підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

,Qen1APOKC1'18L.19TOH¢oc¢aT

1,3-6111ccpoccporn1111..1epaT

AT<t>A.O_,._<t>____l7

061J.1111~ nyTb

Karn6on111aMa ~

~ AT<t>

una

C02 H20, 3Heprnsi

Mll1TOXOH.QP111Sl

uecc Ha3hIBaIOT atta::ipo6HhIM pacmmoM, MJIM aHa::i- po6HhIM rnttKOJIM30M. AHa::ipo6HhIH pacrrau rmoK03hI 3Heprern:qecKM MaJI03cPcPeKTMBeH, HO HMeHHO 3TOT rrpouecc MmKeT CTaTh e)J,.HHCTBeHHhIM MCTOqH.HKOM 3HeprMM )],J151 MhillleqHoil: KJieTKM B OJI.HCaHHOH CMryau.HH. B )J,aJihHettllleM, KOr)J,a CHa6- )l(eHMe Mhllllll KMCJIOp0,!1,0M 6y,!1,eT ,!l,OCTaTOqHhIM B pe3yJihTaTe nepexo,!l,a cep,!1,ua Ha ycKopeHHhIH pHTM, aHa::ipo6HhIH pacrrau rrepeKJI10qaeTC5I Ha a::ipo6HhIH. IlYTH KaTa60JIH3Ma fJIIOK03hI ll llX 3Hepre~eCKHH

::i<P<PeKT JIOKa3aHhl Ha pMC. 7-34.

B. A3POliHhIH f.JUIKOJIM3

A::ipo6HhIM rJIMKOJIM30M Ha3hrna10T rrpouecc OKMCJieHM51 rJIIOK03hl )J,0 JIMpOBMHOrpauHOH KHCJIOThI, rrpoTeKaIOIUMH. B rrpMCYTCTBMM KMCJIOpO,!l,a. Bee <PepMeHThI, KaTaJIM311pyroru11e peaKuMM 3TOro rrpouecca, JIOKaJIM30BaHhl B UMT030Jie KJieTKH.

1. 3manb1 a3po611ow CAUKO.tlU3a

B a::ipo6HOM rnMKOJIM3e MO)l(HO BhI,!l,eJIHTh 2

3Tarra.

1.Ilo,!1,roTOBMTeJihHhJH 3Tarr, B XO)J,e KOTOporo fJIIOK03a <Poc<PopMJinpyeTC51 11 pacruerrAAeTC51 Ha )],Be MOJieKyJihI <Poc<PoTptto3. 3rn cepn51 peaKUHH rrpoTeKaeT c MCJIOJih30BaHMeM 2 MOJieKYJI A T<I>.

2.3rnrr, corrpn)l(eHHhIH c CMHTe3oM AT<I>. B pe3YJihTaTe 3TOH cepMM peaKUMH cPOCcPOTPM03hl rrpeBparuaIOTC51 B IlMPYBaT. 3Heprll51, BhICBO-

6o)l()J,alOIUa51C51 Ha 3TOM 3Tarre, MCilOJlh3yeTC51

)],JI5I c11me3a I0 MOJih A T<D.

2. Pea«u.uu a:Jpo611ow CAUKO.tlU3a

Ilpeeparn.euue rJIJOKOJo-6-<l>oc<l>aTa e 2 MoJieIQ'JibI

muu,epaJib,!l,erH,!1,-3-<l>oc<l>aTa

Ta fJIIOK030cPOCcPaTM30Mepa3hl.

3aTeM CJie,!l,yeT erue O,!l,Ha peaKUM51 cPOCcPOpMJIMpOBaHH51 c HCITOJlh30BaHMeM <Poc<PaTHOro ocTaTKa n 3Hepr1111 AT<I>. B xo,!l,e 3TOH peaKUMM, KaTaJIM3HpyeMOH <Poc<Po<PpyKTOKMHa30H, cPPYK- T030-6-<Poc<PaT npeBpamaeTC51 B cPPYKT030- l ,6- 611c<Poc<PaT. )],aHHa51 peaKIJ,M51, TaK )Ke, KaK reKcoKMHa3Hmr, npaKTMqeCKH Heo6paTMMa, H, KpoMe Toro, oHa Ha116011ee Me)],JleHHa51 M3 Bcex peaKUMH rJIHKOJIH3a. PeaKUH51, KaTaJIH3MpyeMa51 <Poc<PocPPYKTOKMHa3oil:, orrpe,!l,eJrneT CKOpOCTh BCero rJIMKOJ1M3a, JI03TOMy, pery1111py51 aKTMBHOCTh <Poc- <Po<PpyKTOKMHa3hl, MO)l(HO M3MeH51Th CKOpOCTb KaTa6oJIH3Ma rJIIOK03hl.

<l>pyKTo3o-1,6-611c<Poc<PaT ,!l,aJiee pacrueI1JI51eTC51 Ha 2 TpM03o<Poc<Parn: rnttuepaJih,!l,ern)J,-3-<Pocc:PaT M ,!l,Hrn,!l,poKcttaueTott<Pocc:PaT. PeaKUHIO KaTaJIM3MpyeT <PepMeHT <l>PYKT0306uc<1>oc<1>aTaJib,IJ;OJia3a, HJlH rrpOCTO aJib,ll,OJiaJa. 3TOT <PepMeHT KaTaJIH3HpyeT KaK peaKUHIO aJib,!l,OJlhHOro pacruellJleHH51, TaK H aJih-

,--------,

: H-C-OH:

I____ I____ _I

HO-C-H

I

H-C-OH

I

H-C-OH

I

CH20P032- rIllOK030-6-cpoccj:laT

]-'"'°"'"'OMepa"'

HO-C-H

I

H-C-OH

I

H-C-OH

I

H2COPO/-

<t>pyKT030l-6-cj:loccj:la1

AT<t> <t>occj:locj:JpyKToK111Ha3a

AA<t>

I__ ---1-- _____ I

H-C-OH

I

H-C-OH

I

H2COPO}-

PMC. 7-35. npespa~eHMe rnt0K030-6-Q>ocQ>aTa B TPlll03oQ>oc- <t>aTbl.

1l0JlbHOH KOH)leHcau1111, T.e. o6paTHMYJO peaKUHIO. IlponyKTbl peaKUHH aJTb)lOJlbHOfO pacruenJTeHHH - H30MepbI. B noc11enyJOru11x peaKUHHX rn11Ko11113a HCTIOJlb3YeTCH TOJlbKO rn11uepaJTb)lef11).l-3-Q:>ocQ:>aT, no3TOMY n11mnpoKc11aueToHQ:>ocQ:>aT npenparuaeTCH c yqacT11eM Q:>epMeHTa rp11030Q:>ocQ:>aT113oMepa3bI B rn11uepoaJTh.llerHA-3-Q:>ocQ:>aT (p11c. 7-35).

B on11caHHOH cep1111 peaKu11ti: ABa)!(,ll,bI npo11cxo1lHT Q:>ocQ:>op111111ponaH11e c 11cno11b30BaH11eM ATCI>. 0AHaKo pacxo11,osaH11e )J,Byx MOJTeKYn ATCJ> (Ha OAHY MOJTeKyJTY rJTIOK03bl) traJTee 6yneT KOMneHCHpOBaHO CHHTe3oM 6011bwero Ko1111qecTsa ATCI>.

Ilpesparn,eune rnnuepaJib,IJ.erM-3-<t>oc<t>aTa B nnpysaT

3ra qacTb a3po6Horo rn11Ko11113a BKJTIOqaeT peaK- mm, CBH3aHHble c CHHTe30M ATCJ>. Ha116011ee CJTO)l(- HOH B )J,attttoti: cep1111 peaKu11ti: HBJTHeTCH peaKUHH npesparueH11H rmmepMhnemn-3-Q:>ocQ:>ara B l ,3- 611cQ:>ocQ:>orn11uepaT. 3rn npesparuett11e - nepsaH peaKUHH oK11c11eH11H B xo)le rn11Ko11113a. PeaKUHIO KaTaJT11311pyeT r.rmuep3.1Ih,I1.em,IJ.-3-<t>oc<t>aT,IJ.em,IJ.poreua- 3a, KOTOpaH HBJTHeTCH NAD-3aB11c11MbIM Q:>epMettTOM. 3HaqeHHe )laHHOH peaKU1111 3aK.TilOlJaeTCH He TOJlbKO B TOM, lJTO 06pa3yeTCH BOCCTaHOBJTeHHbIH KoQ:>epMeHT, OKHCJTeHHe KOTOporo B ,Il,bIXaTeJTbHOH uen11 conp51)1(etto c CHHTe3oM ATCI>, HO TaK)l(e 11 B TOM, 'ITOCB060,Il,HaH 3Heprmt OKHCJTeHHH KOHUeHTp11pyeTCH B MaKP03prH'leCKOHCB513H npO.UYKTa peaKUHH. fJTHUepaJTb,Il,efH)J,-3-Q:>ocQ:>aT,Il,emnporeHa3a conep)l(J1T B 3KTHBHOM ueHTpe OCTaTOK UHCTe11Ha, cy11hQ:>rH)J,pHJTbHaH rpynna KOTOporo np11tt11MaeT Henocpe)J,cTBeHttoe yqacT11e B KaTaJTH3e. OK11c11emre rn11uepaJTb)lef11)J,-3-Q:>ocQ:>ara npHBO,Il,HT K BOCCTaHOBJTeHHIO NAO 11 06pa3onaH1110 c Yl.JaCTHeM H 3P04 BhI- COK03HepreTw-1ecKoti: aHr11,I1,pH1lHOH CBH3H B l ,3-611c- <1>oc<Porn11uepaTe B noJTo)l(eHHH l. B c11eny10rueti: peaKUHH BbICOK03HepreTWieCKl1H Q:>ocQ:>aT nepenaercH Ha AllCI> c o6pa30BaHHeM AT<ll. <llepMeHT, KaTaJTl1311PYIOIUHH 3TO npesparuemte, H33BaH no 06pamoti: peaKli1111 Q:>ocQ:>orm1uepaTK11HaJoti: (KHHa3hI Ha3bIBaJOTC51 no cy6cTpaTy, H3XOMIUeMYC51 B ypaBHeHHH peaKu1111 no ODHY crnpoHy c AT<ll). )laHHaH cepIDI peaKUHti: noKa3aHa Ha p11c. 7-36.

06pa3osatt11e A T<ll on11caHHhIM cnoco6oM He CBH3aHO c )J,bTXaTeJThHOH uenhJO, 11 ero Ha3bIBalOT . cy6cTpambTM Q:>ocQ:>op111111posatt11eM A.Ilef>. 06pa30BaHHbTH 3-Q:>ocQ:>orn11uepaT y)l(e He conep)l(HT MaKpo3pmqecKoti: CBH3H. B c11eny10w11x peaKUHHX npo11cxonHT BHYTPI1MOJTeKy1rnpHbte nepecTpoti:KH, CMbICJI KOTOpbIX CBO,Il,HTCH K TOMy, lJTO Hl13K03Hep-

----t--1

H-C-OH

I

CH20Pol-

r111.11.1epa11b,qer1.1,q-3-Q:>ocQ:>aT

r111.11.1epanb,Qer1.1p,- cpoccpaT,qer1.1,qporeHa3a

1 c-oPo/-:

1-t--------

H-C-OH

I

CH20POl-

1,3-61.1ccpoccporn1.11.1epaT

AA<I> ~ <1>occporn1.11.1epaTKi.1Ha3a

ATC!>

0

8-o-

I

H-C-OH

1

CH20P032-

3-<l>occt>orn1.11..1epaT

PMC. 7-36. npeepaJ.l.leHMe rn1o14epanbAerMA-3-cpoccpaTa B 3-ctioccporn1o14epaT.

renF1ecKttti <t>oc<t>o3qmp nepexo,lIMT B coe,lI11ttett11e, co,liep)Karn:ee BhICOK03HepreT11qecK11ti <t>oc<t>aT. BHyrp11MoJie1<ym1ptthre npeo6pa3oBaHHH JaKJIIOqaIOTC5:l B nepettoce <t>oc<t>aTttoro ocrnTKa 113 noJIO)KeHMH 3 B <t>oc<t>ormn.1,epaTe B noJio)KeHMe 2. 3aTeM OT o6pa3oBam.uerocH 2-<t>oc<t>orn11ueparn oTmenAAeTcH MOJie1<yJia BO,lihI np11 yqacTMM <t>epMeHTa eHoJia3bI. Ha3BaHMe ,liefM).l,paTttpyiorn:ero <t>epMeHTa ,liaHo no o6paTHoti peaKUMM. B peJyJihTaTe peaKLI,HM o6paJyeTCH 3aMern:eHHhlH eHOJI - <t>oc- <t>oeHOJIIU1pyBaT. 06pa3oBaHHhiii <t>oc<PoeHoJin11PYBaT - MaKP03prlfqeCKoe coe,lIMHeHMe, <t>oc<t>aTHaH rpynna KOToporo nepeHOCMTCH B CJie,liyiorn;eii peaKU,MH Ha A)],<l> npM yqacTMM n11pyBaTKMHa3hl (<t>epMeHT TaK)Ke HaJBaH no o6paTHoti: peam1111, B KOTOpoii npoMCXO,lIMT <t>oc<t>opMJIMPOBaHHe nnpyBaTa, XOTH nO,lI06HaH peaKLI,MjJ B TaKOM BM).l,e He HMeeT MeCTa).

IlpeBparn:eHMe <t>oc<t>oeHoJin11pysarn B n11pyBaT - Heo6paTMMa51 peaKuHj{. 3To BTopaj{ B xo.n:e fJIMKOJIM3a peaKIJ,M51 cy6cTpaTHOfO <t>oc<flopMJIM-

poBaHHH. 06pa3yiorn:a5ICH eHOJihHaH <t>opMa nMpyBaTa JaTeM He<t>epMeHTaTHBHO nepexomfT B 60Jiee TepMO,lIMHaM11qecKH CTa6HJihHYIO KeTo<t>opMy. On11caHHa5I cep11H peaKu11ti rrpe,lICTaBJieHa tta p11c. 7-37.

0

II

C-0-

1

H-C-OH

I

CH20Pol-

3-<l>occporm1L.lepaT

I<l>occporm11.-1epaTMYTa3a

0

II

c-o-

1

H-C-OPol·

I

CH20H 2-<l>occpom1114epaT

TEHonaaa

H20 4-"l

0

II

c-o-

···+------·

! C-dP032-

: II :

tcr12!

<l>occpoeHonn111pyeaT

A,Q<I> ~

n111pyB8TKlllH838

AT<!>

0

II

c-o-

1

C=O

I

CH3

n111oveaT

PMC. 7-37. npeepaJ.1.1eHMe 3-cpoccpornM4epaTa B nMpyeaT.

CxeMa 10 peaKu,11ti, npoTeKaiorn:11x np11 a3po6HOM fJIMKOJIM3e, M )J;aJihHetiwee OKHCJieHMe nMpyBaTa npe,lICTaBJieHhI Ha p11c. 7-33.

3. 0KuCAenue u,umon.n03Mamu'lecJCow NADH

8 Mumoxoni)pullllbnoii i1b1Xame.t1bnoii u,enu. l/e.;ino'lnbte cucme.Mbt

NADH, o6paJyioIIJ,HticH np11 OKMCJieH1111 rn11uep- aJib,lierM).l,-3-<t>oc<t>arn B a3po6HOM rJIMKOJIH3e, no,lIBepraeTC5:l OKHCJieHMIO nYTeM nepettoca aTOMOB BO- ,lIOpO,lia B MHTOXOH,lipMaJihHYIO ,lihIXaTeJihHYIO uenh. 0.n:ttaKo UHT030JihHhIH NADH He cnoco6eH nepenaBaTh BO,ZIOPO,lI Ha ,llhlXaTeJihHYIO uenh, nOTOMy '-ITO

MlffOXOI-LUPllaJihHasI MeM6paHa ,l.l.JUI Hero HenpOHlluaeMa. IlepeHoc Bonopona qepe3 MeM6patty npo11cxon11T c nOMOIUhIO cneUlla.TlbHhIX CllCTeM, Ha3blBaeMbIX «qe;rnoqHhIMll». B 3THX c11cTeMax BOLJ:OPOLJ: TpaHcnopT11pyeTCH qepe3 MeM6patty np11 yqacT1111 nap cy6cTpaTOB, CBH3aHHhlX COOTBeTCTBYJOIU11M11 ne111LJ:p0reHa3aM11, T.e. c o6eHX CTOPOH MHTOXOI-LUPJI- a.JlbHOH MeM6paHhI Haxonmrn cneumtrnqecKasi: ner11nporeHa3a. H3BecTHhI 2 qe;rnoqHhie c11cTeMhI. B nepBoil: m 3THX cJicTeM BOLJ:opon OT NADH B umo3oJie nepenafarn Ha n11rHLJ:poKc11aueToH<Poc- <PaT <PepMeHTOM rn11uepoJI-3-<Poc<PaTLJ:eIBLJ:poreHa- 3oi1: (NAD-3aB11CJIMblH <PepMeHT, Ha3BaH no 06paTHOH peaK11,11JI). 06pa30BaHHhIH B xone 3TOH peaKll,JIJI rnJiuepoJI-3-<Poc<PaT, OKJICJIHeTcH LJ:aJiee <PepMeHTOM'BHyTpeHHeil: MeM6paHhl MJITOXOHJlPJIH - rnJiuepOJI-3-<Poc<PaTLJ:erHLJ:poreHa3oi1: (FAD- 3aB11ClIMhIM <PepMeHTOM). 3aTeM npoTOHhl 11 3JieKTPOHbl c FADH2 nepexoLJ:ITT Ha y6JIXHHOH 11 LJ:aJiee no UI13 (pJic. 7-38).

f JI11Uepon<Poc<PmHasi: qeJIHoqHasi: CJICTeMa pa6oTaeT B KJieTKax 6eJlbIX MhIUIU 11 renaTOUMTOB. OLJ:HaKO B KJleTKax cepneqHblX MblIUll, MJITOXOHLJ:PJiaJihHaH rJI11UepoJI-3-<Poc<PmneIBLJ:poreHaJa OTCYTCTBYeT. BropaH qeJIHoqHaH CJICTeMa, B KOToport yqaCTBYIOT Ma.JiaT, 11,11T030JlhHaH 11 MJITOXOH,Uplla.TlbHasI Ma.TiaTLJ:erHLJ:poreHa3hl, HBJIHeTCH 6onee ytt11BepcaJihHOi1:. B umoIIJia3Me NADH BOCCTaHaBJI11BaeT oKcanoauernT B MaJiaT (pJIC. 7-39, peaKll,JIH 1), KOTOpblH npJI yqacTJill nepeHocq11Ka npOXOLJ:JIT B MJITOXOH,U- PJI11, rne oKMcAAeTcH B oKcanoauernT NAD-3aBJI- c11Moi1: Ma.Jl:aTnemnporeHa3oi1: (peaKu11H 2). BoccrnHoBJieHHhIH B xone 3TOH peaK11,1111 NAD OTLJ:afa Bonopon B M11ToxoH,UpJiaJibHYJO UI13. OnHaKo o6pa3oBaHHhIH 113 MaJiaTa OKCaJioaueTaT BbIHTJI caMOCTOHTeJibHO 113 M11TOXOH,Up11H B U11T030Jlh He MO- )!(eT, TaK KaK MeM6paHa MllTOXOHLJ:PJIH LJ:JIH Hero HenpoHMu;aeMa. Ilo3TOMY oKcanoaueTaT npeBpawa- eTcH B acnapTaT, KOTOpblH 11 TpaHcnopT11pyeTC5I B Ul1T030Jih, rne CHOBa npeBpawaeTcH B oKcanoaueTaT. IlpeBpaweHJIH oKcanoauernrn B acnaprnT JI 06paTHO CBH3aHbl c np11coen11HeH11eM 11 OTIUeIIJieHlleM aMllHOrpynTihl (peaKU11JI TpaHcaM11H11pOBaHMH, CM. pa3)leJI 9). 3Ta qeJIHOqHasi: CJICTeMa Ha3bIBaeT- CH MaJiaT-acnapTaTHOH (p11c. 7-39). Pe3yJihTaT ee pa6oThI - pereHepauJIH U:JITOnJia3MaTJiqecKoro NAO+ JI3 NADH.

06e qeJIHOqHbie CJICTeMhI CYIUecTBeHHO OTJIHqaIOTCH no KOJI11qecrny CJIHTe3JipoBaHHoro A T<D. B nepBOH CllCTeMe COOTHOIIIeHJie p/0 paBHO 2, TaK KaK BOLJ:opon BBOLJ:HTCH B UI13 Ha ypoBHe KoQ.

BropaH c11cTeMa 3HepreT11'lecKH6oJiee 3<P<PeKT11BHa, TaK KaK nepenafa BOLJ:OPOLJ: B UI13 'lepe3M11TOxoH,n:pJiaJihHhii1 NAO+ JI cooTHOllleHJie P/O 6JI113KO K 3.

4. Ell.llanc AT<P npu a3po6noM 2.llUKOAU3e u pacnaiJe e.AIOK03bl iJo col u H]O

Bwxo,n: AT<I> npn a3po6HoM rJIHKOJIHJe

Ha 06pa30BaH11e <l>PYKT03o- l ,6-6Jic<Poc<Parn m OLJ:HOH MOJieKYJibI rJIIOK03hl Tpe6yeTC51 2 MOJieKYJibl AT<D (peaKu1111 I 11 3 Ha p11c. 7-33). PeaKUJI11, CBHJaHHhie c CJIHTe30M AT<D, npoJicXbLJ:HT nocJie pacnana rJIIOK03hI Ha 2 MoJieKYJibI <Poc<l>OTPJI03hI, T.e. Ha BTOpoM 3Tane rn11KOJI113a. Ha 3TOM 3Tane npo11cxoLJ:HT 2 peaKUMH cy6cTpaTHoro <Poc<PopHJIHpoBaHMH JI CJIHTe3JIPYJOTCH 2 MOJieKyJib1 AT<D (peaKUMH 7 11 10). KpoMe Toro, OLJ:Ha MOJieKYJla rJI11UepaJibLJ:ernJl-3-<Poc- <Parn ne111LJ:p11pyeTcH (peaKUttH 6), a NAOH nepenafa BOLJ:opon B MMTOXOI-LUPl1aJihHYJO UI13, me c11me3JipyeTcH 3 MoneKYJihI AT<D nYTeM oK11CJIJITeJibHoro <Poc<PopHJ111posaHJI5I. B naHHOM CJIY'faeKOJil1'-IecTBo AT<D (3 HJ111 2) 3aB11cm OT THIIa qeJlHOqttOH CJICTeMhI. CnenoBaTeJlbHO, oKMcJieHMe no TIHpyBarn OLJ:HOH MOJieKYJlbI rJlJIUepaJib)lerHLJ:-3-<Poc<Parn conpH)!(eHo c cHHTe3oM 5 MOJieKYJl AT<D. Y'-IlIThIBaH,qro 113 rmoK03hI o6pa3YJOTC51 2 MOJieKYJlbl <Poc<l>oTp1103bl, TIOJIYqeHttyIO Be.JIWfJIH)'tty)!(HO YMHO)!(JITh Ha 2 JI 3aTeM Bh1qecTu 2 MoneKYJibI AT<D, 3aTpalfeHHhie Ha nepooM 3Tane. TaKttM o6pa30M, BhIXOLJ: AT<D np11 a3po6HoM rn11KOJI113e cocTaBAAeT (5x2) - 2 = 8 AT<D.

BblX0,11, AT<I> npn a3po6HOM pacna,IJ,e rJIIOK03bl ,11,0 KOHeqffblX np0,11,yKTOB

B pe3yJihTaTe rJIJIKOJ1JI3a o6paJyeTcH n11pYBaT, KOTOphIH nanee OKl1CJI5IeTC51 LJ:O co2 11 H20 B OIIK, on11caHHOM B pa3JleJie 6. Teneph MO)!(HO 011,eHJITh 3HepreT11qecKyIO 3cJ:>¢eKT1IBHOCTb rJI11KOJll13a JI OIIK, KOTOph1e BMecTe coCTaBJI5IIOT npouecc a3po6Horo pacnana rJIIOK03hl LJ:O KOHe'-IHhIXnpOLJ:YKTOB (Ta6Jl. 7-4).

TaKMM o6pa3oM, BhIXOLJ: AT<D np11 oK11cJieH11JI I MOJlb rJIIOK03bl LJ:O co2 11 H20 COCTaBJI5IeT 38 MOJlh AT<D.

B npouecce a3po6Horo pacnana rJIIOK03hI npo11cxoMT 6 peaK11,11i1: nerMLJ:p11poBaHAA. 0LJ:Ha 113 H11x npOTeKaeT B fJIJIKOJime 11 5 B OIIK (cM. pa3LJ:eJI 6). Cy6cTpaThI LJ:JIH cneu11<P11qecK11x NAD-3aB11c11MhIX nemnporeHa3: rnJiuepaJihLJ:erMLJ:-3-<Poc<PaT, n11pyBaT, JI30UJITpaT, a-KeTornyrnpaT, MaJiaT. OnHa peaKUAA nerJILJ:PJIPOBaHJrn B u1npaTHOM 11,11KJie non

LJ,ettCTBHeM cyKUHHaT,11,erHL1,poreHa3hl rrpOHCXOLl,HT

c yqacnieM Ko<PepMeHTa FAD. 06mee KOJrnqecrno

AT<l>, cttttTe3upoBaHttoe nYTeM oK11c1111TeJibttoro <Po<Pop111rnp0Bamrn:, cocTaBJrneT 17 MOJib AT<l> Ha 1 MOJib rn11uepanh)].erHLJ,<Poc<Parn. K 3TOMY Heo6xonn:Mo rrptt6aBttTh 3 MOJib AT<l>, cttttTe311poBaHHhIX rryreM cy6cTPaTHOro <Poc<t>opllJIHpOBaHIHl (LJ,Be peaKUHH B rJIHKOJil13e l1 OLJ,Ha B UHTpaTHOM UHKJie).

YqttThrnasi, qro rnJOK03a pacrraLJ,aeTcSI Ha 2 <Poc- <PoTPtt03hI l1 qTo CTeXHOMeTPHqec:KHi1 K03cPcPHUl1eHT LJ,aJihttei1urnx npeBpamettuil paBett 2, nonyqeH-

HYIO BeJittqlfHY HaLJ,O YMHOX<HTh tta 2, a H3 pe3yJihTaTa Bh1qecTh 2 MOJih AT<l>, MCITOJih30BaHHhie Ha nepBOM 3Tarre rJIMKOJIH3a.

B. AHA3P06HhIH PACIIA,11. fJIIOK03hl

(AHA3P06HhIH fJIHKOJIH3)

AHa3po6HhIM rnMKOJitt30M Ha3hIBaIOT rrpouecc pacmerrneHMSI rn10K03hI c o6pa3oBaHHeM B Kaqecrne Kotteqttoro rrpOJ1.YKTa JiaKTaTa. 3TOT npouecc rrpoTeKaeT 6e3 HCITOJih30BaHMSl KHCJIOpOLJ,a If rro3TOMY He 3aBMCMT OT pa60Thl MHTOXOHLl,pMaJihHOM LJ,hIXaTeJihHort uerrn. AT<l> 06pa3yeTrn 3a cqfa peaKUMil cy6cTpaTHoro <Poc<PopMm1pOBaHMSI. CyMMapttoe ypaBHeHMe rrpouecca:

C 6H 1206 + 2 H 3P04 + 2 NJ:<l> = 2 C 3H 60 3 +

2 AT<l> + 2 Hp.

I. Pea1ai.uu aH03po6now lAUKOAU3a

IlpH aHa3po6HOM rJIHKOJIM3e (pttc. 7-40) B UlfT030Jie rrpoTeKatOT BCe ] 0 peaKUHM' H)].eHTHqHblX a3po6HOMY rJIHKOJIM3y. JlHillh ] 1-SI peaKuHSI, rne

rrpOHCXO)],HT BOCCTaHOBJiem1e nupyBaTa UHT030JlhHhlM NADH, SIBJUieTCSI crreuM<PnqecKoi1 J1.JISI atta3po6ttoro rnHKOJIH3a (p11c. 7-41). BoccrnttoBJietttte IlHPYBaTa B JiaKTaT KaTaJil13ttpyeT JiaKTaTLJ,erM)].p0reHa3a (peaKUMSI o6paTHMaSI, If <PepMeHT Ha3BaH ITO o6paTHOll peaKuMM). c IlOMOlllhlO 3TOM peaKUMH o6ecrreqMBaeTcSI perettepau11si NAO+ 113 NADH 6e3 yqaCTMSI MMTOXOHLl,pMaJihHOll )1,hIXaTeJihHOM uerrH B CMzya11,1rnx, CBSI3aHHhIX c He)],OCTaTOqHhlM ctta6X<e- HtteM KJieTOK K11c11opoJ1.0M. PoJih aKuerrrnpa BOJ1.0- po)].a OT NADH (rro)].o6tto KHCJiopoey B LJ,hrxaTeJihttoi1 uerrM) BbITIOJIHSieT rrMpyBaT. TaKHM o6pa3oM, 3HaqeHMe peaKU:HH .BOCCTaHOBJieHHSI TlMpyBaTa 3aKJIJOqaeTCSI He B o6pa30BaHHM JiaKTaTa, a B TOM, qTO )1,attttasi U.HT030Jihttasi peaKuM>I 06ecneq11BaeT perettepau,M10 NAD+. K TOMY X<e JiaKTaT He SIBJISieTcSI KOHeqHhIM rrpOJ1.YKTOM Mern6oJIM3Ma, YJ1.aJISieMhIM lf3 opraHlf3Ma. 3To BellleCTBO BhlBO)],lfTCSl B KpOBh If

YTl1Jllf3lfpyeTC51, rrpeBpamasich B rre'!ett11 B rn10Ko3y, l1Jllf rrpM )1,0CTYITHOCTM KHCJIOpO)],a rrpeBpamaeTCSI B IlMpyBaT' KOTOPhlM BczyrraeT B 06lllMM nyTh KaTa60JIH3Ma, OKHCJI515lCh )1,0 co2 If H20. CTPOeHMe JiaKTaT)].erHLJ,poreHa3hI, MexaHM3M )1,ettCTBHSI l1 3Ha- 'leHlfeonpeneneHMSI aKTIIBHOCTtt 3Toro <PepMeHTa )1,Jl51 )1,MarHOCTHKH 3a6oJieBaHHM OITMChlBaJIMCh paHee B Pa3J1.eJie 2.

BaJiauc AT<I> npu aua3po6noM muKoJiu3e

AHa3po6Hhirt rJIHKOJIM3 rro cpaBHettn:IO c a3po6HhIM Mettee 3<l><PeKTHBeH. B 3TOM rrpouecce KaTa6oJIM3M 1 MOJih rJIIOK03hl 6e3 yttaCTMSI MlfTOXOH)],- pttaJihHOll llhIXaTeJibHOll uerrlf corrpOBO)((Ll,aeTCSI CMHTe3oM 2 MOJih AT<l> 11 2 MOJih JiaKTaTa.

06pa3yeTcsi 3a cqeT

rnt0K03a

AT<!l ----j 1 A,Q<!l .--1

I 2

AT<!l ----j 3

A,Q<!l ...-4

<!lpyKTo3o-1,6-61-1ccpoccpaT

I~ ,Q1-1rn,qpoKc1-1a4eT0HocpoccpaT

rn1-14epaJ1b,Qeri.1,Q cpoccpaT

n1-1pysaT ;:===============~==:::::::'.'.:::::!. naKTaT

P1tc. 7-40. AHa3po6Hbrill rmtKon1ta.

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f. JHAqEHME KATABOJIM3MA fJIIOK03hl

OcttoBttoe c)l11311onorttqecKoe Ha3tta'letttteKarn6ontt3Ma fJIIOK03bl 3aKJIIO'IaeTC51B HCilOJib30BaHHH 3tteprttn:, ocB060)1()1aIOmeitc51 B 3TOM npouecce )1Jl5I

CttHTe3a AT<l>.

3Hepr11H, BbI,UeIDIIOIUMC51 B rrpouecce rroJittoro pacna):(a fJIIOK03bl )10 co2 H H20, COCTaBJI51eT

2880 K)J,)l(/MoJih. EcJitt 3Ty BeJitt'IHHY cpaBHMTh c 3Hepnreii rtt)JpOJitt3a BbICOK03HepreTtttieCKHX CBH-

TIOJIY'IttM:50x38 = 1900 K)J,)I(, 'ITOcocrnrurneT 65%

OT BCeM 3Hepnm, BbI,UeIDIIOlll,eHC51 nptt IIOJIHOM pacnan:e rJIIOK03hI. T aKOBa 3c)lc)leKTttBHOCTh ttcrroJih30BaHIDI 3Hepnm pacrra,n:a rJIIOK03hI M5I cttHTe3a AT<l>. Heo6xon:ttMO yqttTbIBaTb, 'ITOpeanbttM 3<Jlc)leKTttBHOCTh npouecca MO)l(eT 6bITh Hlf)l(e. To'IHOoueHttTh BhIXO!J AT<l> MO)l(HO TOJibKO nptt cy6cTPaTttOM <Pocc)lopMJittpOBaHHH, a COOTHOWeHtte Me)l()Jy nocryrnreHtteM BO!JOPO)Ja B )JblXaTeJibHyIO uenh H CHHTe30M AT<l> 51BJrneTc51 nptt6Jitt3ttTeJibHbIM.

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KpoMe 3HepreTtt'lecKoi1c)lyttKUtttt, rrpouecc Karn6oJitt3Ma fJIIOK03bl MO)l(eT BhIITOJIH51Tb H aHa6oJIH'leCKtte<PYttKUtttt. MeTa6oJittThI rnttKOJitt3a ttcITOJih3YJOTCH )JJIH cHttTe3a HOBhIX coe)JttHeHtti1. TaK,

cppyKToJo-6-cpoccpaT 11 nrnuepa.rrh,aenm-3-cpoccpaT yqaCTBYJOT B o6pa30BaHHH ptt6030-5-cpoccpaTa - CTPYKTYPHOro KOMilOHeHTa HYKJieon1,aoB; 3-cpoc- cponrnuepaT MO)KeT BKJIIO'laTbC51 B Cl1HTe3 aMHHOKHCJIOT' TaKHX KaK cepHH, fJIHUHH, UHCTeHH (cM. paJ,aeJI 9). B neqeHH H )KHPOBOH TKaHM aue- TMJI-KoA, o6pa3y:IOIUMHC51 M3 nttpYBaTa, ttcnoJihJyeTcH KaK cy6cTpaT npM 6MOCMHTe3e )l<HpHbIX KMCJIOT, XOJieCTepMHa, a llMfMllPOKCMaueTOHcpoccpaT KaK cy6cTpaT MH cttHTeJa rnttuepon-3-cpoccparn (cM. paJ,aen 8).

,II,. PEfYJUIUIHl KATABOJU13MA fJIIOK03hl

IlocKOJihKY OCHOBHOe 3Ha'leHMefJIMKOJIM3a coCTOHT B CHHTeJe AT<l>, ero cKopocTh ,UOJI)l(Ha Koppem1ponaTh c JaTpaTaMM 3Heprtttt B opraHMJMe.

BoJihlllMHCTBO peaKUMH rJIMKOJIMJa o6paTMMhI, Ja MCKJIIO'leHMeMTpex, KaTaJIM3MpyeMbIX reKCOKHHa30H (MJIM fJIIOKOKHHaJOH), cpoccpocppyicTOKMHa30H H nttpYBaTKMHaJotf. PeryAATOPHhie cpaKTophI, H3MeH51IOIUMe CKOpOCTb fJIHKOJIHJa, a 3Ha'IMTM o6pa30BaHHe AT<l>, HanpaBJieHhI Ha Heo6paTMMhie pe- aKUHH. IloKaJaTeJieM noTpe6JieHM51 AT<l> 51BJI5leTC51 HaKoilJieHMe A,IJ,<l> 11 AM<l>. Ilocne,aHMH 06pa3yeTc51 B peaKUHM, KaTaJIM3MpyeMOH a,aeHMJiaTKMHa3oi1: 2 A,IJ,<l> H AM<l> + AT<l>

,1],a_)i(e tte6oJibmoi1 pacxo,a AT<l> Be,aeT K JaMeTHo-

MY YBeJIH'leHMIOAM<l>. OrnorneHMe ypoBHH AT<l> K A,IJ,<l> M AM<l> xapaKTepMJyeT 3HepreTM'lecKHi1crnTYC KJieTKH (cM. paJ,aeJI 6), a ero cocTaBJIHIOIUHe CJIY)KaT aJIJIOCTepM'leCKHMHperyAATOpaMM CKOpocTH KaK o6iuero nyTM KaTa60JIM3Ma, TaK M rJIMKOJIM3a. Ha pttcyttKe 7-42 noKaJaHa aJIJIOCTeptt'lecKaH peryAAUM51 CKOPOCTH KaTa60Jili3Ma fJIIOK03hI B CKeJieTHbIX MhIUIUax.

Cyiuecrnettttoe JHa'lem1ellJl5l peryAAUHM rJIMKOJIMJa MMeeT M3MeHeHHe aKTMBHOCTM cpoccpocppyicTOKHHa3hI, IlOTOMY 'ITO3TOT cpepMeHT, KaK yrTOMMHaJIOCb pattee, KaTaJIH3ttpyeT Hatt6onee Me,UJieHHYJO peaKUHIO npouecca.

<l>occpocppYJCTOKHHaJa aKTHBttpyeTcH AM<l>, HO HHrn6ttpyeTCH AT<l>. AM<l>, CB513hIBAACh c aJIJiocTepM'leCKMMueHTpOM cpoccpocppYJCTOKHHaJhI, YBeJIM- 'lHBaeTcpO,UCTBO. cpepMeHTa K <l>PYJCT030-6-cpoc<PaTY l1 IlOBhilIIaeT CKOPOCTh ero .cpoccpopHJIMPOBaHH51. 3<Pcf:>eKT AT<l> Ha 3TOT cpepMeHT - npHMep roMOTporrnoro aJIJIOCTepmMa (cM. PaJlleJI 2), nocKOJihKy A T<l> MO)[(eT BJaHMo,aeiicTBoBaTu KaK c aJIJiocrepttqecKHM, TaK M c aKTHBHbIM ueHTpOM, B IlOCJie,UHeM cnyqae KaK cy6cTpaT.

Ilptt cptt3MOJIOrM':IeCKHXJtta'!emrnxAT<l> aKTHBHhIH ueHTp cpoccpocppyKTOKHHa3bI BCeri.i:a HaChIIUeH cy6cTpaTaMH (B TOM 'lHCJie AT<l>). IlOBhirnetttte ypoBHH A T<l> OTHOCHTeJihHO A,I1,<l> Cttll)[(aeT cKopocTb peaKUHH, nocKOJihKY AT<l> B 3THX ycnoBH51X ,aetfcrnyeT KaK MHfl16MTOp: CB513bIBaeTC51 c aJIJIOCTepM'leCKHMueHTpOM cpepMeHTa, BhI3bIBaeT KOttcpopMaI.l,MOHHbie M3MeHeHI151 l1 yMeHhrnaeT CpO)lCTBO K ero cy6cTpaTaM.

113MeHeHMe aKTMBHOCTM cpoccpocppyicTOKl1Ha3hI cnoco6crnyeT peryAAUHM CKOPOCTH cpoccpopHJil1p0BaHM51 rJIIOKOJhl reKCOKMHaJoti:. Cttll)[(eHtte aKTMBHOCTH cpoccpocppyKTOKHHa3hl IIPM BbICOKOM ypOBHe AT<l> Be,aeT K ttaKoilJiettMIO KaK cppyKTOJo-6-cpoc- <Parn, TaK M fJIIOK030-6-cpoccjJaTa, a IlOCJie,UHMH ttttrtt6ttpyeT reKCOKMHaJy. Cne,ayeT HanoMHMTh, 'ITO reKCOKMHa3a BO MH0fl1X TKaH51X (3a MCKJIIO'leHMeM ne'leHMM ~-KJieTOK IlO,U)KeJIY,UO'lHOH)[(eJie3hI) l1Hm611pyeTC5l rJIIOK030-6-cpoccpaTOM.

Ilptt BhlCOKOM ypoBHe AT<l> CHM)[(aeTcH cKopocTh UMKJia JIMMOHHOH KHCJIOTbl l1 llbIXaTeJihHOH uenM. B 3TMX ycnoBH51X npou,ecc rnttKOJIMJa TaK)[(e JaMe,a- AAeTcH. Cne,ayeT ttanoMHHTh, 'ITOaJIJIOCTepwqecKAA peryAAUMH cpepMeHTOB OTIK 11 llhIXaTeJihHOH uen11 TaK)[(e CB513aHa c l13MeHeHtteM KOHUeHTpauHH TaKHX KJIIO'leBh1xnpo,ayKTOB, KaK NADH, ATCI> tt

HeKOTOPhIX Mern6oJIMTOB. TaK, NADH, HaKailJittBAACh B TOM CJiyqae, eCJIM He ycneBaeT OKHCJIHThCH B llhIXaTeJihHOH uenH, l1HrH6ttpyeT HeKOTOpbie aJI-

JIOCTepM'leCKMecpepMeHTbl UHTpaTHOfO UHKJia (CM. paJ,JJ,eJI 6).

<l>M3HOJIOfH'leCKaHpOJib fJIHKOJIH3a B ne'leHHIi

)KHPOBOH TKaHH HeCKOJihKO HHaH, 'leMB llPYfHX TKaHHx. B neqeHH 11 )[(l1pOBOH TKaHM rnMKOJIH3 B nepHO)l IlMIUeBapeHHH <PYHKUHOHHpyeT B OCHOBHOM KaK MCTO'IHHKcy6cTpaTOB )J,JI5l Cl1HTe3a )[(l1poB. PeryAAUHH rnttKOJIHJa B ne'leHHHMeeT cnovr oco6eHHOCTl1 Ii 6y)leT pacCMOTpeHa IIOJ)[(e.

E. 2,3-B11.ot>oot>orn1111,EPATHhliI UMK.JI

B rnvrKOJIHTH'leCKOMnyTH MO)l(eT npoTeKaTh i.i:o- noJIHHTeJihHaH peaKUHH, KaTaJIH3HpyeMAA 611ccpoccpornttuepaTMyTa30H, npeBpammoiueif l,3-6ttccpoc- cpornttuepaT B 2,3-611ccpoccpornvruepaT (2,3-B<l>f), KOTOpbIM MO)l(eT npH yqacTHM 2,3-611ccpoccporn11- uepaTcpoccpaTa3bl npeBpamaThCH B 3-cpoccporn11ue- paT - MeTa60JIMT rJIMKOJIH3a (pHC. 7-43).

B 6oJihIIIHHcrne TKaHei1 2,3-E<l>f 06pa3yeTrn B He6oJihIIIHX KOJIH'leCTBax. B 3pHTpoui..nax 3TOT MeTa60JIHT 06pa3yeTCH B 3Ha'lt1TeJibHbIXKOJIH'le-

CTBax M BbUTOJIIDieT pOJib a.JIJIOCTep11'-ieCKOrOpery- lliITOpa QJyHKL(MM reMorno6MHa. 2,3-fi<l>r, CBH3bIBaHCb c reMOrJI06MHOM, TIOHIDKaeT era CPOACTBO K KMCJIOPOAY'cnoco6crnyeT AMCCOL(MaUMM KMCJIOPOAa M nepeXOAY era B TKaHM (CM. pa3AeJI 1).

06pa30Bam1e 2,3-E<l>f npeAnOJiaraeT n0Tep10 :meprMM MaKp03pmqecKOH CBH3M B 1,3-6wc- <Poc<Porn11uepaTe, KOTOpaH He nepeHOCMTCH Ha

AT<I>, a paccewBaeTcH B <jlopMe TeTIJIOTbI, qTo 03HaqaeT CH11)1(eH11e 3HepreTwqecKoro 3cPcPeKTa rm1KOJIM3a.

IX. CJllHTE3 rntOK03bl

B nE'"IEHJllcrntOKOHEOrEHE3)

HeKOTOpble TKaHM, HanpwMep M03r, ffY)l(AalOTCH B TIOCTOHHHOM TIOCTYTIJieHMM rJIIOK03bI. Kor;::i:a noCTYTIJieHMe yrneBO)l;OB B COCTaBe illfiUl1 He)l;OCTaTOqHO, co;::i:ep)l(aHMe rJilOK03bI B KPOBM HeKOTOpoe BpeMH TIOMep)!(l1BaeTCH B npe;::i:eJiaX HOPMbI 3a cqer pacureTIJieHMH rnwKoreHa B neqeH11. 0AHaKo 3anaCbI rn11KoreHa B neqeHM HeBeJIMKM. OHM 3HaYMTeJibHO )'MeHblllalOTCHK 6-10 l{ roJIO)l;aHMH M npaKTM-