підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

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B MOJieKYJie reMorno6nHa S (TaK Ha3BaH aHoMaJihHhIH reMorno6nH) MYTaHTHhIMH OKa3aJIHCh 2

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Moneey11b1 Hb S

PMC. 1-40. ACCOl.IM81.1M51 1111one1<yn Ae30KCMreMorno6MH8 s.

B oKcnreMorno6nHe S KOM11J1eMeHTapHh1i1 yqa-

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napa1111eJlbH8ft YKilBAKa

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2. HJMeueuUR aM-UHOKUCAomuow cocma6a

6 o6.11acmu aKmuBuow u,eumpa zeMoZ.11o6uua

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feMorno6ttH XaMMepeMttra - no6HHa A, me B noJio)Kett1111

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3. HJMeuenUR aM-UHOKUCAomuozo cocma6a, iJetjJopMupy10111,ue mpemurmy10 cmpyKmypy leMOZ.llOOUHa

Bo Beex HOpMaJihHhIX reMorno6ttttax H B MHOrno6HHe B Meere nepeeeqeHIDI ,UByx a.-enttpaneii B H E Haxo,UttreH aMttHoKHenora rn11u11tt. TaK KaK fJIHUHH BMeero pa,11,HKana eo,Uep)Klff aTOM BO,llopo- )la, B 3TOM Meere )lBe enrrpaJIH ITJIOTHO npHJieraror JJ.pyr K )lpyry.

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VI. MHOr006PA3111E 6EflKOB

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M3 KOTOpbIX OHM nOCTpOeHbl.

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A. KrrAccH<I>HKAQHH BEJIKOB

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1. Cmpoeuue u </JyuKu,uu KOMa2euo6

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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.

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.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).

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

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2. Cmpoeuue u tjJyHKU.UR 3./lacmuua

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JIHIIIb HeMHOro rn,llpOKCMnpoJIMHa; ITOJIHOCTbIO OTCyTCTByeT raupoKCMJIH3MH.

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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.

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-

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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,

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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-

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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-

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-

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

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).

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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%

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.IlBYMH .IlOMeHaMH.

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

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2. CynepceMeucmBo UMMy11ouo6y.11u1106

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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.

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3. CeMeucmBo UMMy110Z.1106y.11u1106

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

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

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MH B-nHMcPOU:HTaMH B 6onhIIIHX KOJittqecrnax rrp11

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BTOPHO norra.uaeT B opraHl13M.

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