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підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

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1-40).

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CeprroBll,ll;HO-KJieToqHaH aHeMHH - roM03llfOTHoe peuecCHBHOe 3a60JieBaH.11e; Ilp05IBJUieTCH TOJibKO B TOM CJiyqae, KOf)la OT o6oMX po,!1,HTeJieii HaCJie,UyIOTC5I 2 MYTaHTHhIX retta ~-uerrew rno6ttHa. Ilocne po)l(}].eHHH pe6eHKa 6oJie3Hh He npo&B- JIHeTCH ,!1,0 Tex rrop, IlOKa 3HaqHTeJibHbie KOJI}lqeCTBa HbF He 3aMecrnTcH Ha HbS. Y 6oJihHhIX Bbl5IBJI5IIOT KJIMHllqecKHe CMMITTOMhl, xapaKTepHbie ,ll,JIH aHeMllll: fOJIOBOKpj')KeHHell fOJIOBHhle 60JI.11, o,UhIIIIKa, yqalll,eHHoe cep,!1,ue6.11emrn, 60JI.11 B KoHeqHocTHX, IlOBbIIIIeHttyIO BOCIIPllllMqHBOCTb K llH- <PeKUHOHHbIM 3a6oJieBaHHHM.

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BhieoKAA qacrora retta HbS epe,UH )l(ITTeJieU: AcpPHKH (,UO 40% HaeeneHAA B HeKOTOpbIX paU:ottax) o6yCJioRJieHa reM, qro rerepo3HroThI Mettee qyBCTBHreJibHbI K MaJI51pmr, qeM JIIO,UH e HOPMaJibHbIM reMorJIOOHHOM A. Plasmodium falciparnm - B036y,UHTeJlh MaJI5IPHH, o6JIHrarttyIO qaerh eBoero )l(H3HeHHOfO UHKJia OH TipoBO,llHT B 3PH'IpOUHTax.Ta.K KaK 3pHT- (JOUHTbl rerepo3HrorHbIX no HbS JIIO)leH 11Me10r 6o- nee KOpoTKHH epoK )l(H3HH, qeM HOPMaJibHbie 3Pl1T- (JOUHTbI, oo36y,UHreJ!b Mairnpmr He yeneBaeT 3a.KOWilITh Heo6XO,llHMyIO era,UHIO pa3BHT1151. ~ e03,Uaer H36HpareJibHOe npeHMYIUeerBo M5I rerepo311fOTHhIX no HbS JIIO,UeU: B rex o6naernx, r,Ue MaJI51pH51 BhI3hIBaer rn6eJlh MHornx JIIO,UeU:.

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CTaBJUUOT OKOJIO 1/4 scex 6eJIKOB opraHH3Ma qeJIOBeKa. B Me)l(KJieToqHoM MaTp11Kce MOJieK}'JihI KOJIJiareHa 06pa3y10T IIOJIHMepbl, Ha3bIBaeMbie cjm6p11JIJiaM11 KOJIJiareHa (6oJiee rro.upo6Ho 3TO OIIHCaHO B pa3,lleJie 15). <l>116p11JIJihl KOJIJiareHa o6Jia,UalOT orpoMHOH npO'lHOCThlOH rrpaKTH'leCKM HepacTIDKHMhI. 0HH MOfYT Bhl,Uep)((HBaTh Harpy3-

KY, B 10 000 pa3 rrpeBbIUialOIUYIO HX co6crneHHhlll Bee. TIO IIpO'lHOCTHKOJIJiafeHOBbie qm6pHJIJihl rrpeBOCXO,ll,HT rrpoqHOCTh CTaJihHOH rrpoBOJIOKM TOfO )((e ce'leHHH. HMeHHO II03TOMY 6oJihUioe KOJI11qecrno KOJIJiareHOBblX BOJIOKOH, COCTOHIUHX 113 KOJIJiareHOBhIX cjm6pHJIJI, BXOllHT B COCTaB KO)((Jf, cyxO)((JfJIHH, xpHIUeH: H KOCTeH:.

Heo6b1qHhie MexaH11qecKMe cBoH:crna KOJIJiareHOB CBH3aHbl c HX nepBH'lHOHH rrpocTpaHCTBeHHOH CTpYKTYPaMH. MoJieK}'JihlKOJIJiareHa COCTOHT 113 Tpex IIOJIHIIeIITHJlHhIX uerreH:, Ha3hIBaeMhlX cxuellilMH. lfaeHT11cp11u11poBaHo 6oJiee 20 cx-uerreH:, 60JihlllHHCTBO KOTOpblX HMeeT B CBOeM COCTaBe 1000 aMHHOKHCJIOTHhlX OCTaTKOB, HO uerr11 HeCKOJihKO OTJIH'lalOTCH aMHHOKHCJIOTHOH IIOCJie,llOBaTeJihHOCThlO. B COCTaB KOJIJiareHOB MOryT BXOJl,HTh TPH O,llHHaKOBbJe HJIH pa3Hble uerr11.

TiepBH'lHaHCTPYKTYPa cx-uerreH: KOJIJiareHa Heo6hI'lHa,TaK KaK Ka)((JlaH TpeThH aMHHOKHCJIOTa B IIOJIHIIeIITHllHOH uerr11 rrpe.ucTaBJieHa fJIHUHHOM, OKOJIO 1/4 aMHHOKHCJIOTHhIX OCTaTKOB COCTaBJIHlOT IlpOJIHH HJIH 4-fH,llpOKCHIIpOJIHH, OKOJIO 11 % - anaHHH. B KOJIJiareHe OTCYTCTBYIOT TaKMe aMHHOKHCJIOThl, KaK UHCTeHH H TPHIITOcpaH, a fHCTH,llHH, MeTHOHHH H THP03HH HaXO,llHTCH JIHlllh B O'leHh He6oJihllIOM KOJI11qecrne. B cocrnse rrepBH'IHOHCTpYKTYPhl cx-uerr11 KOJIJiareHa co.uep)((HTCH TaK)((e He06bI'!HaH aMHHOKHCJIOTa - fHJJ,pOKCHJIH3HH. TioJI11rreIITHllHYIO uerrh KOJIJiareHa MO)((HO rrpe.ucTaBMTh KaK rrocJieJJ,osaTeJihHOCTh TpmmeTOB fJIH-X-Y, r.ue x H y MOryT 6hITh Jil06bIMH aMHHOKMCJIOTaMH, HO 'laIUe B IIOJIO)((eHHM x CTOHT rrpOJIMH, a B IlOJIO)((eHMM y - fHllPOKCMIIPOJIMH HJIH r11.upoKCMJIH3MH. Ka)((JlaH 113 3THX aMHHOKHCJIOT HMeeT 6oJihUioe 3Haqemrn MH cpopM11posaHHH KOJIJiareHOBhIX cp116p11JIJ1.

TipOJIHH 6Jiaro.uapH CBoe:H CTpYKTYPe Bhl3bIBaeT M3fl16bI B IIOJIHIIeIITHJlHOH uerr11, CTa6MJIH3HPYH JieB03aKpyqeHHYlO CIIHpaJihttylO KOHcpopMaUHIO. Ha OJl,MH BMTOK CIIMpaJIH TTpHXO,llHTCH 3 aMHHOKMCJIOTHhIX OCTaTKa, a He 3,6, KaK 3TO xapaKTepHO MH BTOpM'IHotiCTPYKTYPhI rno6yJIHpHhIX 6eJIKOB. Crr11paJih rrenTHJlHOH: uen11 KoJIJiaretta crn6MJIH3MposaHa He 3a C'leTBOllOPO,llHhIX CBH3eti (TaK KaK

npOJIHH MX He 06pa3yeT), a CMJiaMM CTep11qecKOfO OTTaJIKMBaHHH IIMPPOJIHJlHHOBhIX KOJieU B OCTaTKax npoJI11Ha. B pe3yJihTaTe paccTOHHHe Me)((JJ,y aMHHOKHCJIOTHhIMM OCTaTKaMH no OCH CIIHPaJIH yseJIH'lMBaeTCH,11 OHa OKa3bIBaeTCH 6oJiee pa3BepHYTOH no cpaBHeHHIO c TYTO 3aKpy'leHHOH:cx-crr11- panh10 rno6yJIHpHblX 6eJIKOB.

Crr11pan113osaHHhie rroJI11rremH,llHhie uerr11, rrepeB11BaHch .upyr OKOJIO .upyra, o6pa3YIOT TpexuerroqeqHYIO rrpas03aKpy'leHHYIO cyrrepcrr11paJihHYIO MOJieK}'ny, 'laCTO Ha3bIBaeMYIO TPOIIOKOJIJiareHOM (p11c. 1-41). Uen11 y.uep)((HBaIOTCH .upyr OKOJIO .upyra 3a cqfa BO.UOPOllHhIX CBH3eti, B03HHKalOIUHX Me)((- llY aMHHO- H Kap60KCHJihHhlMM rpyrrrraMM rreIITHJlHOfO OCTOBa Pa3HhlX IIOJIHIIenTHJlHhIX ueneH:, BXO-

.UHIUMX B COCTaB Tpexcn11paJihHOH MOJieKyJihl. «)l(ecTKHe» aMMHOKHCJIOThl - npOJIHH H fHllPOKCHIIPOJIHH - orpaHH'lHBalOTspaIUeHHe IIOJIHIIenTHJlHOfO CTep)l(HH H yseJIH'lHBalOTTeM caMbIM CTa6HJibHOCTh TpOHHOH CIIHpaJIH. fJIHUHH, HMelOIUHH BMeCTO PMMKaJia aTOM BO,llopo.ua, scer.ua HaxO.LlMTCH s MecTe rrepece'!eHHHuerretl:; OTCYTCTBHe pa.u11KaJia II03BOJIHeT uerrHM IlJIOTHO rrpllJieraTb .upyr K

.upyry.

B pe3yJihTaTe TaKoro cKpyq11saHHH nerrTHJlHhIX OCTOBOB IIOJIHIIeIITH,llHhlX uerre:H H HaJI:HqHH YMHHeHHOH CTp}'KTYPhl,ll,Ba .upyrHX pa,UHKaJia 113 TpMallhl aMHHOKMCJIOT fJI11-X-Y OKa3bIBalOTCH Ha Ha- p~ott IIOBepXHOCTH MOJieKYJihl TpOIIOKOJIJiareHa. HeKOTOphre KOMilJleMeHTapHhie yqacTKH MoJieKYJI TpOIIOKOJIJiareHa MOryT o6'hellHHHThCH.upyr c .upyfOM, cpopMHPYH KOJIJiareHOBbie cpH6pllJIJihl, rrpH'leM 3TH yqaCTKll paCIIOJIO)((eHbl TaKMM o6pa30M, 'ITO O,ll,Ha HMTh TpOIIOKOJIJiareHa CllBHttyTa no OTHOUieHHIO K JJ,pyrott rrp11MepHo Ha 1/4 (p11c. 1-42). Me)((- llY pa,UMKaJiaMH aMHHOKMCJIOT B03HMKalOT MOHHhie, BO.UOPO.UHbie H fHJlpOcpo6Hhle CBH3M.

a-1.1enb Ko1mareHa

PMC. 1-41. CTpOeHMe MOneK'fnblTpOnOKOnnareHa (ct>parMeHT).

57

15).

 

06pa3oBaHLlle nonepeYHblX

Kom1areH

CBSl3eill M9>KAY MOJleKynaMLll

KonnareHa

!

PMc. 1-42. CTpoeHMe KonnareHoaoi.t <t>M6pMnnb1 (cpparMeHT).

BaJKHYIO poJib B <t>opMHpoBamn:1 KOJIJiareHOBhIX <l>H6pHJIJI HrpaIOT MO,lll'l<l>m.1,HpOBaHHbieaMl1HOKHCJIOTbI: rH,llpOKCHnpoJmH 11 raupOKCHJil1311H. fauPOKCHJibHbie rpynrrhI raup0Kc11npomrna coce,UHl1X uenell. TponOKOJIJiareHa o6pa3yIOT BO,llOpO,llHbie CB51311, yKpenJI5110m11e CTPYKTYPY KOJIJiareHOBbIX <l>H6pH:JIJI. Pa,U11KaJib1 JIH3HHa H rn,llpOKCHJIH3HHa Heo6xo,UHMbI ,llJI5I o6pa3oBaHH51 npo'IHbIXnonepe'l- HhIX CIIIHBOK Me)J()zy MOJieKYJiaMl1 rponOKOJIJiare-

Ha, eme CHJibHee YKPeIIJI51IOII.J,He CTPYKTYPY KOJIJiareHOBbIX <l>H6pH:JIJI. KpoMe Toro, K raupoKCHJihHOll. rpynne rH,llpOKCRJIH3HHa MOryT np11coe,llHH51TbC51 yrneBO,llHbie OCTaTKM (rJIHK03HJIHpOBaHHe KOJIJiareHa), <l>YHKI.J,1151 KOTOpblX noKa He51CHa.

TaKHM o6paJoM, aM11HOKMCJIOTHa5l nocJie,UoBaTeJibHOCTh nOJIHneIITH,Il,HblX uenell. KOJIJiareHa no3BOJI51eT c<t>opM11poBaTb YH11KaJibHYIO no CBOl1M MexaHH:'lecKHMcBolicTBaM CTPYKTYPY, o6Jia,UaIOII.J,yIO orpoMHOM npo'IHOCTbIO.l13MeHeHHe B nepBH'IHOH crpyKrype KOJIJiareHa MO)K.eT npl1BO,llHTb K Pa3Bl1THIO HacJie,llCTBeHHblX 6oJie3Hei1 (CM. pa3,lleJI

2. Cmpoeuue u tjJyHKU.UR 3./lacmuua

B OTJIH'IHeOT KOJIJiareHa, o6pa3YIOII.J,ero npo'l- Hh1e <l>H6pH:JIJibI, cnoco6Hbie Bbl,llep)K.aTh 6oJibIIIHe Harpy3KM, 3JiaCTHH (TaK)K.e 6eJIOK Me)K.KJieTO'IHOro MaTpHKca) o6Jia,UaeT pe3HHono,Uo6HbIM11 CBOMCTBaMH. H11TH 3JiaCTHHa, CO,llep)K.aII.J,HeCH B TKaHHX JierKHX, B CTeHKaX cocy,UOB, B 3JiaCTl1'1HbIXCB513Kax, MOryT 6b1Th paCT51HyTbl B HeCKOJibKO pa3 no cpaBHeHl1IO c HX 06bl'IHOM,llJIMHOM, HO nocJie CH51TH51 Harpy3KM OHH B03BpaII.J,aIOTC51 K CBepHyTOH KOH- <t>opMaUMM.

3JiaCTl1H CO,llep)K.HT B COCTaBe OKOJIO 800 aMMHOKMCJIOTHblX OCTaTKOB, cpe,llll KOTOpblX npeo6Jia,UaIOT aMMHOKHCJIOTbl c HenoAApHblMl1 pa,llMKaJiaMM, TaKMe KaK fJil11.J,11H, BaJIMH, aJiaHMH. 3JiaCTl1H co- ,llep)K.MT ,llOBOJibHO MHOfO npoJIMHa M JIM3MHa, HO

JIHIIIb HeMHOro rn,llpOKCMnpoJIMHa; ITOJIHOCTbIO OTCyTCTByeT raupoKCMJIH3MH.

Hantt'l11e6oJihIIIoro KOJIH'lecrnaraupo<t>o6HbIX pa,Ul1KaJIOB nperr51TCTByeT C03,llaHl1IO CTa6RJibHOM rno6yJihI, B pe3yJihTaTe noJittnerrTH,llHbie uentt 3JiacTttHa He <t>opMttpyIOT peryJI51pHhie BTOPM'IHYIO11 rpeTtt'IHYIOCTPYKTYPhI, a npHHttMaIOT B Me)K.KJleTO'IHOMMarpMKCe pa3Hble KOH<l>opMaI.J,MM c np11MepHO paBHOH CB060,llHOM 3Hepmei1 (pttc. 1-43).

3TO KaK pa3 TOT CJiyqall. CTpOeHl151 nepBM'IHOM cTpyKTYPhI, KOr,lla OTCyTCTBHe O,llHOM cTa6HJihHOM ynop51,llO'leHHOMKOH<l>OpMaI.J,1111 npttBO,llMT K B03HHKHOBeHMIO He06XO,llMMblX 6eJIKY CBOHCTB.

EoJiee no,Upo6tto oco6ettHocrn cTpoemrn H <l>YHKUMOHttpoBaHAA 3JiaCTMHa paccMorpeHbl B pa3,lleJie 15.

B. KJJAccH«1>HKAU,1u1 EEJIKOB

no XHMHqECKOMY CTPOEHHIO

1. Ilpocmbte 6e.nKu

HeKOTOpbie 6eJIKM CO,llep)K:aT B CBOeM COCTaBe TOJibKO IIOJIMnerrTH,llHbie uentt, COCT051II.J,Me M3 aMMHOKMCJIOTHblX ocraTKOB. Mx Ha3bIBaIOT «DpoCTble

OMKH». ITp11MepoM rrpocTbIX 6eJIKOB MOryT cJiy- )K.HTb OCHOBHble 6eJIKl1 xpoMaTMHa - fMCTOHhl; B HX COCTaBe CO,llep)K:MTC51 MHOfO aMl1HOKMCJIOTHblX OCTaTKOB JIH3MHa H aprHHMHa, pa,UMKaJibI KOTOPbIX MMeIOT IIOJIO)l(JfTeJibHbIM 3ap51,ll (6oJiee no,llpo6HO

PMC. 1-43. Cny'l&MHbleKOH<l><>PM8l.IMM MOneKYnbl 3"8CTMH8.

58

mcTOHhI ormcatthI B pa3,a:ene 4). PaccMoTpetttthrtt Bblllle 6eJIOK Me)l(KJieTO'lHOro MaTpMKCa 3JiaCTMH TaK){(e OTHOCHT K npoCTblM 6eJIKaM.

2. Ctlo:J1Cnb1e 6e.lllcu

O.uttaKo oqetth MHorMe 6enKM, KpoMe non11nen-

TMHhIX ueneli, co.uep)l(aT B CBOeM COCTaBe He6eJIKOBYJO 'laCTh,npMcoe,0:11HeHHYJO K 6emcy CJia6hIMM HJ111 KOBa.rrettTHhIMM CBH3SIMM. He6eJIKOBaH qacTh MO)KeT 6bITb npe,a:CTaBJieHa 110HaMM MeTaJIJIOB, Ka- KMMM-JIM6o opraHM'leCKMMM MOJieicyJiaMM c HH3KOH HJIM BhICOKOH MOJieKYAAPHOtt Maccort. TaKtte 6eJIKl1 Ha3hIBaIOT «CJI01Kllble 6eJIKH». ITpo'IHOCBH-

3aHHaSI c 6eJIKOM He6eJIKOBaH 'laCTbHOCMT Ha3BaHHe npocTeTl1'1eCKOHrpyTIIIbl.

ITpocTeTH'leCKaSIrpynna MO)KeT 6bITh npe.ucrnBJietta BemecrnaMH pa3Hoi1: np11:po.uhl. HanpHMep, 6eJIKl1, coeD,HHeHHhie c reMOM, HOCSIT Ha3BaH11e reMorrpoTeHHhI. B cocrnB reMonpoTe11ttoB, KpoMe

~e paCCMOTPeHHhIX BbIIIIe 6eJIKOB reMOfJI0611HOB

H MHOfJI06HHa, BXO.UHT Q:>epMeHTbl - UHTOXpOMhl,

KaTa.rra3a H nepoKcMa3a. feM, np11coea11HeHHhri1: K pa3HhIM 6eJIKOBbIM CTPYKTypaM, BbrTIOJIHHeT B Hl1X xapaKTepHbie JlJIH Ka){()loro H3 6eJIKOB cPYHKUHH (HanpHMep, B COCTaBe reMomo611Ha nepeHOCITT 02, a B COCTaBe UMTOXpOMOB - 3JieKTpOHbI).

EenK11, coea11:HeHHhie c ocrnTKOM Q:>oc¢opHoi1: Kl1CJIOTbI, Ha3hIBaIOT ¢ocQ:>orrpoTeHHaMH. <Doc¢opHhle OCTaTKH npHCOe)lHHHIOTCH CJIO)KH03cPHPHOH CBH3bl0 K mapoKCHJihHblM rpynnaM cepHHa, TpeOHl1Ha 11JIH THp0311Ha npH yqaCTl111 ¢epMeHTOB, Ha3bIBaeMbIX npoTe11HKl1Ha3aMH.

B cocrnB 6eJIKOB qacTo BXOJlSIT yrneBOJlHbie ocTaTKM, np11JJ:aIOIUHe 6eJIKaM JlOTIOJIHHTeJihHYJO cneU11cPl1'1HOCTbH qacTO YMeHh1IIa10m11:e CKOPOCTb HX ¢epMeHTaTl1BHoro np0Teon113a. TaK11e 6eJIK11 HOCHT tta3Batt11e rnHKorrpoTeHHOB. MHorHe 6eJIKH KpOBH, a TaK){(e peuerrTopHbie 6eJIKl1 KJieTO'lHOH nonepXHOCTH OTHOCHT K fJIHKOnpoTel1HaM.

EeJIKH, Q:>yHKUMOHHpy10m11:e B KOMIIJieKce c JIHIIH.uaMl1, Ha3bIBaIOT JIHITOrrpoTel1HaMH, a B KOMTIJieKce c MeTaJIJiaMH - MeTaJIJIOTIPOTeHHaMH.

CJIO)J(Hb1i1: 6enoK, cocTOSillltti1: 113 6eJIKOBoi1: qaCTH (anonpOTeHH) H He6eJIKOBOM qacTH (npocTeTHqecKaH rpynna), Ha3hIBaIOT «XOJIOnpOTeHH».

r. KJIACCH<l>HKAIJ,IDI I>EJIKOB no <l>YHKIJ,IDIM

neJIKH BblTIOJIHHIOT B KJieTKax MHO)l(eCTBO 6HOJIOfl1'IeCKl1X Q:>yttKU11M. flo IlpH3Haicy CXO,IlCTBa Bbl-

TIOJIHSieMhIX 6eJIKaMl1 <l>YHKU11H HX MO)l(HO Pa3JleJil1Tb Ha c11e.uyiom11e 6onbIII11e rpyrrrrhI.

1. <PepMeHmbl

<DepMeHThI - cneU11aJIH3HpoBaHHh1e 6eJIKH, ycKopHIOlllHe Te'leHHeXHMl1'1eCK11XpeaKUHli. EnaroaapH Q:>epMeHTaM B KJieTKe CKOpOCTH XHM11'leCK11X peaKUHH no3pacrn10T B MHJIJIHOHhI pa3. TaK KaK Q:>epMeHThl, KaK 11 JII06bie 6eJIK11, 11MeIOT aKTHBHblll ueHTp, OHl1 cneuHcPH'leCKHCBH3bIBaIOT Oilpe)leJieHHbIM JIHraHD, (11JIH rpynny TIOXO)[(HX JIHfaHD,OB) H KaTaJIH3HpyIOT onpe.ueneHHbIM THTI Xl1MH'leCKOf0 npeBpameH11SI JlaHHOH MOJieKYJibl. B HaCTOHlllee BpeMH H3BeCTHO oKoJio 2000 pa.3.JllillHhIX Q:>epMemoB, ycKopS110m11x pa3Jil1'1Hhie x11MM'lecK11e peaK111111:. Hanp11Mep, npOTeOJI11T111IeCKHH ¢epMeHT Tp11nc11H pa3pyIIIaeT B 6eJIKax neTITH,IlHhie CBH311, o6pa3oBaHHhre Kap60KCl1JlhHOH rpynnon OCHOBHhIX aMl1HOKHCJIOT - apmHHHa 11Jil1 Jil13MHa. <DepMeHT p1160HyKJiea3a pacmeTIJIHer <t>oc<Po3Q:>11pttyio CBH3h MeJK.U.Y ttyKJieOT11JJ:aM11 B IlOJI11HYKJieOT11JJ:HOH 11en11.

EnaroaapH Ha6opy Q:>epMeHTOB B KJieTKax npeBpameHHH TIOCTynaIOIUHX B HHX semecTB npoTeKaIOT He xaOTH'lHO,a B CTpOfO onpe)leJieHHbIX HanpaBJieHHHX.

2. Pezy.llHmopnble iJeAKu

K peryJIHTOpHbIM 6emraM OTHOCHT OOJibllIYJO rpyn- ny 6eJIKOBblX ropMOHOB, yqaCTByIOllll1X B TIOD,Jlep)l(a- Hl111 IlOCTOHHCTBa BHYTpeHHe.ti: cpeD,hl opraHH3Ma, KOTOph1e B03,a:e.tiCTByIOT Ha cne1111Q:>mecKHe KJieTKMMmIIeH11. Hanp11:Mep, ropMOH MHcymrn BhlJleJIHeTCH B KpOBb np11 IlOBbillleHHH KOHlleHTpaUMH fJIIOK03bl B KPOBH nocne e)lbl M, CTHMYJIHPYSI HCTIOJib30BaHHe rnIOK03hI KJieTKaMM, CHl1)[(aer KOHlleHTpaUHIQ rmoK03hI .uo HOpMbl, T.e. BOCCTaHaBJIMBaeT roMeOCTa3.

KpoMe TOfO, K peryJIHTOPHbIM OTHOCHT 6emrn, npMcoe)lHHeHHe KOTOPbIX K JlPYfHM 6enKaM 11J111 HHhIM CTPYKTYPaM KJienrn peryJIHpyeT HX cPYHKumo. HanpMMep, 6enoK KaJihMOJlYJil1H B KOMTIJieKce c 'leThIPhMH110HaM11 CaH MO)l(er np11coe,a:ttHSITbCSI K HeKOTOPbIM ¢epMeHTaM, MeHHH l1X aKTHBHOCTb.

PeryJIHTopHbie D,HK-cmI3hIBaIOllll1e 6eJIKM, npMcoe.uttHHHCh B onpe)leJieHHhie MOMeHTbl K cne11M- $H'IHblMyqaCTKaM D,HK, MOryT peryJIHpOBaTh CKOPOCTb C'lttThIBaHHH reHeTH'lecKoit11HQ:>opMa1IHH (OHH OTIHCaHbl B Pa3.UeJie 4).

3. Peu,enmopnbte OeAKu

CttrHaJihHhie MOJieicyJihI (ropMOHbI, He.ti:poMe)lM-

aTOpb1) .ueiicTByIOT Ha BHYfPHKJieTO'lHhienpouec-

59

Chi qepe3 B3aHMo.ue:tkTBMe co cneum}:mqecKMMM 6enKaMH-pe11e1nopaMH. TaK, ropMOHhI, IIHPKYJIHpy101utte B KpOBH, HaXO,!IJIT KJieTKH-MmlleHH H B03- L(eMCTBYJOT Ha HHX, cne11HqmqHo CBH3hIBa.SICb c 6eJIKaMH-peuenTOpaMH, 06h1qHo BCTpOeHHhIMH B KJieTOqHYJO MeM6paHy. .[(JIH rHLJ:pO<p06HhIX peryJIHTOpHhIX MOJieKYJI, npOXOMIUHX qepe3 KJieToq-

HYJO MeM6patty, peuenTOphl JIOKaJIH3YJOTCH B UHTOTIJia3Me KJieTOK.

4. TpaucnopmHbte 6eAKu

MHome 6eJIKH KPOBH yqacrnyroT B nepeHoce cne11ttqmqecKMx JIHraHLJ:OB H3 O.llHOro opraHa K LJ:pyroMy. qacTO B KOMTIJieKce c 6eJIKaMH nepeHOCHTC.SI MOJieKYJihI, TIJioxo pacrnopHMhie B BOLJ:e. TaK, 6enoK TIJia3MhI KPOBH aJih6YMHH rrepeHOCHT )l{lfpHhie KMCJIOTbl H 6ttJittpy6HH (npOLJ:YKT pacrraL(a reMa), a reMOfJI06HH 3PMTPOUMTOB yqacrnyeT B rrepeHoce 0 2 oT nerKHX K TKaHHM. CTepoHLJ:Hhle ropMOHhl rrepeHOCHTc.si: B KPOBH crreumpttqecKMMH TpaHcnopTHhIMH 6eJIKaMH.

TpattcrropTHhie 6eJIKM yqacrnyroT TaIOKe B rrepeHoce fH.llPOQlllJlhHhIX Bemecrn qepe3 rHLJ:po<t>o6-

Hhie MeM6paHhI. TaK KaK TpaHcnopTHhie 6enKH o6JiaL(aJOT CBOMCTBOM crreump~HOCTH B3aHMOLJ:ei1-

CTBllil c JIMraHL(aMH, HX Ha6op B KJieTOqHoM MeM6paHe onpeLJ:eJIHeT, KaKMe rHLJ:poq11UihHh1e MOJieKYJihI MOryT rrpOMTM B LJ:aHHyro KJieTKY. c IlOMOIUhJO 6eJIKOB-rrepeHOC'lHKOBB KJieTKY rrpOHMKaJOT rmoK03a, aMRHOKMCJIOTbl, HOHbl M LJ:pyrtte MOJieKYJibl.

5. CmpyKmypuble OeAKU

HeK0Toph1e 6eJIKH, pacrrono:JKeHHhie orrpeLJ:e- neHHhlM o6pa30M B TKaHHX, IlpHLJ:aJOT HM QlOpMy, C03LJ:aJOT orropy, orrpeLJ:eJIHIOT MexaH~ecKMe CBOMcrna LJ:aHHOM TKaHH. HarrpttMep, KaK Y:JKe roBoPllJIOCh BbIIlle, maBHbIM KOMITOHeHTOM XPHIUeM H cyxo:JKHJIHM HBJI.SleTCH qm6pllJIJIHpHbIU 6eJIOK KOJIJiareH, HMeIOIUHH BblCOKYIO rrpoqHOCTb. )l,pyroi1 CTPYKTYPHbIM 6eJIOK (3JiaCTHH) 6naroL(apH CBOeMY yHttKaJihHOMY cTpoeHttIO o6ecneqttBaeT orrpeLJ:eneHHhIM TKaHHM CBOMCTBO paCTHfHBaThCH BO BCex HarrpaBJieHHHX (COCY.llhl, JierKtte).

6. 3aw,umHbte oe.aKu

HeKOTOPbie 6enKH, B qacTHOCTH HMMYHOrJio6yJIHHhr, o6JIMaIOT cnoco6HOCThIO Y3HaBaTb H CBH3blBaTb qy.JKepoLJ:Hhre MoneKYJihl, BHPYCHhie qacTttIJhI H 6aKTepttH, B pe3yJihTaTe qero npoHCXOLJ:MT HX HeHTpaJitt3aUll.SI. KpoMe Toro, KOMTIJieKc qY:JKepoLJ:-

Hou qacTHUhI c HMMyttomo6yJIHHOM JierKo y3HafacH H yttttqTO:JKaeTCH KJieTKaMH HMMyttHOH CHCTeMhl.

3aIUMTHbIMH CBOUCTBaMH o6naL(aIOT 6eJIKH CBepTh1Ba10meu CHCTeMhl KPOBH, HarrpMMep qm6pHHOreH, TpOM6HH. 0HH yqaCTBYJOT B <t>opMHpoBaHHH TPOM6a, KOTOphIH 3aKYITOPHBaeT ITOBpe:lK,lleHHblM COCY.ll M rrpenHTCTByeT TIOTepe KpOBH.

7. CoKpamume.abHbte oe.aKu

HeKOTOphie 6enKH rrptt BhIITOJIHeHHH CBOHX <l>YH-

KUHH HaLJ:eJIHJOT KJieTKY cnoco6HocTbIO ;m6o co-

KpamaThCH, JIH6o nepeLJ:BHraThCH.

K TaKHM 6en-

KaM OTHOCHT aKTHH M MM03MH -

qm6pttJIJIHpHbie

6eJIKM, yqacTBYJOIUHe B COKpameHMH CKeJieTHhIX MbllllU. )l.pyroll. npMMep TaKllX 6eJIKOB - ry6yJIMH, H3 KOTOporo IlOCTpOeHhl KJieToqHhie opraHeJIJihl - MMKpoTpy6oqKM. MttKpoTpy6oqKM B nepMO.ll LJ:e- neHll.SI KJieTKM perynttpyroT pacxo:lK,lleHtte xpoMa-

THLJ:. MHKPOTpy6oqKM - Ba:JKHhie 3JieMeHThI pec-

H~eK M :JKIYTMKOB, c TIOMOIUbIO KOTOpbIX KJieTKH nepe.UBMraIOTCH.

0LJ:HaKO CYJUeCTByeT 60JiblllOe KOJTIIlieCTBO 6eJIKOB, MMeIOIUHX YHMKaJibHbie QlYHKUMH, KOTOpbie He BOIIIJIH B 3TY .llOBOJihHO rrpocryIO KJiaccmpHKaUMIO.

,II,. CEMEHCTBA PO,IJ;CTBEHHhIX BEJIKOB

B XOLJ:e 3BOJIIOUMM B rrpe.uenax o.uHoro 6ttonomqecKoro BHLJ:a 3aMeHhl aMMHOKHCJIOTHhIX OCTaTKOB MOryT npttBO.llMTb K B03HMKHOBeHMIO pa3HblX 6eJIKOB, BbIIlOJIHHIOIUMX PO.llCTBeHHbie cPYHKUMM H MMeIOIUMX fOMOJIOfM'lHhie IlOCJie.llOBaTeJihHOCTM aMMHOKllCJIOT. f OMOJIOfMqHblMM Ha3hIBaIOT IIOCJie-

.llOBaTeJibHOCTM, MMeIOIUMe MHOfO CXOLJ:HblX qepT. OHM co.uep:JKaT BO MHOfHX ITOJIO:JKeHMHX O)J.HM M Te

.JKe aMMHOKllCJIOTbl, Ha3bIBaeMbie MHBapttaHTHhlMM, a B HeKOTOpblX IIOJIO:JKeHll.SIX MOryT HaXO)lMTbCH pa3Hbie, HO 6JIM3Klle no cPM3MKO-XllMMqecKHM CBOUCTBaM aMMHOKllCJIOTHbie OCTaTKff.

3rn 6eJIKM MMeIOT nopa3MTeJihHO CXO:JKHe KOH-

<t>opMaIJMM: KOJIM'IeCTBO M B3aMMOpacrroJIO:JKeHMe cx-cnttpaneu H/llJIH ~-CTPYKTYP, 60JibillHHCTBO 110BOPOTOB M M3fM60B ITOJIMITeIITM.llHblX uerrei1: cxo.u- HO MJIM HLJ:eHTM'lHO. TaKMe 6eJIKH, MMeIOIUMe roMonorttqHhie y11acTKM IlOJIMITeITTM)J.HOM uerrM, CXO.llHYJO KOH<t>opMaIJMIO M POLJ:CTBeHHhie cPYHKIJMM, BbI)J.eJIHIOT B ceMei1:crna 6eJIKOB.

IIpttMep ceMell.crna po.ucrneHHhlX 6enKoB - ceMei1:cTBO MMOfJI06MHa, Ky.Ila BKJIIOlfeHbl, KPOMe CaMOfO MHOfJI06MHa, M BCe BH.llbl reMorno6MHa.

60

major histocompatibility

1. CeMeucmBo cepu1106b1X npome03

K ceMeH:cTBY PO.IlCTBeHHhIX 6eJIKOB OTHOCHT ce-

pMHOBhie npoTea3bl. 3TO ceMeHCTBO <PepMeHTOB.; KOTOpbie MCIIOJib3YIOT yttMKaJibHO aKTMBMpoBaHHbIH OCTaTOK cepttHa, paCIIOJIO)KeHHblH B aKTMBHOM ueHTpe, L(JIH CBH3hIBaHHH M KaTaJIHTH<IeCKOfO rHL(- pOJIM3a rreIITHL(HbIX CBH3eH: B 6eJIKOBblX cy6cTpaTax. MmneHH ,LUIH cepMHOBhrx 11p0Tea3 - crreuMqmqecKHe neIITHL(Hbie CBH3H B 6eJIKax (<IaCTO B

.IlPYrHX cepHHOBblX rrpoTea3aX).

,1l,JuI Bcex 6eJIKOB 3TOfO ceMeHCTBa xapaKTepHO HaJimtte B aKTMBHOM ueJITpe ocTaTKOB Cep195 , fwc57 ,

Acrr

Im

(3TY ttyMepaumo MCIIOJih3YIOT He3aBHCHMO

 

u

OT HX TOqHoro paCIIOJIO)KeHHH B rrepBHqHoM CTpYKzype orrpe.ueJJeHHhIX cepttHOBhIX rrpoTea3). BbrnBJieHa TaJOKe BbICOKaH CXO)KeCTb HX npocTpaHCTBeHHbIX CTpYKTYP, HeCMOTpH Ha TO, qTo TOJibKO B 40%

IIOJio:>KeHHH: OHM COL(ep)KaT HL(eHTttqHhie aMHHOKHCJIOTbI (pMc. 1-44). KaTaJIHTmecKHH: yqacToK cepwHOBhIX npoTea3 paCIIOJIO)KeH B pacmeJIMHe Me)KL(y

.IlBYMH .IlOMeHaMH.

HeK0Topb1e aMHHOKMCJIOTHbie 3aMeHhr rrpwBeJIM K H3MeHeHHIO cy6cTpaTHOH cneumtmqHOCTM 3TMX 6eJIKOB H K B03HHKHOBeHHIO cPYHKUMOHaJibHOro MHOroo6pa3MH BHYTPM :noro ceMeHCTBa. TaK, IIMllleBapMTeJibHbie cepMHOBbie npoTea3bI yqaCTBYIOT B rrepeBapMBaHHH (fM.IlPOJIMTMqecKOM pacmerrJJeHMM rrerrTM.IlHhlX cBH3eH:) .IleHaTypMpoBaHHhIX IIHllleBblX 6eJIKOB. K HMM OTHOCHT TPHIICHH, XHMOTPHIICHH, 3JiaCTa3y, HO Ka)K,IlblH H3 3THX <PepMeHTOB rrpe.IlIIOqMTaeT pa3pbIBaTh nerrTM.IlHble CBH3H, o6pa30BaHHbie orrpeL(eJieHHbIMH aMHHOKMCJIOTaMH.

A

6

PMC. 1-44. npocTpaHCTB&HHbl& CTpyKTypw anaCT83bl (A) M XMMOTpMnCMHa (6).

Eme 6oJihllle:H cy6cTpaTHoi1 crreutt<l:>MqttocThIO

o6nMaIOT cepMHOBbie rrpoTea3bI, yqacrny101uwe B

TlllaTeJibHO KOHTPOJIHpyeMhIX cPH3HOJIOfH<IeCKHX

rrpoueccax, TaKHX KaK aKTMBaUMH KaCKaL(a 6eJIKOB CBepTbIBaHHH KpOBM, cPM6pHHOJIM3a, aKTHBaUHH 6eJIKOB CHCTeMbJ KOMIIJieMeHTa, o6pa30BaHHH 6eJIKOBbIX ropMoHoB. B npouecce aKTHBauww HanrnHhIX 6eJIKOB cepMHOBhJe rrpoTea3hl rHL(pOJIM3yIOT O.IlHY mm .IlBe oco6eHHhie rrerrTHL(Hhre CBH3H M3 COTeH CBH3eH:, HMeIOlllHXCH B 6eJIKOBOM cy6cTpaTe. 3TO CBH3aHO c TeM, qTo B HaTHBHOM 6eJIKe <PepMeHT y3HaeT He TOJlbKO aMHHOKHCJIOTbI, HenocpeL(CTBeHHO <l:>opMMPYIOlllMe rreIITHL(Hyio CBH3b, HO H HeKOTOpbie aMHHOKHCJIOTHbie OCTaTKH, OK- p~aIOlllMe CBH3b, IIOL(Bepra10ruy10cH <PepMeHTaTHBHOMY fHL(pOJII13y.

BoJJee IIO.Ilpo6Ho o cepttHOBhIX npoTea3ax MO)K- HO rrpoqecTb B pa3.IleJiax 9, 14.

2. CynepceMeucmBo UMMy11ouo6y.11u1106

B pa6oTe HMMyttHO:H cMcTeMbI orpoMHyio poJih ttrpaIOT 6eJIKH, OTHOCHlllHeCH K cyrrepceMe:HcTBY HMMyttOrno6yJIHHOB. 3TO cyrrepceMeH:cTBo BKJIIOqaeT 110 KpaH:He:H Mepe TPH 60JiblllMX ceMe:Hcrna 6eJIKOB, yqaCTBYIOIUMX B HMMyttHOH 3alUMTe opraHM3Ma: ceMeHCTBO HMMyttOrno6yJIHHOB, ceMeHCTBO T-KJieTO<IHhIX aHTttreHpacrr03HaI01UMX peuerrrnpoB H 6eJIKH rnaBHOfO KOMIIJieKca fMCTOCOBMeCTMMOCTH I H II KJiaccoB, KOTOPhie B JIHTepazype o6o3Ha<IaIOT MHC (oT aHrn.

complex). B 3TO cyrrepceMe:Hcrno BKJIIO<IeHo TaIOKe ceMeHCTBO aL(re3HBHblX 6eJIKOB, yqaCTBYIOIUHX B y3HaBaHHM orrpe):(eJieHHbIX THilOB KJieTOK H HX Me)K- KJieTOllHbIX B3aMMo.neH:cTBI1HX.

OcHOBHoH: KpHTepHif BK.JIIOqeHHH 6eJIKOB B cyrrepceMe:Hcrno HMMyttOrno6ymrnoB - HX .IlOMeHHaH opraHH3fillHH, .IlOCTOBepHaH fOMOJIOfHH aMHHOKHCJIOTHblX IlOCJieL(OBaTeJibHOCTeH: H rrpocTpaHCTBeHHbIX CTpYKTYP OT.IleJibHhIX .IlOMeHOB. KpoMe rnro, 6eJIKH 3T0f0 cyrrepceMeHCTBa MMeIOT CXO)KHe cPYHKUHH: HMMyttOfJI06yJIHHbI B3filfMOL(eHCTBYJOT c 1.JY)KepO.LI.HblMH CTpYKTYPaMH, HaxO):(HlllHMHCH B KPOBM, JIHM¢e, Me)KKJieTOllHOH )KHL(KOCTH HJIH ceKpeTax )KeJifo, a peuemophI T-JIHMcPOIIHTOB H 6emm rnaBHoro KOMTIJieKca IBCTOCOBMeCTHMOCTH - c aHTHrtmaMH, HaXO.IlHlIIHMMCH Ha IIOBepXHOCTH KJieTOK .IlaHHOro opraHH3Ma.

3. CeMeucmBo UMMy110Z.1106y.11u1106

11MMyttorno6yJIHHhI, HJIH aHTHTena, - crre1111<PHqecKHe 6emm, Bh1pa6aTb1BaeMble B-JIHMcPoUHTaMM

61

20-30

B orner Ha rrorra,n,amre B opraHl13M tzy)Kepo,n:HhIX erpyicryp, HaJhIBaeMhIX attn1reHaM11. B opraH113Me qeJIOBeKa Bh1pa6aThmaercH OKOJIO I07KJIOHOB B-JIH:M- qxn.1,ITTOB, KIDK,ll,hIH 113 KOTOphIX erreI.J,11aJI113lfPOBaH Ha Bhipa6orKe O,UHOfO U3 107 BU,UOB 11MMyHorno6yJIUHOB.

Bee 11MMyttorno6yJI11HhI xapaKrep113y10TeH o6lll.11M IIJiaHOM erpoeH11ff, KOTOphIM Mhl paeeMOTpuM Ha rrp11Mepe erpoeHM lgG.

MoJieKYJia lgG eoeTOl1T U3 qerh1pex IIOJI11nerrTU,UHhIX I.J,eneM:: ,ll,Byx H,llem11qHhIX JierKH:x (L - OT aHfJI. light), eo,n:ep)Kam11x OKOJIO 220 aMl1HOKH:e- JIOTHhIX oerarKOB, 11 ,ll,Byx Tff)KeJihIX (H - OT aHrn. heavy), eoeTOfflll.11X 113 440 aMl1HOKl1eJIOT KIDK,UM. Bee 4 I.1,err11 eoe,n:11HeHhI ,n:pyr e ,n:pyroM MHO)KeeTBOM HeKOBaJieHTHhIX " qeThiphMH ,n:11eyJihQ)l1,ll,- HhIMU eBH3ffMl1. Il03TOMY MOJieKYJIY JgG OTHOeHT K MOHOMepaM.

Jlenrne ueHH IgG eoeroHT 113 2 ,ll,OMeHOB: Bap11a6eJihHOfO (VL), H3XOM11J.erocH B N-KOHI.J,eBoii 06JiacT11 IIOJil1IIeIITl1,UHOH I.1,err11, 11 KOHeTaHTHOfO (CL),

paenoJio)KeHHOro Ha C-KOHI.J,e. KIDK.UhIH m ,n:oMeHOB eoerom 113 2 CJIOeB e ~-cKJia,n,qaTOH erpyxry-

poM:, r,n:e yqaCTKl1 IIOJil1IIeIITl1,ll,HOH I.1,en11 Jie)KaT aHrunapaJIJieJihHO. ~-CJIOl1 CBH3aHhI KOBaJieHTHO ,ll,11CYJihQJH,llHOH CBH3hl0 rrp11MepHO B cepe,n:11He ,ll,0- MeHa (p11c. 1-45).

TJDKCm.le uenu lgG 11Me10r 4 ,n:oMeHa: o,n:H:H sap11a6eJihHhIH (VH), HaxOJJ:H11I.11iicH Ha N-KOHI.J,e, 11 rp11 KoHeraHTHhIX (CHI'CH2, CH3). ,lloMeHhI TH)KeJihIX I.J,erreM: IgG 11MeIOT roMOJIOfH:qHOe crpoeHue c ,n:oMeHaM11 JierKH:X I.J,eneM:. MeX<.llY JJ:BYMH KOHeraHT-

HhIM11 ,n:oMeHaM11 rIDKeJihIX I.J,erreM: CH, 11 CH2 ecrh yqaeroK, co.n:ep)KamH:H OOJihllIOe KOJIH:qecrno oeTarKOB rrpoJil1Ha, KOTOPhie rrpeIUITeTBYIOT ¢opM11poBaHH:IO BTop11qHoH crpyicryphI 11 B3aHMO,ll,eHeTBl1IO coce,llHHX H-uerreM: Ha 3TOM orpe3Ke. 3ror yqaeroK HaJhIBaIOT «lliapHH:pHOM o6JiaCThIO»; OH npH,llaer MOJieKYJie rn6KOCTh.

Me)KJJ:y Bap11a6eJihHhIM11 ,n:oMettaM11 Tff)KeJihIX u

JierK11x uerreit Haxo,n:HTeH ,n:sa 11,n:eHT11qHhIX yqacTKa, CBH3h1Ba10m11x JJ:Ba o,n:11HaKOBhIX crreI.1,11¢11qecK11X aHr11reHa; II03TOMY TaKH:e aHTl1TeJia qaeTo Ha3hIBaIOT «6HeaJieHTbl». B eBH3hIBaH1111 aHr11reHa c aHT11TeJIOM yqaCTByeT He BeH aMl1HOKl1CJIOTHaff rroeJie,ll,OBaTeJihHOCTh Bap11a6eJihHhIX ,ll,OMeHOB o6e-

11X I.J,eneM:, a Beero Jil1llih aMl1HOKl1CJIOT,

paerroJio)KeHHhIX B r11nepnap11a6eJihHhIX o6naernx KIDK.UOH I.J,erru. HMeHHO 3Tl1 06JiaeT11 orrpe,n:e.rnnor yttl1KaJihHhie enoco6HOCTl1 KIDK,UOfO KJIOHa aHTl1-

r- AHrnreHCB513bIBat01.J.111e

\ . . yYaCTKl1

N

N

.. ----- ''WapHl1pHaSI o6nacTb"

PMc. 1-45. CTpoeHMe MMMyHorno6ynMHa G.

TeJI B3al1MO,ll,eHCTBOBaTh c COOTBeTCTBYIOlll.11M

(KOMIIJieMeHrapHhIM) aHrureHOM.

OcHOBHhie ¢YHKu1111 aHTureJI - 06Hap~ett11e

11 CBH3hIBaH11e q~epO,ll,HhlX aHTl1reHOB, HaXO,UH- I.UUXCH B opraHl13Me BHe ero KJieTOK (B KPOBl1, JI11MQJe, Me)!(KJieToqHOH )!(ff,ll,KOeTl1, B CJIU311CTblX ceKperax). 3To npo11cxo.uuT e IIOMOI.UhIO cneI.1,u- ¢11qecK11x aHTl1reHCBH3h1Ba10m11x yqaCTKOB pa3HhIX KJIOHOB 11MMyHorno6yJIHHOB. KpoMe Toro, 6Jiaro,n:apH CBH3hIBaHl1IO am11reHa c aHTl1TeJIOM 06JierqaercH rrpouece .UaJihHeM:lliero pa3pYllieH11H qy- )l(epo.11,HhIX Bemecrn. Cneu11¢11qHoeTh nyr11 pa3PYllleHM KOMIIJieKea aHT11reH-aHT11TeJio 3aB11c11T

OT KJiacea aHTl1TeJI.

KJiaCCbI HMMynorno6yJIHHOB. CymecrnyeT 5

KJiaCCOB Tff)KeJihIX ueneM: 11MMYHOfJI06yJrnHOB, OTJI11qa10m11xeH no eTpoeHl1IO KOHCTaHTHhIX ,ll,OMeHOB: a, 8, £, y 11 µ. B cooTBeTeTB1111 c Hl1MU pa3n11qa10T 5 KJiaceoB 11MMyttorno6yn11HoB: A, D, E,

G u M. Oeo6ettHocru eTpoeH11H TH)l(eJihIX uerreM: rrptt,D,aIOT 11x «lliapHttpHhIM yqacrKaM» 11 C-KOH- ueBhIM o6naeTHM xapaKreptty10 MH Kax,n:oro KJiaeca KOHQJOpMaI.J,uIO. CBH3hIBattue aHT11retta e attTttTeJioM 113MeHHer KOHQJOpMal.1,1110 KOHCTaHTHhlX ,D,oMeHoB Tff)KeJibIX ueneM:, qTo onpe,D,eJIHeT nyrh pa3pYllieHl1H KOMIIJieKca B opraHl13Me (eBH3bIBaHl1e e 6eJIKaM11 eucTeMhI KOMnJieMeHTa UJIU nornomeH11e KOMIIJieKea ¢arounTupy10mttMM KJierKaMu).

llMMYffOrJio6yJIHHbI M - nepBhIH KJiace am11reJI, c11HTe311PYI011I.UHeH B pa3n11sa10mnxeH B-JI11M- ¢ou11Tax. PaJJI11qa10T 2 ¢opMhI 11MMyttorno6yJI11HOB M: MOHOMepHaH, MeM6paHHO-eBH3aHHaH

62

1-47).
1-46).

ct>opMa 11 rremaMepHaH, ceKpernpyeMaH B-JJttMcl>o-

u11rnM11 B KPOBh.

MeM6pauuo-csuauuas cl>opMa HMMy1t0mo6yJiuuos M. Co3peBaIOm11e B-JIHMcPOUHThI cHHTe311pyIOT MOHOMepHhie 6ttBaJJeHTHhie MOJJeKYJJhI lgM, no CTPYKTYPe rroxo)l(Me Ha paccMaTp1rnaeMhie Bhrrne

IgG' KOTOphle BCTpattBaIOTCH B IlJia3MaT11qecKYIQ MeM6paHy KJJeToK 11 11rpaIOT ponh rrepBhIX attTH- rett-pacrroJttaIOm11x peu:errTopoB. ITpttKpermetttte lgM K MeM6paHe ocyurecrnm:1eTCH c IIOMOlll,hIO fl1,Il,- pocl>o6Horo yqacTKa, ttaxo.uHmerorn B C-KOHU:e- BOH ( «XBOCTOBOH») o6JJaCTH rn)KeJihIX u:erreM., co-

.uep)KameM. 25 rmi:pocl>o6HhlX aMHHOKHCJIOTHhlX OCTaTKOB.

B3a11Mo.ueM.crn11e aHTHreHa c peuenrnpoM Ha rroBepXHOCTH B-JIHMcPOUHTa BhI3hIBaeT ero pa3MHO- )l(em1e 11 o6pa3oBami:e uenoro KJJoHa JIHMcPOU:H- TOB, npOHCXOL(Hlll,11X 113 OL(HOH, CTHMYJJHPOBaHHOH aHTttreHoM KJJeTKH. 3ToT KJJOH B-JIHMcPOU:HTOB 6y.ueT Bhipa6aThIBaTh HMAfYHOrno6yJIHHhI c OL(HHaKOBhIMH attTttrettcBH3hrna10mttMH yqacTKaM11. O.u- ttaKo B-JIHMcPOU:HThI crroco6tthI rrepeKJJIOqaThCH Ha Bhlpa60TKY .upyr11x KJJaCCOB aHTHTeJI.

CelQ)e'Topuascl>opMa HMMYffomo6yJiuuos M. Kor-

.ua B-JIHMcPOU:HThI BnepBhie BCTpeqaIOTCH B )l(ML(- KOCTHX opraHH3Ma c HeH3BeCTHhIM paHee aHT11reHOM, OHH CHHTe311PYIOT H ceKpeTHPYIOT B KPOBh lgM, KOTOphre COL(ep)l(aT IIHTh MOHOMepHhIX cy6be- L(HHHU, CB513aHHhIX .upyr c L(pyroM )1,11CYJ1hcPl1L(HhIMH CB51351MH 11 )1,0IIOJIHHTeJJhHOH IIOJIHIIeIITH)J,HOH J-uerrhro (p11c.

B TH)l(eJihIX 11,ell:IX 11X MOHOMepoB OTCYTCTByeT rl1L(pocl>o6ttaH «XBOCTOBaH» qacTh. ITeHTaMepttaH MOJieKYJia co.uep)Kl.fT I0 yqacTKOB CBH3hrnamrn c aHTHreHoM, 'ITOo6nerqaeT Bepo51THOCTh rrpttKperrnettIDI HeH3BecTttoro pattee attTHreHa K 11MMYHOrno6yn11tty (p11c.

B3a.11Mo)J,etfcrn11e attT.ttretta c JgM H3MettHeT ero KOHcl>opMaU11IO 11 11HL(YUHpyeT CB513hIBaHHe ero «XBOCTOBOM» o6JiaCTl1 c rrepBhIM KOMIIOHeHTOM CHCTeMhl KOMilJieMeHTa. Ecn11 aHTHfeH pacrrono- )KeH Ha IIOBepXHOCTH MHKpoopraHl13Ma, aKTHBHpoBaH.tte C.HCTeMhl KOMilJieMeHTa Bhl3hIBaeT HapyIIIeHHe u:enOCTHOCTH KJieToqtto:i1: MeM6patthl H rn6eJih 6aKTepHaJJhHOH KJieTKH.

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63

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