazhi paazhaya eiii. tii. pii jalavishleshanam thadayunnathinulla oru suraksha loock aayi pravartthikkunnu.
klinikkal praadhaanyam.
eiii. tii. pii. 5eph1iii" jeenil undaakunna parivartthanangal mitocondrial complexes vii kuravu, nuclear 3 (emm. sii. 5dn3), dysmorphic savisheshathakal, psychomoter retardation, hypotonia, valarchaa retardation, cardiomayoppathy, karal, hypoplastic vrukkakal, moothram, plasma, cerebrospinal draavakam ennivayile uyarnna laaktett alavu ennivayulppedeyulla vaividhyamaarnna klinikkal prakadanangalulla oru mitocondrial disorderinu kaaranamaakunnu. rogalakshanangalude vyathiyanangalil "eiii. tii. pii5iii" jeenile oru homocygus tyre12sii mutation ulppedunnu, ithu navajaathashishukkalude sankeernamaaya vii kuravumaayi laaktik acidosis, 3-methylglutaconic aciduria, neriya maanasika vaikalyam, peripheral neuropathy ennivayumaayi bandhappettirikkunnu.
eiii. tii. pii. 5eph1iiiyude kuranja aavishkaaram pappillary thyroid cancer roganirnayavumaayi ganyamaayi bandhappettirikkunnu, ithu rogathinte aadyakaala tumour markeraayi varthichekkaam. thyroid kaansarinte ettavum saadhaaranamaaya tharamaan papillary thyroid cancer, ithu ellah thyroid cancer kesukalilum 75 sathamaanam muthal 85 sathamaanam vare prathinidheekarikkunnu. ithu sthreekalil kooduthalaayi kaanappedukayum <aii. dii. 1> prayaparidhiyil kaanappedukayum cheiyunnu. thyroid cancer ulla kuttikalilum thalayilum kazhuthilum munpu vikiranamundaaya thyroid cancer rogikalilum ithu pradhana cancer tharamaan.
ashayavinimayam.
28 qoo-complexes edapedalukal ulppede 34 bainari protein-protein edapedalukal eiii. tii. pii. 5eph1iiiyill undennu theliyikkappettittund. eiii. tii. pii. 5eph1iii. eiii. tii. pii. 5eph1dii, agtrap, saip17eiii1, ube2enn ennivayumaayi samvadikkunnathaayi kaanapedunnu.

