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підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

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IllarrepoHbI, yqacrnyiow11e B 3aIUMTe KJieTOY.HhIX 6eJIKOB OT 1l,eHarypMpYJOIUMX B031l,eHCTBl1H, KaK Y%e fOBOPMJIOCh Bhlllle, OTHOC5IT K 6eJIKaM TeTIJIOBOfO WOKa (BTIII) 11 B JJHTepaType Y.aCTO o603HaY.aIOT

KaK HSP (oT attrn. heat shock protein).

IlpM D.eticrmm pa3JJWIHhIX CTpeCCOBblX cpaKTOpoB (BhlCOKa51 TeMnepaTypa, fl1IIOKCM51, HHcpeKIJ,M51, Y<l>O, M3MeHeH11e pH cpe1l.hI, M3MeHeHMe MOJJHpHOCTM cpe)J,bl, )J,eHCTBl1e TOKCl1'-1.HhIX XMMH'-1.eCKMX BewecTB, TIDKeJihIX MeTaJIJIOB 11 ·T.Jl..) B KJieTKax yc11JIMBaeTC5l c11HTe3 ETIII. l1MeH BhICoKoe cpo1l.CTBO K rM)J,pocpo6HbIM yqaCTKaM Y.aCTH'-1.HO1l.eHaryp11po-

MPHK

CMHT93111PYIOll.lllliliCA

f --- non111nenr111A

Wanep0Hb1-70

A

l

Wanep0Hb1-60

WanepoHOBblili KOMnneKc

.1.

~ ..,___ HaT111BHb11i1 6enoK 6

P1tc. 1-24. Y1.1acr1te wanepoHOB e cj>OnA1tHre 6enKoe. A-yYa- crne wanepoHOB-70 B npei:iorspalJ.leH111111 rni:ipo¢o6Hb1X B3a111Mo- i:ieiiicrs111iii Me>KAy yYacrKaM111 c111Hre3111py10lJ.lerocsi non111nenrni:ia; 6 - cpopM111poBaH111e HSTlllBHOiii KOHcj:lOpMSL.1111111 6enKa B wanepOHOBOM KOMnneKCe.

37

BaHHblX 6eJIKOB, OHH MOryr rrpernITCTBOBaTb HX ITOJIHOH ,UeHazypall,HH H BOCCTaHaBJmBaTb HaTHBttyIO KOHQ:>opMaumo 6eJIKOB.

YcrnHoBJieHo, 'ITO KpaTKOBpeMeHHbie CTPeccoBhie Bo3,l.l,ettcTBIDI yBeJIH'IHBaIOTBb1pa60Tey BTIII H IIOBbIIIIaIOT yCTOH'IHBOCTb opraHH3Ma K MHTeJibHbIM CTPeccoBhIM BOJ,UettcTBIDIM. TaK, KpaTKOBpeMeHHmr HIIIeMIDI cep,Ue'IHOHMhlIIIUbl B rrepHO)J, 6era rrpH YMepeHHhIX TPeHHPOBKaX JHa'IHTeJihHOIIOBhIIIIaeT YCTOM'IHBOCTbMHOKap,Ua K MHTeJibHOH HIIIe-

MHH, Bhl3BaHHOH CTeHOKap,UHeH HJIH JaKYfIOPKOH cocy,UoB cep,Uua TPOM60M. B Hacm~nnee Bpewi: nep-

crreKTHBHbIMH HCCJie,UOBaHIDIMH B Me,UHUHHe C'IH-

TaIOT IIOHCKH <l>apMaKOJIOf.H'IeCKHX H MOJieeyllilp- H0-6.HOJIOrH'IeCKHXcrroco6oB aKTHBaUHH CHHTe3a BTIII B KJierKax.

4. hO.lle3HU, C8Jl3llHHbte c napyweHUeM fjJO.llOUHlll 6eAK08

Pac'IerhIrroKa3aJTH, 'ITOJIHIIIh tte6oJihIIIaH qacTb

TeopeTH'IeCKH B03MO)!(HhlX BapttaHTOB ITOJIHnerrTH)J,HhIX uerrei1: MmKeT IIPHHHMaTb 0,UHY CTa6HJibttyIO npocTpaHCTBeHHYIO CTPYKTYPYnoJihIIIHHCTBO )Ke TaKHX 6eJIKOB MO)!(eT rrpHHHMaTb MHO)!(eCTBO KOHQ:>OpMaUHH c rrpHMepHO O,UHHaKOBOH 3Heprn:- ei1: ftt66ca, Ho c paJJIH'IHhIMHcBoifcrnaMH. IIepBH'IHasi CTPYKTYPa 6oJibIIIHHCTBa H3BeCTHhIX 6en-

KOB, OT06paHHhIX 3BOJIIOUHeH, o6ecrre'IHBaeT HCKJIIO'IHTeJihHYIO CTa6HJibHOCTh O)J,HOH KOH<l:>op-

MaUHH.

0,UHaKO HeKOTOpbie paCTBOpHMble B BO,Ue 6eJI - KH rrpH H3MeHeHHH YCJIOBHH MOryr nptto6peTaTh KOHQ:>opMaUHIO TIJIOXO paCTBOpHMhIX, cnoco6HbIX K arperaUHH MOJieKyJI, o6pa3yIOIUHX B KJieTKax Q:>H6pHJIIDlpHhie OTJIO)l(eHHSI, HMettyeMhie aMHJIO- H)J,OM (oT naT. amylum - KpaxMan). TaK )Ke KaK H KpaXMaJI, aMHJIOH)J,Hbie OTJIO)!<eHHH BhIBBllilIOT nptt OKpacKe TKaHH HO,UOM. 3-ro MO)[(eT IIPOHCXO,UHTh:

• npH mnepnpO,UYKll,HH HeKOTOpbIX 6eJIKOB, B pe3yJihTaTe qero yBeJIH'IHBaeTCSI HX KOHUeHTpauIDI B KJieTKe;

TOK, Ha6JIIO,UaIOT HX ,UereHepaTHBHhie H3MeHeHHSI H pa3paCTaHHe coe,UHHHTeJihHOTKaHHhIX HJIH fJIHaJlhHhIX KJieTOK. Pa3BHBaIOTCH 6oneJHH, Ha3hrnaeMbie aMHJIOH)J,03aMH. J.(Jrn Ka)l()J,orO BH,Ua aMHJIOH- )J,03a xapaKTepeH orrpe,UeneHHhIH THII aMHJIOH,Ua. B ttacrm1mee BpeMH onttcatto 6onee 15 TaKHx 6one3Hei1:.

DOJie3Hb AJiLU,XaifMepa

£one3Hh Anhuxai1:Mepa - Hatt6onee qacTO OTMe'IaeMhIH ~-aMHJIOH)J,03 HepBHOH CHCTeMbl, KaK npaBHJIO, rropa)[(aIOIUHH JIHU npeKJIOHHOfO B03paCTa H xapaKTepHJyIOIUHHCSI rrporpeccttpyIOIUHM paccrpoHCTBOM naMHTH H ITOJIHOH ,Uerpa,uauHeJI JIH'IHOCTH. B TKaHH M03ra oTKJia,uhrnaeTcH ~-aMtt­

JIOH,U - 6eJIOK, o6pa3YIQIUHH HepacrnopHMbie <1>116-

PHJIJihl, HapyrnaIOIUHe CTPYKTYPY H <l>YHKUHH HepBHhIX KJieTOK. ~-aMHJIOH)J, - rrpO,UYKT H3MeHeHHSI

KOH<t>opMaUHH HOpMaJihHOro 6eJIKa opraHH3Ma qenoBeKa. OH 06pa3yeTCH 113 6onee Kpynttoro npe,U- rnecrBeHHHKa 'IaCTH'IHhIMrrpoTeOJIH30M H CHHTe3HpyeTCSI BO MHOfHX TKaHSIX. ~-AMHJIOH)J,, B OTJIH- 'IHe OT CBOero HOpMaJThHOfO rrpemneCTBeHHHKa, CO,Uep)!(amero MHOfO a-CnHpaJibHblX yqaCTKOB, HMeeT BTOpH'IHYIO ~-CKJia,u'IaTYIO CTpyKTypy, arpernpyeT c o6pa30BaHHeM HepacTBOPHMhIX <l>H6PHJIJI, ycTOH'IHB K ,UettcTBHIO npoTeOJIHTH'IecKHx <t>epMeHTOB.

TipH'IHHblHapyrneHHSI <t>oJI,UHHra HaTHBHhlX 6eJIKOB B TKaHH M03ra eme rrpe,UCTOHT BbIBCHHTb. Bo3MO)[(HO, c B03paCTOM YMeHbIIIaeTCSI CHHTe3 IIIarrepOHOB, crroco6HhlX yqacTBOBaTb B <l>opMHpOBaHHH H ITOLUI,ep)!(aHHH HaTHBHOH KOH<t>opMaUHH 6eJIKOB, HJIH yBeJIH'IHBaeTCSIaKTHBHOCTh rrpoTea3, 'ITOrrpHBO)J,HT K yBeJIH'IeHHIO KOHUeHTpaUHH 6eJIKOB, CKJIOHHbIX H3MeHSITb KOH<t>opMaUHIO.

IlpHOHOBLie 6oJie3HH

npHOHhl - OC06hlH KJiaCC 6eJIKOB, o6Jia,uaIOIUHX tttt<l>eKUHOHHbIMH CBOHCTBaMH. Ilorra,uasi B opraHH3M qeJioBeKa HJIH CITOHTaHHO B03HHKaH B

rrptt nona,uaHHH B KJieTirn HJIH o6pa3oBaHHH B HeM, OHH crroco6Hbl Bhl3hIBaTh TSJ)[(eJihie HeH3JieHHX 6eJIKOB, cnoco6HbIX BJIIDITh Ha KOH<t>op- 'IHMbie3a60JieBaHHH UHC, tta3hIBaeMhie rrpHOHO-

Maumo ,Upymx Moneeyn 6enKa;

npH aKTHBaUHH npoTeOJIH3a HOPMaJihHhIX 6enKOB opraHH3Ma, c o6pa3oBaHHeM HepaCTBOpHMhIX, CKJIOHHbIX K arperaUHH <t>parMeHToB;

B pe3yJihTaTe TO'Ie'IHhIXMYTaUHH B CTpyKzype

6enKa.

B pe3yJihTaTe OTJIO)!(eHIDI aMHJIOH,Ua B opraHax H TKaHSIX ttapyIIIaIOTCSI CTpyKTypa H <l>YHKUHSI KJie-

BhIMH 6one3HHMH. Ha3BaHHe «np»_OHhI» rrpo11cXO,UHT OT a66peBHaTyphI attfJIHHCKOH <t>pa3bl

proteinaceous infectious particle - 6eJIKOBaSI HH-

<t>eKUHOHHaH qacTHUa.

npHOHOBhlll 6eJIOK KO,UHpyeTCSI TeM )Ke reHOM,

'ITO H ero HOpMaJihHhIH attanor, T.e. OHH HMeIOT H,UeHTH'IttyIO nepBtt'IHYIOCTPYKTYPY· 0,UHaKo ,UBa 6eJIKa o6na,uaIOT pa3JIH'IHOHKOH<t>opMauHett: rrpH-

38

10-15

OHOBhIH 6eJIOK xapaKTepinyeTCSI BhlCOKttM CO,IJ,ep- )!(ami.eM 13-CJIOeB, B TO BpeMSI KaK HOPMaJihHbIH 6eJioK ttMeeT MHOro cx-crrttpaJibHbIX yqacTKOB. KpoMe Toro, npuoHOBbIH 6eJIOK o6na,.uaeT ycTOH'-IMBO- cThIO K )leHCTBUIO npoTea3 u, rrona,.uasi B TKaHh M03ra UJIU o6pa3ySICb TaM CTIOHTaHHO, cnoco6cTByeT npeBparueHuIO HopMaJihHOro 6eJIKa B npuoHoBbIH B pe3yJibTaTe Me)!(6eJIKOBblX B3aUMO,lleHCTBttH.

06pa3yeTCSI TaK Ha3hIBaeMoe «jJ,llpO IlOJIUMepu3a-

UUU», COCTOHruee U3 arperupoBaHHbIX npuoHOBblX 6eJIKOB, K KOTopoMy crroco6HbI np:ucoe,UuRHThCjJ HOBhie MOJieKYJibI HOPMaJihHoro 6eJIKa. B peJyJihTaTe B HX npOCTPaHcTBeHHOH CTpYJCTYpe rrpoucxoMT KOH¢opMaUttOHHbie rrepeCTPOHKM, xapaKTepHbie ,II;JijJ rrpuoHOBbIX 6eJIKOB.

113BeCTHbl cnyqaw HacJie,ll,CTBeHHhIX ¢opM npttOHOBblX 6oneJHetl:, BbI3BaHHhIX MYTauIDIMM B CTPYKzype ,IJ,aHHoro 6enKa. QzmaKo B03MO)!(HO u JapIDKeHtte 'IeJIOBeKarrpttOHOBblMtt 6eJIKaMH, B pe3yJibTaTe qero B03HttKaeT Ja6oneBaHtte, npttBOMruee K rtt6eJIH 6oJihHoro. TaK, KYPY - npttoHOBAA 6one3Hb a6op11reHOB HoBo:H: fBttHett, 3ITH,UeMtt'-leCKMH xapaKTep KOTOpo:H: CBH3aH c TPMMUUOHHblM KaHHU6aJitt3MOM B 3TMX 11JieMeHax tt rrepe,I1,a11e:H: ttH¢eKuuoHHOro 6eJIKa OT O,IJ,HOH oco6:u K ,IJ,pyro:H:. B CBH3M c uJMeHeHtteM o6paJa ux :lKM3HM aaHHoe Ja6oneBa-

Htte npaKTHl.JeCKM ttCl.Je3JIO.

B HaCToHruee BpeMjl umepec K rrpuoHOBhIM 60JieJHSIM B03poc B CBH3tt c Japa)!(eHHeM JilO,lleH npttOHaMU nptt ynoTPe6JieHutt MHconpo,IJ,yKTOB, nony- t.JeHHhIX OT )!(MBOTHhIX, HBJIHIOIUMXCH HOCUTeAAMtt rrpttOHOB, Bbl3bIBaIOIUttX «6erneHCTBO KOpOB» (6oJie3Hb Kpotl:Tu¢eJih,IJ,Ta-51Ko6a). HecMOTPH Ha TO, 'ITOrrpttOHOBbie 6emrn 'IeJIOBeKaM )l(J1B()THhlX pa3JIHl.JaIOTCH JIUllib He3HalJ:HTeJibHO, ,ll,OJiroe BpeMH noJiaraJIU, 'ITOcyruecrnyIOT Me)!(BH,llOBhie 6apwpb1 Ha

TIYTtt nepe,I1,a11tt 6oJie3Htt. 0,UHaKo nocJie,IJ,Htte ,IJ,aHHbie IlOKaJaJIU, 'ITO3TU 6aphepbl He a6COJIIOTHhl, H

'ITO cyruecrnyeT rrpHHUttITHaJibHaH B03MO)!(HOCTb

nepe,IJ,at.J:u 6one3Htt OT O,IJ,Horo BH,Ua ,IJ,PYfOMY. TaK, B BenHKo6pttTaHuu K cepe.uMHe 1999 r. 6bmo JapemCTPMPOBaHO oKono 40 cnyqaeB ,IJ,aHHoro Ja6one:saHIDI. IlporH03 He ucKJI1011aeT paJBttnrn 3nH,UeMMM

npHOHOBOH 6oJie3HM B 6JIIDKaHWMe JieT.

W.~YHKUHOHHPOBAHHE

6EnKOB

Ka)!(,ll,hitl: MH,IJ,MBH,IJ,YaJibHhIH 6eJIOK, :uMe10w11H:

YHMKaJibHYIO nepBH'-IHYIO CTPYKTYPY H KOH¢op-

Mauuro, o6na,UaeT :u yHttKaJihHOH: ¢YHKu:ue:H:, OTJIM'-laIOIUetl:ero OT BCex OCTaJibHbIX 6eJIKOB. Ha6op IfH,ll,MBM,IJ,yanbHbIX 6eJIKOB BbIIlOJIHHeT B KJieTKe MHO)!(eCTBO pa3H006pa3HbIX H CJIO)!(HbIX ¢yHKUUH.

Heo6xo,IJ,HMoe ycnoBHe ,ll,JijJ <l>YHKUHOHHpoBaHIDI 6enKOB - np:ucoe,IJ,uHeHne K HeMy ,Upyroro BerueCTBa, KOTopoe Ha3bIBaIOT «JIHrall)J.». JlnraH,IJ,aM:u MOfYT 6bITh KaK Hl13KOMOJieKYAAPHhie BerueCTBa, TaK u MaKpOMoneKYJihI. B3auMo,IJ,etl:crn:ue 6enKa c JlUfaH,ll,OM BblCOKOCneun¢:u11HO, 'ITO orrpe,lleJIHeTCSI cTpoeHHeM yqacTKa 6eJIKa, HaJbrnaeMoro ueHTpoM CBH3bIBaHIDI 6enKa c JI:UraH,IJ,OM MJTH aKTMBHblM ueHTPOM.

A. AKTMBHhIH QEHTP BF.JIKOB

H H3BHPATF.JlhHOCTh CBH3hIBAHHH EfO

C JIHfAH,LI,OM

AKTHBHblH ueuTp 6MKOB - onpe,IJ,eneHHhIH y•rncToK 6eJIKOBOH MOJieKYJihl, KaK npaBMJIO, HaxOMIUMHCjJ B ee yrny6JTeHMU ( «KapMaHe» ), c¢opMHpOBaHHblH pa,.unKaJiaM:u aM:uHOKMCJIOT, co6paHHbIX Ha onpe,IJ,eneHHOM npocTpaHCTBeHHOM yqaCTKe np:u ¢opMupoBaHuu TpeTM'IHOHCTPYJCTYPhI H cnoco6HhIH KOMilJleMeHTaPHO CBH3bIBaTbCjJ c JllffaHD:OM. B n:utte:H:ttoii: rrocne,UoBaTeJibHOCTM rroJT:urrerrTn.n:Hotl: uenu pa,.u:uKanhI, ¢opMupyroru11e aKTHBHhIH uettTp, MOfYT HaXO,ll,MTbCjJ Ha 3Hal.JHTeJTbHOM paCCTOHHUH

.n:pyr OT .n:pyra.

BL1c0Ku1 cneu.uct>u11uocTh CBJ13b1BauHJ1 6eJIKa c

Jiurall)J.OM o6ecnet.JMBaeTCH KOMilJleMeHTapHOCThIO CTPYKTYPhI aKTMBttoro uettTpa 6enKa CTPYKType nttraH,IJ,a (p:uc. 1-25).

Ilo,IJ, KOMITJTeMeHTapHOCTbIO IlOHHMaIOT npoCTpaHcTBeHHOe If XHMH'IeCKOeCOOTBeTCTB:Ue B3aUMO,IJ,eHCTBYIOIUUX MOJieKyn. JIHraH.n: ,ll,OJI)!(eH o6na,IJ,aTh crroco6ttocTh10 BXOD:MTb If npocTpaHcrnettHo COBrra,.uaTb c KOH¢opMau:ue:H: aKTHBHOfO ueHTpa. 3To COBna,.uettue MO)!(eT 6bITb HeITOJIHbIM, HO 6JTaro,IJ,apH KOH¢opMauMOHHOH JTa6:UJibHOCTM 6eJIKa aKTUBHhIH ueHTP cnoco6eH K He60JTbllIHM HJMeHeHHHM If «no.n:roHHeTCH» non: n:uraH,IJ,. KpoMe TOro, Me)l(,lly <l>YHKU:MOHaJihHblMH rpynnaMH mi:raH.n:a 11 pa,.u:uKaJiaMu aMHHOKHCJIOT, o6pa3yro- m:ux aKTMBHblH ueHTp, ,ll,OJI)!(HbJ B03HMKaTb CB,SJ3U, y,IJ,ep:lKMBaIOIUHe JTMraH,IJ, B aKTHBHOM ueHTPe. CBH3M Me)!(ll:y JTttraH,IJ,OM H aKTMBHhIM u;eHTpoM 6eJIKa MOfYT 6bITb KaK HeKOBaJieHTHblMif (HOHHhIMM, BO,ll,OpO,ll,HbIMM, rH,Upo¢o6HbIMH), TaK M KOBaJieHTHbIMU.

39

PMC. 1-25. BsaMMOAeMCTBMe 6enKa c nMrBHAOM. AM 6 - HSKOMnneMSHTapHoe B38MM0/:18iiiCTBM8 M paspyweHMS CB5138iii MS>KAY 6enKOM M n1o1raHJ:IOM; B - KOMnneMeHrapHOe B38MM0/:18iiiCTBMe 6enKa c n1o1raH/:IOM.

1. XapatcmepucmU1ca atcmuBnow u,enmpa

AKTHBHhlll ueHTp 6eJIKa - OTHOCHTeJihHO 11.30JIHPOBaHHhlll OT OKpy)K.aIOmeM. 6eJIOK cpe)J.hl yqacTOK, cq>apMHPOBaHHbIH aMHHOKHCJIOTHhIMll OCTaTKaMH. B 3TOM yqacTKe KaJKLJ,hIH ocTaTOK 6Jiaro)l,ap51 CBOeMy HH)l,llBM.)l,yaJihHOMY pa3Mepy H <l>YHKl.(HOHaJibHbIM rpynrraM <t>opMwpyeT «peJibe<t>» aKTHBHOro ueHTpa.

06be)l,ttHeHtte TaKHX aMMHOKHCJIOT B e)l,HHhlH <l>YHKLlHOHaJihHhlll KOMIUieKc H3MeH5IeT peaKl.(HOHHYIO cnoco6HocTh MX PMHKaJIOB, no;::i:o6Ho TOMY, KaK MeH51eTC513ByqaHtte MY3hIKaJihHOro HHCTpyMeHTa B aHcaM6Jie. Tio3TOMY aMHHOKHCJIOTHhie ocTaTKtt, BXOMlIIHe B COCTaB aKTHBHOro ueHTpa, qaCTO Ha3hIBaJOT «aHCaM6JieM» aMHHOKHCJIOT.

YHHKaJihHhie cBoM.cTBa aKTHBHOro ueHTpa JaBHC5IT He TOJlhKO OT XHMH'leCKMX CBOHCTB <t>opMHPYIOlIIHX era aMHHOKHCJIOT, HO H OT wx Toq - HOM. BJattMHOM optteHTaUHH B rrpocTpattcTBe. CTo3TOMY ;::i:a)K.e He3Haq11.TeJihHhie ttapyrneHH51 06meM. KOH<l:>OpMal.(HH 6eJIKa B pe3yJihTaTe TOqeq-

HhlX HJMeHeHttti ero nepBwrnoti CTPYKTYPhI HJIH ycJIOBHH OKp~a10metf cpe)l,hl MOryT TIPHBeCTH K H3MeHeHHJO XHMHqecKHX H <l>YHKUHOHaJihHhIX CBOHCTB pa)l,HKaJIOB, <f>opMHPYIOlIIHX aKTHBHhlll ueHTp, HapyrnaTh CB513hIBaHHe 6eJIKa c JIHraH)J;OM H era <l>YHKUHIO. Tiptt )l,eHaTypaU,HH aKTHBHhlH

ueHTP 6eJIKOB pa3pyrnaeTC5I, H rrpowcxo)l,HT YTPaTa HX 6HOJIOrttqecKOM aKTHBHOCTH.

llaCTO aKTHBHhIM uemp <t>opM:vrpyeTC51TaKMM06paJOM, 4TO )J;OCTyn BO)l,hI K <l>YHKUHOHaJihHhIM rpynnaM ero pa;::i:HKaJIOB orpaHH4eH, T.e. C03)l,aIOTCH ycJIOBM51 M51 CB513hIBaHH51 JittraH)l,a c PMHKaJiaMtt aMHHOKHCJIOT.

B HeKOTOPbIX CJiyqa5Ix JittraH)l, rrpttcoe;::i:ttHHeTcH TOJlhKO K O)J;HOMY H3 aTOMOB, 06Jia)l,a10meMY orrpe- ;::i:eJieHHOH peaKUHOHHOH crroco6HOCThJO, HaTipHMep rrpwcoe,u:irnettwe 0 2 K )K_eJie3y MHorno6wtta HJIH reMorno6ttHa. O;::i:ttaKo cBoM.cTBa ;::i:attHoro aToMa H36ttpaTeJihHO B3aHMO)l,etiCTBOBaTh c 02 orrpe)l,e- Jl5IIOTC51 CBOHCTBaMM pa)l,HKaJIOB, OKpy)l(aIOIUHX aTOM )l(eJie3a B COCTaBe reMa. f eM CO)lep)!(HTC5I H B ;::i:pyrwx 6eJIKax, TaKMX KaK 11,HTOXpOMbl. 0)1,HaKo <l>YHKUM51 aTOMa )K:eJie3a B 11,llTOXPOMaX HHa51, OH

CJI~MT rrocpe;::i:HMKOM M51 rrepe;::i:a4H 3JieKTPOHOB OT o;::i:Horo semecrna )l,pyroMy, nptt 3TOM )l(eJie3o CTaHOBMTC51 TO )lByx-, TO TpeXBaJieHTHhJM.

UeHTp CB5I3hIBaHM51 6eJIKa c JittraH)l,OM qacTo pacrroJiaraeTC51 Me)K:)ly ;::i:oMettaMH. HanpttMep, rrpoTeOJIHTttLiecKHtf <t>epMeHT TpttncttH, yqacTBYIOIUHti B m;::i:poJIM3e IIeIITM)J;HhIX CB513eti IIHIUeBhlX 6eJIKOB B KHrneqHHKe, HMeeT 2 AOMeHa, pa3,IIeJieHHhIX 6oP03,IIKOH. BttyTpeHH515I rroBepxHoCTh 6opo3.IIKH <t>opMttpyeTC51 aMHHOKMCJIOTHhIMH PMMKaJiaMtt 3THX

40

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