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підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

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2. HiJbl - cneu,llfjJU'leCKUe JlUlQHObl

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5 cneuHQlH'leCKHXD.HCYJih<i>H)lHbIX cmJJett (B 3aBHCHMOCTH OT BH)la TOKCHHa). Cpo,UCTBO HeiilpoTOKCHHOB K XOJIHHepr11qecKHM peuerrTopaM OlJeHb BhICOKO (KnHcc = 10-11 ). Ol!eBH)lHO, Me)J()ly TOKCH-

CH3"-

 

 

/CH3

CH3-N+-CH2-CH2-0-C-(CH2)z-C-O-CH2-CH2-W -CH3

CH3/

o

o

"-CH3

,Ql-1Tlllfll-1H

H2C-CH-CH2

0

H

I

'- "..

II

~

N-CHyCH-O-c-9~

H2C-CH-CH2

 

CH2

I

OH

ATporn1H

CxeMa A

OH OH

>=--'

HO~CH-CH2-NH

I

CH3

AApeHan1-1H

Me3aTOH

CxeMa 6

44

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V.OCOEiEHHOCT~

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0JlHrDMepHbie 6eJlKH np051BJl51TOT CBOHCTBa, OTCYTCTByIOWHe y MOHOMepHhIX 6eJlKOB. BmrnHHe lfeTBePTHlJHOH CTPYKTyphl Ha cpyHKUHOHaJlhHhre CBOHcma 6eJIKa MO)KHO paCCMorpeTh, cpaBHHBaH crpoeHHe H cl>YHKIIHH )lByx pO)lCTBeHHhIX reMCO)lep)KaWHX 6eJlKOB: MHOfJl06HHa H reMorno6HHa. 06a 6eJlKa HMeTOT o6wee 3BOJl!OUHOHHOe npOHCXO)l(,!l,eHHe, CXO)l- HyIO KOHcpopMaUHTO OT)leJlhHhlX nommenTHllHhIX uenei1: H CXOllHyIO ¢YHKIIHIO (yqacrnyIOT B TPaHCnopTe KHCJlOpO)la), HO MHOrno6HH - MOHOMepHhIH 6eJloK, a reMorno6HH - TeTPaMep. HanHlJHe lJeTBepTHIJHOH CTPYKTYPhI y reMorno611Ha npH)lafu 3TOMY 6eJlcy cBo:Hcrna, OTCYTCTBYIOWHe y MHorno611Ha.

A. CTPYKTYPA II ct>YHKUIIII MIIOfJIOBIIHA

Mttorno6HH OTHOC5IT K KJlaccy reMCO)lep)KaWHX 6eJlKOB, T.e. OH CO)lep)KHT npoCTeTH'leCKYTO rpynny - reM, llOBOJlhHO rrpo'IHOCB5l3aHHYTO c 6eJlKOBOH lJaCTbTO. M HOfJl06HH OTHOC51T K rno6yll51PHblM 6eJlKaM; OH HMeeT TOJlhKO OllHY noJlHnenTHllHYTO uenh.

TeHCHBHOH Mhillle'!HOHpa60ThI, KOr)la rrapuHaJlbHOe )laBJleHHe KHCJlOPO)la B TKaHH na]laeT, 02 ocB060)l(,!l,aeTC5l H3 KOMTIJleKca c MHOfJl06HHOM H HCTIOJlh3yeTC5l B MHTOXOH)lpHHX KJleTOK llJl51 TIOJlylJeHHH Heo6XOllHMOH llJl51 pa60Thl MhIIIIU 3HepmH.

2. Cmpoeuue MUOl.!loouua

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CB513aHhI lJeTblpbM.H KOOp)lHHaUHOHHbIMH CB51-

351MH c Fe2+, HaxOMWHMC51 B ueHTpe MOJleKy-

JihI (pHC. 1-29).

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TpeTHIJHAA CTPYKTYPa HMeeT BHll KOMrraKTHO:tl: fJl06yJlbJ (BHYTPH npaKTH'!eCKH HeT CB060)l- HOro MeCTa), o6pa30BaHHOH 3a C'leTneTeJih H TIOBOPOTOB B 06JlaCTH HeCrrHpaJIH30BaHHhIX yqacTKOB 6eJIKa. BHYTPeHHHH '!aCTh MOJleKyJihI IlOlJTH ueJlHKOM COCTOHT H3 fH)lpocpo6HhIX PMHKaJIOB, 3a HCKJlTO'!eHHeM llByx ocTaTKOB fHC, pacnoJlaraTOWHXCH B aKTHBHOM ueHTpe.

I. KAemo11naa .!lOKCl.!lU3a1<UR u f/JyHKUiUR

3. CBRJbtBauue zeMa c anoMuol.!lofJuuoM

M11orno611H co)lep)J(HTC51 B KpacHhIX MhIIIIUax H

f eM - crreuHcpH'!eCKHHJlHraH)l arrOMMOfJl06H-

yqacrnyeT B 3anacaHHH KHCJlopoLI,a. B ycJlOBH51X HH-

Ha, rrpHcoemrnHTOWHHC51 K 6eJlKOBOH qacTH B yr-

45

1-30).
)1(11BOT-

(J

NH

n111ppOilbHOe KOilbl.10

PMC. 1-29. CrpoeHMe reMa, BXOA511J.19ro B COCTaB MMorno6MHa MreMorno6MHa.

ny6nemu1 Me)l()J:y ABYMH a-c1rnpanHMH F 11 E.

I.l,eHTP CB513bIBaHl151 c reMOM o6pa30BaH rrpeHMyrn:ecTBeHHO m;:i:p0<po6HblMl1 OCTaTKaMl1 aMl1-

HOKHCJIOT' OKpy)l(alOIUHMl1 rtt;:i:po<t>o6Hble IlHp-

pOJihHhie KOJihUa reMa. ,ll.Be 6oKOBhie rpyrrrrh1 rrporrHOHOBhIX KHCJIOT, 110Hl13HpoBaHHhie rrp11 qm-

3HOJIOrlfqecK11X 3HaqeHl151X pH, BhlCTyrraIOT Ha IlOBepXHOCTl1 MOJieKyJihl.

B aKTl1BHhIH ueHTP arroMttorno6ttHa KPOMe rHApo¢o6HhIX aM11HOKI1CJIOT BXOMT TaK)l(e 2 OCTaTKa fttc (fttc64 H f11c93 l1Jil1 fttc E7 11 r11c Fg), 11rpa10- m:11e Ba)l(HYlO pOJih B cPYHKUHOHHpOBaHHH 6eJIKa. 0HH pacrroJIO)l(eHhI no pa3Hhie CTOpOHhI OT ITJIOCKOCTl1 reMa 11 BXOMT B cocTaB crr11panei1 F H E,

Me)l()zy KOTOPhIMl1 pacrronaraeTcH reM. ATOM )l(e- ne3a B reMe MO)l(eT o6pa30BhIBaTh 6 Koop;:i:11Hau110HHhIX CB513ei1, 4 113 KOTOphlX y;:i:ep)l(l1BalOT Fe2+ B ueHTPe rrp0Torrop¢11p11Ha IX (coe;:i:11H5IH ero c aTOMaMH a30Ta nttppOJihHhIX KOJieu), a 5-51 CB513h B03Hl1KaeT Me)l()zy Fe2+ 11 aTOMOM a30Ta 11MHAa30JibHOro KOJihUa fttc F8 (pttc.

fttc E7 xorn 11 He CB513aH c reMOM, HO Heo6xo- ;:i:11M AJI5I npaBI1JlhHOH op11eHTaUHH H np11coe;:i:H- HeHl15I ;:i:pyroro n11raH;:i:a - 0 2 K M11orno611Hy.

AMl1HOKHCJIOTHOe OKp~eHHe reMa C03,LJ,aeT ycJIOBl151 MH AOBOJihHO npoqHoro, HO o6paTHMoro CBH3hIBaHl151 0 2 c Fe2+ M11orno611Ha. f11Apo¢06Hh1e OCTaTKl1 aMl1HOKHCJIOT, OKpy)l(alOIU11e reM, npenHTCTBYIOT npOHHKHOBeHHlO B ueHTP CB513hlBaHl151 MHOrno6HHa BOJJ:hl H OKHCJieHHlO Fe2+ B Fe3+. TpexsaneHTHoe )l(eJie3o B cocrnse reMa He crroco6Ho rrp11coe;:i:HH5ITh Or

Ii. CTPYKTYPA H Cl>YHKQHH fEMOfJIOBHHA

feMOrJIOOHHhl - poJJ:CTBeHHbie 6eJIKH, HaxOAfilUHecSJ B 3pl1TpOUHTilX qeJIOBeKa H Il03BOH0qttblX

HhIX. ~ 6eJIKI1 BhITIOJIIDIIOT 2 sa;Kllhle ¢YJIKUHH:

nepeHOC 0 2113 nerKHX K neptt¢ep11qeCKHM TKaHHM;

yqaCTl1e B nepeHOCe C02 11 TipOTOHOB 113 neptt$ep11qecK11X TKaHeH B JierKHe MH IlOCJie;:i:y-

lOIUerO Bhrne;:i:eHl151 113 opraHl13Ma.

KpOBh e)l(e,LJ,HeBHO JJ:OJI)l(Ha rrepeHOCl1Tb 113 nerKHX B TKaHl1 OKOJIO 600 JI 02" TaK KaK 02 IlJIOXO pacTBOpl1M B BO,LJ,e, TO npaKTmecKH BeCb KHCJIOPOA B KpOBH CB513aH c reMorno611HOM 3Pl1TPOUHTOB.

OT crroco6HocT11 reMornofilma HaChIIUaThCH 0

2

•.

B nerKHx 11 OTHOCHTeJibHO nerKO OTJJ:aBaTb ero

B

I

/

F-cmt- J E-cm1- J

paflb panb

PMC. 1-30. Pacnono>KeHMe reMa B BKTMBHOM L\9HTpe anOMMorno6MH8 MnpoToMepoa anoreMorno6MHa.

46

1-31, 6).
1-31, A).

KaIUIJIJUipax TKaHeH 3aBl1CHT KOJIM'feCTBOIIOJiyqaeMOro TKaHHMl1 02. 11 11HTeHCl1BHOCTb MeTa60Jil13Ma. c .upyroti CTOpOHhl, 02 - CMJibHbIH OKMCJil1TeJib, 1136bITOK rroczyIIJieHMH 02 B TKaHl1 MO)l(eT rrp11BecTH K IIOBpe)J()J;eHHIO MOJieKyJI 11 HapyrneHl1IO CTpYKTYPbl 11 cj:Jymmttti KJieTOK. Ilo3TOMY Ba)!(Hei1:rnaH xapaKTep11cT11Ka reMorno6mm - ero crroco6HocTb peryJIHPOBaTb CPO.UCTBO K 02 B 3aBl1Cl1MOCTl1 OT TKaHeBbIX ycJIOBl1H.

feMOrJI0611Hbl, TaK )Ke KaK Ml10rno611H, OTHOCHT K reMorrpoTeHHaM, HO OHM 11MeIOT qeTBepTM'I- HYIO CTPYKTYPY (cocTOHT 113 4 rron11rrem11,UHhix uerreti), 6Jiaro.uapH KOTopoii: B03HHKaeT B03MO)l(HOCTb pery.Jl51U1111 11X cPYHKUl1H.

1. I'eMOM06UHbl 'le.ll06e1Ca

I'eMOrJI06HHblB3POCJIOro 'leJioBeKa

B 3PHTpOUMTax B3pocnoro 'IeJioBeKareMorno611H COCTaBJUieT 90% OT Bcex 6eJIKOB .uaHHOH KJieTKM.

I'eMomo6uuA - ocHOBHOH reMorno611H B3pocnoro opraHl13Ma, COCTaBJIHeT OKOJIO 98% OT o6mero KOJIH'IeCTBareMorno611Ha, TeTpaMep, COCTOl1T 113 2 IIOJIHIIeIITH)J,HbIX uerreti a H 2 p

(2a2p).

feMOrJI06HH ~ HaXO.U11TCH B opraHl13Me B3pocJIOro qeJIOBeKa B MeHbllleii: KOHUeHTpau1-rn:, Ha ero .UOJIIO rrpHXO.Ul1TCH OKOJIO 2% o6mero reMorno611Ha. Ott cocTOMT 113 2 a- 11 2 S-ue- rreH:.

feMorJio6uu A1c - reMornoforn A, MO.UHcPHU11poBaHHhIH KOBaJieHTHblM rrp11coe.u11tteH11eM K HeMy fJIIOK03bl (TaK Ha3bIBaeMbIH fJ111K0311mipoBaHHblH reMorno611H).

feMorno6nHb1, cnHTe3upyrow.uecB B nepuo.u Buyrpuyrpo6uoro pa3BHTHJI UJIO)J,a:

3M6pHOHaJibHblH reMOfJI06HH Cl1HTe311pyeTCH B 3M6p110Ha.JibHOM )i(e.JITOqHoM MellIKe 'Iepe3 HeCKOJibKO He)J,e.Jih ITOC.Jie OIIJIO.UOTBOpeHl1H. Ilpe.ucrnB.JrneT co6oi1 TeTpaMep 2~2£. l!epe3 2 He.u IIOC.Jie cpopM11POBaHMH rreqemf IIJIO,Ua B Heif Haq11HaeT C11HTe311poBaThCH reMOfJI06HH F, KOTopb1ii: K 6 Mee 3aMemaeT 3M6pttoHanhHhIH reMorno611H.

I'eMomo6uuF - cpeTaJihHhIH reMorno611H, c11me311pyeTCH B rreqemf 11 KOCTHOM M03re IIJIO)J,a .uo rrep110.ua ero po)J()J;eHMH. HMeeT TeTpaMepHYJO CTpYKTYPY, COCTOHJUYIO 113 2 a- 11 2 y-uerreH:. Iloc.Jie po)!(UeHMH pe6ettKa rrocTerretttto JaMemaeTcH Ha reMorno611H A, KOTOphIH Ha'I11HaeTc11H-

Te3HpoBaTbCH B KJieTKax KOCTHOro M03ra ~e Ha

8-M MeCHUe pa3Bl1TMH ITJIO)J,a.

2. Cmpoenue zeMoMo6una A

CTJJoeuue npoTOMepos reMorJio6uua

KoHcpopMau11H oT.ue.JihHhIX rrpoToMepoB reMorno611tta y.u11B11TeJibHO HarroM11ttaeT Kott¢>opMau1110 Ml10rno611tta, HeCMOTpH Ha TO, qTO B rrepB11qttoH CTPYKTYPe HX rro.JI11rrerrTHUHhIX uerreH: 11,UeHTlfqHbI TO.JlbKO 24 aMl1HOKMCJIOTHbIX OCTaTKa. IlpoTOMepbI reMorno611tta, TaK )Ke KaK 11 arrOMl10fJI0611H, COCTOHT 113 8 crr11paneti, CBepHYThIX B IIJIOTHYJO rno6y-

.JI5IPHYIO CTpYKTYPY, co.uep)!(:aIUYIO BHYTPeHHee fl1,U- pocj:Jo6Hoe MPO 11 «KapMaH» MH CBH3bIBaH11H reMa. Coe.u11HeH11e reMa c rno611ttOM (6e.11KOBOH 'IaCTbIO) aHaJIOr114HO TaKOBOMY y MHOf.JI0611Ha - r11,Upocj:Jo6HOe OKp~eHHe reMa, 3a HCKJIIO'IeHHeM2 OCTaTKOB r11c E7 11 r11c F8 (p11c. 1-31). 0.uttaKO TeTpaMepHaH CTpyKzypa reMorno611Ha rrpe.ucTaBJIHeT co6oH: 60-

.Jiee CJIO)!(HbIH CTpYKTYPHO-cpyttKUHOHaJibHbIH KOM-

IIJieKC, 4eM MHOf.JIOforn.

PoJib rucTu.uuua E7 B fi>YHKUHOHHposauuu MHOfJIOOHHa H reMOfJIOOHHa

feM HMeeT BbICOKOe cpo.ucrno K OKCHUY yrne-

po.ua (CO). B BO)J,HOH cpe.ue CB060)J,HbIH OT 6e.JIKOBOH qaCTl1 reM CBH3bIBaeTCH c co B 25 000 pa3 CMJibHee, qeM Or BhICOKaH cTerreHb cpo.ucrna reMa K co ITO cpaBHeHl1IO c 02 o6bHCHHeTCH pa3HbIM rrpocTpaHCTBeHHbIM pacrrO.JIO)l(eH11eM KOMIIJieKCOB Fe2+ reMa c CO 11 0 2 (pHc.

B KOMIIJieKce Fe2+ reMa c CO aTOMhI Fe2+, yrnepo.ua 11 KMC.JIOpo.ua pacrrOJIO)!(eHhI Ha O.UHOH rrpHMOH, a B KOMIIJieKce Fe2+ reMa c 0 2 aTOMhI )l(e.Jie3a 11 KHC.Jiopo.ua pacnoJIO)l(eHbI rro.u yr.JIOM, irro OTpa- )!(aeT 11X OIITl1MaJibHOe rrpocTpaHCTBeHHOe pacrro-

JIO)l(ettHe.

B MHorno611He H reMorno611He HM Fe2+ B 06.11aCTH rrp11coe)J,HHeHMH 02 pacrro.JIO)l(eH r11c E7, HapylllaIOIUHH OIITl1MaJibHOe pacrrO.JIO)l(eH11e co B ueHTpe CBH3bIBaHMH 6e.JIKOB 11 OC.Jia6.JI51IOIU11H ero B3al1Mon:eii:CTBHe c reMOM. HarrpOTHB, TOT )Ke rue E7 C03n:aeT OIITl1Ma.JibHhie yc.JIOBHH )J,JIH CBH3b!Ba-

HMH 0 2 (pwc. B pe3y.JihTaTe cpo.ucrno reMa K CO B 6e.JIKax Bcero B 200 pa3 rrpeBhilllaeT ero cpon:crno K 0 2.

CHtt)l(eHwe cpon:crna reMco.uep)l(aIUHX 6e.JIKOB K co HMeeT Ba)l(HOe 6110.JIOfHqecKoe 3Ha'IeH11e. co 06pa3yeTCH B He60.Jibllll1X KOJ111qecTBax np11 KaTa60Jil13Me HeKOTOpbIX BeIUeCTB, B 'IaCTHOCTH

47

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-o- P-O-CH2-CH2- COO-

o'- 0I

I

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I

o-

2,3-6Mcj>occj>ornMl.48PaT

PMc.. 1-37.. BnMRHMe pH Ha KpMeyio i:1.11cco1.4Mal..lMM 0 2 MA reMorno6MHa..

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Perymm,us c noMOUl,LIO 2,3-6nclK>cclK>nmu,epaTa

cpO,!l;CTBa reMOrJIOOHHa K 02

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51

1-38).

UettTpaJibHaH rroJiocTh - MecTo rrpHcoemrnemrn

B<l>f.

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IlpHcoenHHeHtte B<l>f K ne3oKcHreMorno6HHY

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pbIB HOHHbIX CBH3eH Me)l(,Ily nHMepaMH a A H a A

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2,3-6<l>r=O

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52

2,3-

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B KOHCepBHfJOBaHHOH KpOBH

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6. Pezy.MlmopHbte CBoiicmBa O.llUWMepnow 6e.aKa ZeMOi!.!lo6una

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KUHIO K H3MeHeHH51M, npOHCXOMll.l,HM B OKpy)l(a- IOll.l,ett cpe,Ue.

7. Oco6ennocmu cmpoenll.Jl

u rPYHKl(UOHUp06QHll.Jl leMOi!.!I06una 11.11oiJa

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B <Ptt3HOJIOrttqecKMx ycJIOBH51X HbF MMeeT 60nee BhICOKoe cpo,II,CTBO K 0 2, qeM HbA, qTo co3,.!1,a- eT OIITMMaJihHhie YCJIOBMH )lJI51 TpaHCnOpTa 0 2 H3 KpoB11 MaTepH B KpOBh TIJIO,Ua. 3To cBoi1crno HbF o6ycJioBJieHo TeM, qTo OH cJia6ee, qeM HbA CBH3hrnaeTcH C E<I>f. <l>M3110JIOIBlfeCKHe OC06eHHOCTH HbF CBH3aHbl c oco6eHHOCT51M11 ero CTpoeH11H: BMecTo ~-mo6HHOBhIX uenei1 B HbA, oH co,Uep)l(J[T ,UBe y-1.1,en11 (~-no)lo6Hb1e). CBH3hIBaHMe 2,3-E<l>f c HbA npo11cxO)lMT npH yqacT11H noJio- )l(J{TeJihHO 3ap5l)l(eHHbIX pa,UHKaJIOB aMHHOKH:CJIOT ,UByx ~-uene.H, HeKOTOpbie H3 KOTOPhIX OTCYTCTByIOT B nepBHqHo:tl: CTPYKTYPe y-uene:tl:. B cpe,IJ,e, JUf-

IIIeHHOH 2,3-E<l>f, HbA 11 HbF rrpoHBAAIOT o,IJ,11HaKoBoe BblCOKOe cpo,UCTBO K 02.

B. HACJIE)J,CTBEHHhIE HAPYIIIEHIUI IIEPBWIHOH CTPYKTYPhl H <I>YHKQHH fEMOrJIOHHHA A - HACJIE)J,CTBEHHhIE fEMOfJIOHHHOIIATHH

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M pa3BMTMe aHeMHM, qTO ITPMBO,UMT K T5l)l(eJibIM KJIMHMqeCKMM TIOCJie,IJ,CTBH51M.

B aHOMaJihHhIX reMorno611ttax H3MeHeHHH MOryr 3aTparMBaTb aMMHOKMCJIOTbJ:

53

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