
keey. ell. eff. 2

krupelsarkha ghatak 2 (keey. ell. eff. 2), jyala fuffus krupelsarkha ghatak (ell. keey. ell. eff.) asehi mhanatat, heey eka prathine aahe jeey manavanmadhye gunasutra 19 vareel "keey. ell. eff. 2" janukadware encode kele jaate. teey zink boat pratilekhan ghatakanchya krupelsarkhya ghatak kutumbat aahe aanee teey manvi shariratil vividh jaivarasayanik prakriyanmadhye guntalele aahe, jyat fuffusacha vikas, bhrun erythropoisis, upakala akhandata, tii-seela vyavharyata aanee edipogenesis yancha samavesh aahe.
shodh.
erythroid krupelsarkha ghatak (ekalph kinwa keey. bull. eff. 1) haah krupelsarkha pahila ghatak sapadala. atyant sanrakshit see. aii. see. see. kshetranshi bandhun garbhachya haemoglobin (haemoglobin eff) pasoon praudh haemoglobin (haemoglobin aii) januk abhivyaktimadhye badal karnyas protsahan denyasathi bhrun erythropoisisasathi heey mahatvache aahe. "undaranchya garbhanmadhil "ekalph" apghatan eka pranghatak raktakshay phenotype tayar karate, jyamule garbh 14 vya divasaparyant mrutyu hoto aanee naisargik utparivartananmule manavanmadhye β + thalassemia hoto. tathapi, 'ekalph'-kamatarata asalelya undaranmadhye bhrun haemoglobin aanee garbh haemoglobin janukanchi abhivyakti samanya aahe aanee manvi beeta-globin sthanavaril serve januke see. aii. see. see. ghatak pradarshit karat asalyane, sanshodhakanni itar krupelsarkhya ghatakancha shodh ghenyas suruvat kelee.
keey. ell. eff. 2, jyala suruvatila undaranchya praudh fuffusatil uchch abhivyaktimule fuffus krupelsarkha ghatak mhatale jaat asey, 1995 madhye sankarikaran tapasani mhanoon eka. ell. eff. chya zink boat kshetracha vapar karoon pahilyanda vegale kele gele. maus fibroblastmadhil sankraman chachanidware, keey. ell. eff. 2 heey β-globin januk pravartkashi bandhalele dekhil disun aale, jyamadhye see. aii. see. see. see. kram ii. keey. ell. eff. sathee bandhan sthal asalyache darshavile gele aahe, jeey keey. ell. eff. 2 laa crupel-sarkhya ghatak kutumbacha sadasya mhanoon pushti karate. tevhapasoon, itar anek keey. ell. eff. prathine sapadali aahet.
rachana.
keey. ell. eff. kutumbache mukhya vaishishtya mhanje prathinanchya see-terminswar sthit 21 kinwa 23 amino acid avasheshanchya lambichya teen atyant sanrakshit cysteine 2/hystidine 2 zink botanchi upasthiti. heey amino acid anukram pratyek done cysteine aanee done hystidine avasheshanmadhye samanvayit ekach zink ionla chillet karatat. hii zink bote sanrakshit saat-amino aaml kramane jodali jaatat; tii. jee. eae. keey. pii. (vaay/eff) ex. zink bote serve keey. ell. eff. prathinanna see. aii. see. see. see. januk pravartkanshi bandhanyas saksham karatat, tyamule jaree teey vividh karye purn karoo shakatat (zink botanpasoon door samarupatechya abhavamule), teey serve samaan bandhan kshetra olakhatat.
keey. ell. eff. 2 hii sanrachanatmak vaishishtye dekhil darshavite. emm. aar. aine. aii. pratilekhachi lambi andaje 1.5 kilobes aahe aanee 37.7 kiloda prathinanmadhye 354 amino aaml asatat. keey. ell. eff. 2 heey aine-terminswar eka. ell. eff. saha kahee samarupata dekhil samayik karate, jyat proline-samruddha pradesh transactivition domain mhanoon karya karato asey manale jaate.
januk abhivyakti.
"keey. ell. eff. 2" haah pratham undaranchya praudh fuffusat aadhalala aanee too atyant vyakt keela jaato, parantu erythroid peshi, endothelium, lymphoid peshi, pleeha aanee pandhya charbiyukt uutinmadhye bhrunjanan darmyan dekhil too tatpurata vyakt keela jaato. heey endotheliummadhe garbhachya 9.5vya divasapasunach vyakt kele jaate.
"erythroid peshinmadhye "keey. ell. eff. 2" chey vishesh manoranjak abhivyakti swaroop aahe. heey adim aanee garbhachya nishchit erythroid peshinmadhye kiman vyakt kele jaate, parantu praudh nishchit erythroid peshinmadhye, visheshtah proerythroblast aanee polychromatic aanee orthochromatic normoblastmadhye atyant vyakt kele jaate.
maus nockout.
keey. ell. eff. 2'-kamatarata asalele under bhrun tayar karnyasathi bhrun mool peshinche samaroop punarsanyojan vaparale gele. garbhanmadhye vasculogenesis aanee angiogenesis heey donhi samanya hote, parantu gambhir raktasravamule 14.5 vya divashi teey mrut pavale. sookshm tunica madhyam aanee eneurismal dialations, jyache vighatan jhaale, vasculaturemadhye sadosh aakarvidnyan disun aale. mahadhamani raktavahinyansambandhi gulgulit snayu peshi samanya tunica madhyamat sanghatit karnyat ayashasvi tharalya aanee periciteschi sankhya qamee hotee. asha prakare yaa "keey. ell. eff. 2". chee kamatarata asalelya undaranni bhrunjanan darmyan raktavahinyanchya sthirikaranat "keey. ell. eff. 2". chee mahattvachi bhoomika darshavili.
keey. ell. eff. 2'-kamatarata asalelya bhrunatil bhrunatil pranghataktemule, fuffusancha vikas aanee karya yasarkhya samanya prasutinantarchya sharirshastrat 'keey. ell. eff. 2' chee bhoomika tapasane katheen aahe.
karya.
fuffusacha vikas.
undaranchya 'keey. ell. eff. 2' yaa apuya bhrunatoon fuffusachya kalya kadhoon takalya jaatat aanee samanya shwasanalikechya zadanpasun sanvardhit kelya jaatat. samanyatah "keey. ell. eff. 2"-kamatarata asalelya bhrunanmadhye aadhalanaya bhruniy pranghataktela talhanyasathi, "keey. ell. eff. 2" samaroop null mouse bhrun mool peshi tayar kelya gelya aanee chimerick prani tayar karnyasathi vaparlya gelya. heey "keey. ell. eff. 2"-abhavi bhrun mool peshi hadanche snayu, pleeha, hriday, yakrut, mootrapind, pooat, mendu, garbhashay, vrushan aanee tvachechya vikasat lakshaniy yogdan detat, parantu fuffusachya vikasat nahee. yaa garbhanmadhye fuffusachya vikasachya kalbahya tappyachya uttaradhat, naan pasarlelya esinar nalikansah fuffuse adakali hotee. yaulat, jangali prakarche bhrun fuffusachya vikasachya pesheey tappyat vistarit vayuvijanasaha janmala yetat. hyamule asey soochit hote qee keey. ell. eff. 2 haah fuffusachya vikasasathi garbhadharanechya usheera aavashyak asalela eka mahattvacha pratilekhan ghatak aahe.
bhrun erythropoisis.
keey. ell. eff. 2 aata bhrun erythropoiseesmadhye, visheshtah bhrun aanee garbh β-sarkhya globin januk abhivyaktiche niyaman karnyat mahattvachi bhoomika bajavate asey manale jaate. murine "keey. ell. eff. 2". chya kamataratechya bhrunat, samanyatah adim erythroid peshinmadhye vyakt kelya jaananya β-sarkhya globin janukanchi abhivyakti lakshaniyaritya qamee jhaali, jaree praudh β-globin januk abhivyaktivar parinam jhaala navhata.
manvi β-sarkhya globin januk abhivyaktimadhye klf2 chee bhoomika murine "klf2"-kamatarata asalelya garbhache manvi β-globin lokassah sankraman karoon aanakhi spasht kelee gelee. asey aadhalun aale qee ε-globin (garbhatil haemoglobinmadhye aadhalanare) aanee γ-globin (garbhatil haemoglobinmadhye aadhalanare) januk abhivyaktisathi keey. ell. eff. 2 mahatvache hote. tathapi, purvipramanech, keey. ell. eff. 2 praudh beeta-globin januk abhivyaktimadhye konatihi bhoomika bajavat nahee; heey eae. keey. ell. eff. dware niyantrit kele jaate.
tathapi, keey. ell. eff. 2 aanee eka. ell. eff. heey bhrun erythropoiseesmadhye parasparkriya karnare aadhalale aahet. undaranchya garbhatil 'keey. ell. eff. 2' aanee 'eka. ell. eff.' yaa donhi goshti kadhoon takalyane 10.5 vya divashi ekach divashi kadhoon taknyapurvi ghatak raktakshay hoto. heey soochit karate qee keey. ell. eff. 2 aanee eka. ell. eff. heey garbh aanee garbhatil β-sarkhya globin januk abhivyaktimadhye parasparkriya karatat. sashart nockout undarancha vapar karoon heey dakhavale gele aahe qee keey. ell. eff. 2 aanee eka. ell. eff. donhi thet β-sarkhya globin pravartkanshi bandhale jaatat. asey suchavanyasathi dekhil purave aahet qee keey. ell. eff. 2 aanee eka. ell. eff. heey 'maay. see.' pravartkashi sahakriyatmakpane bandhale jaatat, joou eka pratilekhan ghatak aahe joou garbhatil proerythroblastmadhil α-globin aanee β-globinchya januk abhivyaktishi sambandhit aahe.
endothelial physiology.
"keey. ell. eff. 2" hii abhivyakti drav laminar pravah katarane tanamule prerit hote, jase qee samanya endotheliummadhil rakt pravahamule hote.
heey yantrik sanvedanshil marg sakriy karate, jeey tyaa badalyat done marg sakriy karate; meek5/ark5 marg, joou mef2 sakriy karato, eka pratilekhan ghatak joou "klf2" januk abhivyaktiche niyaman karato; aanee piay3keey avarodh, joou "klf2" mrnach sthirata vadhavato. tii. aine. eff. aii. aanee aay. ell.-1bii. aii. sarkhya cytokinesche tyanchya receptors bandhan heey pratilekhan ghatak pii. 65 sakriy karate, jeey "keey. ell. eff. 2" abhivyakti dekhil prerit karate. tyanantar keey. ell. eff. 2 chee endotheliummadhe chaar pramukh karye aaheth
asha prakare samanya endothelium shariravidnyan niyantrit karnyat keey. ell. eff. 2 chee mahattvachi bhoomika aahe. asey gruhit dharale jaate qee etherosclerosismadhye myeloid-vishisht keey. ell. eff. 2 snrakshak bhoomika bajavate. keey. ell. eff. 2 dware prerit endothelial peshinmadhil januk abhivyakti badal heey atheroprotective asalyache siddha jhaale aahe.
tii-peshi bhinnata.
tii-lymphocytechya vibhedanamadhye keey. ell. eff. 2 chey eka mahattvache karya aahe. tii-peshi sakriy asatat aanee keey. ell. eff. 2shivay apoptosis honyachi shakyata jaast asate, jeey soochit karate qee keey. ell. eff. 2 tii-peshichi shantata aanee astitva niyantrit karate. "keey. ell. eff. 2"-kamatarata asalelya thymocytes dekhil thymuschya sthalantarasathi aanee sphingosine-1 phosphet receptor 1 sarkhya paripakv tii-peshinmadhye farak karnyasathi aavashyak asalele anek receptors vyakt karat naheet.
edipogenesis.
keey. ell. eff. 2 heey edipocytechya vibhedache nakaratmak niyamak aahe. keey. ell. eff. 2 heey preadepocytesmadhye vyakt kele jaate, parantu paripakv edipocytesmadhye nahee aanee pravartak kriyakalapanna pratibandhit karoon teey pii. pii. aar.-jee. emm. (peroxisome proliferator-activated receptor-jee. emm.) abhivyaktila prabhavipane pratibandhit karate. heey preadepocytes edipocytesmadhye honare vibhajan rokhate aanee asha prakare edipogenesis rokhate.