Добавил:
Upload Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:

підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

.pdf
Скачиваний:
32
Добавлен:
25.02.2016
Размер:
44.13 Mб
Скачать

Tep11030M 11 HapyweHHHMH WH3HqecKoro pa3BHTH5I pe6eHKa.

IlpH06pcreHHD1e ¢opMh1 rraroJTornH Moryr Ha6moAaTbC5J rrpH KHrneqHhIX 3a60JTeBaHHHx, HarrpHMep raCTPHTax, KOJTHTax, 3HrepHTax. CJTe;:iyeT 3aMeTHTh, '!TO B 3THX CJTyqa5IX oco6eHHO 3aMeTHO CHIDKeHHe aKTHBHOCTH JTaKTa3hI. KaK )')KeroBopHJTocb, aKTHBHOCTh JTaKTa3hT B KHrneqHHKe HIDKe, qeM APYTHX )l,11CaxapH)J,a3, IT03TOMY YMeHhllleHHe ee aKTHBHOCTH CTaHOBHTCH 3aMeTHbIM ,[I)IH opraHl13Ma B rrepBYJO oqepe)l,h.

,ll,e<!)HUMT JTaKTa3bl y B3pOCJTblX JTIO)J.etl: MO)!(eT 11MeTh H D,pyryIO rrp11q11Hy. Bo3MO)!(HO CHIDKett11e 3KCrrpeCCHH reHa JTaKTa3bT B03paCTHOfO xapaKTepa. Y)!(e YTIOMHHaJTOCb, qTo aKTHBHOCTb JTaKTa3hI y B3pOCJTblX JTIOD,etl: B HOpMe 3Haq1neJTbHO HH)!(e, qeM y D,eTetl:. Ilo3TOMY CHIDKeHHe aKTHBHOCTH JTaKTa3bl OTHOCHTeJTbHO )')KeHMe101uerOCH HH3K0f0 ypoBHH y OT)l,eJTbHbIX JllO)l,eM MO)!(eT rrpOHBJTHTbCH HerrepeHOCHMOCTbIO MOJTOKa. HocHTeJTHMH rraToJTorH11, Cml3aHHOM c ,ne<lJttUHTOM JTaKTa3bT, HBJTHIOTCH <Jame Bcero Jrnua a<lJpHKaHCKOfO 11 a3HaTCKOfO rrpOHCXO)!(,Uemrn. Cpe,UHHH qacroTa ,nattttoif ¢opMbI rraTOJTorHH B CTpaHax EBpOITbl COCTaBJTHeT 7-12%, B K1nae - 80%, B oT,neJThHbIX patl:oHax A¢p11KH - ,no 97%. Ilo,no6Hhie Ha6JTIO,UeHHH pacrrpocTpatteHHH JTaKTa3HOM He,UOCTaTOqHOCTH CBH3bIBalOT c HCTOpHqeCKH CJTO)!(HBlllHMCH paUHOHOM Ill1TaHJ1H H

Ta6m11..1a 7-2. HapyweHMSI nepesapMBaHMSI AMCaxapMAOB

OTCYTCTBHeM MOJT04H0f0 CKOTOBO)J,CTBa B YIIOMH-

HYTblX pernottax. IlpHMephI 11 rrpw111tth1 ttapywe-

HHH rrepeBap11BaHHJI )J,HCaxapH)J,OB rrepe4HCJTeHbl B

Ta6JT. 7-2.

Cywecrny10T pell,Ktte ¢opMhI ttapyweHHR nepeBapHBaHHH yrneBO)lOB. Hanp11Mep, 111BeCTHa ttaCJTe)l- crnettHaH Hell,OCTaTOqHOCTh TperaJTa3hl, KOTOpaH rrpoJIBJTReTCH )lHCrrerrc11etl: nocJTe yrroTPe6JTemrn rptt6oB, coll,ep)[(awHX Tperan03y.

B OT1leJThHhIX cJTyqaRx MaJTba6cop6uHH MO)!(eT 6hITh BhI3Batta HeCKOJThKHMH rrpH411HaMH. Harrp11Mep, rrocJTe onepawm Ha )!(eJTyAKe B03MO)!(Hbl yxyAwettHe CMelllHBaHHH IIl1W11 c rr11weBapHTeJThHblMH COKaMH, CHl1)[(eHHe HX ceKpeU,HH, ycKopeH11e rrpoxo)!(,UeHHH IIl1Wl1 qepe3 KHllle4HHK, KOJTOHH3au11H 6aKTepHHMl1 CJTerrotl: H rrpHBOMWetl: neTeJTh.

B. HAPYIIIEHIDI BCAChIBAHIUI

MOHOCAXAPMJJ,OB

Hapywemn1 BcacbrnaHHH MOfYT 6bITh cJTeACTBHeM 1le¢eKTa KaKOfO-JI1160 KOMilOHeHTa (6eJTKa HJTH <l>epMema), yqacrny101uero B cttcTeMe Tpattcnoprn MOHocaxapHAOB qepe3 MeM6patty. OrrHcattbI rraroJTor1111, CBH3aHHbie c Ae<PeKTOM HaTpHM3aBHCHMOro 6eJTKa rrepeHOC4HKa fJllOK03bl.

,ll,JTH )J,11arHOCTl1Kl1 pa3JIH4HhIX ttapyweHHM nepeBap11BaHHR 11CI10Jlb3)'IOTrrpo6bI c Harpy3KOM onpe-

Ilpuquua3a6oneeauus

KnuuuqecKue nposeJ1euus u na6opaTopHh1e AaHHhie

 

 

HacneACTBeHHbIH

BcTPetJ:aeTCJI OTHOCHTeJibHO peAKO. Ilocne npHeMa MOJIOKa

Aeqnni:1u rraKTa3bI

Ha6JIIOL(aIOTCJI pBOTa, L(Hape51, crra3Mbl H 60JIH B )KHBOTe,

 

MeTeopH3M. CttMIITOMbI pa3BHBaIOTCJI cpa3y rrocne pO)KL(eHHH.

HeAOCTaToqHOCTb naKTa3bI

XapaKTepHa AJIH B3pocJibIX H L(eTeii cTapwero B03pacTa. Jis1rneTCJ1

BCJie):\CTBHe CHH)KeHHJI 3KCrrpeCCHH

CJieL(CTBHeM B03paCTHOro CHH)KeHHJI KOJIHqecTBa JiaKTa3bl.

reHa qiepMeHTa B OHToreHe3e

CHMIITOMbl HenepeHOCHMOCTH MOJIOKa aHaJIOrHtJ:Hbl

 

Hacrre;:\CTBeHHOH cl>opMe AeqrnQHTa JiaKT03bl.

HeAOCTaToqHOCTb naKTa3bI

3rn speMeHHaJI, rrptto6peTeHHaJ1 qiopMa. HenepeHocHMOCTb MOJIOKa

BTOpHqHOro xapaKTepa

MO)KeT 6bITb crre;:\CTBHeM KHJIIetJ:HbIX 3a6011esamrii:, HaIIpHMep,

 

KOJIHTOB, raCTpHTOB. KpoMe Toro, speMeHHbIH Aecl>HIIHT naKTa3bI

 

MO)KeT 6bITb CJieL(CTBHeM onepaIIHH Ha )l{KT.

HacneL(CTBeHHM HeL(OCTaToqHoCTb

Ilp051BJIJleTC5J, KOrL(a B paIIHOH L(eTeH A06aBJIJIIOT caxapo3y H

caxapa30-H30MaJibTa3HOro

KpaxMa.rt. oOJibHbie AeTH o6bltJ:HO HeoxoTHO eMT cnaAKOe. Ilocne

KOMITJieKca

Harpy3KH caxap030H OTMetJ:aeTCJI He3HatJ:HTeJibHaJI rHrreprnHKeMHll.

 

)lpyrne caxapa (rnIOK03a, cl>PYKT03a, rraKT03a) rrepeHOCJITCJI

 

xopowo.

Ilptto6peTeHHaJI He;:\OCTaTO'IHOCTb

Mo)KeT B03HHKaTb scneACTBHe KHIIIetJ:HbIX 3a6011esaHHii:.

caxapa30-H30Ma.rtbTa3Horo

Ilp051BJI5JeTC5J L(HCITencHeii:, rrpOBOIIHpyeMOH KpyrraMH, KpaXMaJIOM,

KOMIIJieKCa

a TaK)Ke IIHBOM H ApyrHMH HaITHTKaMH Ha OCHOBe COJIOL(a.

 

 

314

neJieHHhIMH yrneBonaMH. HeaocrnTO'-IHOCThKmue'-1- HhIX .UHCaxap11.ua3 MO>KHO .UHarHOCTHpOBaTh c nOMOlLl,blO BBeneHH51 .uHcaxapH,lla 11 nocJie;::ryIOwero onpeneJieHH51 KOHUeHTpauHH fJIIOK03bl B KPOBH. ,L1,JI51 60JihUieti '-IYBCTBHTeJihHOCTH 3TOT TeCT npOBO.U51T' BBO,WI CHaY.aJia ,llHCaxapH.U (50 r), a 3aTeM 3KBHBaJieHTHOe KOJIH'-!eCTBOCOCTaBJHllOlll,HX ero MOHOcaxapttJJ,OB (no 25 r Ka)K,Il,Oro). CTocJie Harpy3KH KOHUeHTPaUIDl rnJOK03hI B KpOBH yBeJIHY.HBaeTrn npHMepHo Ha 50% OTHOCHTeJibHO HOpMbl. CTpH naTOJIOnrn OTMeY.a!OT He3Ha'-!HTeJihHYIO rnneprnHKeMHIO.

ECJIH TeCT npH Harpy3Ke MOHOCaxapH.UOM conpoBO)K,Il,aeTC51 aneKBaTHhIM nOBhIWeHHeM ero KOHUeH-

TpauHH B KPOBH, a Harpy3Ka ,Il,HCaxapH.UOM He .uaeT HOPMaJihHOH peaKUHH, TO 3TO, CKOpee BCero, yKa3bJBaeT Ha ne<PeKT KHWeY.ttoti m1caxapH.ua3hI, a He CHCTeMbl TpaHcnopTa.

0 HenocTaTOY.HOCTH JiaKTa3hl MO)!(HO cy,LI,HTh, onpe.uemrn BO.UOPOll B Bhlllh!XaeMOM B03.Uyxe (BO.UOpO,LI,HblH TecT). Bo.uopo.u 06pa3yeTCH B pe3yJihTaTe nettCTBH51 6aKTepHaJihHbIX <PepMeHTOB Ha JiaKT03y.

v. META60nVl3M rntOK03bl B KnETKE

CTocJie BCaCbIBaHH51 B KHWe1 IHHKe MOHOCaxapH-

.!lbl nocrynaJOT B BOpOTHYIO Betty H naJiee npeHMYwecrneHHO B rreY.eHb. nocKOJihKY B COCTaBe OCHOBHhlX yrneBO.UOB OHWH npe06JianaeT fJIJOK03a, ee MO)l(HO C'-IHTaTb OCHOBHbIM npo,LI,yKTOM nepeBapHBaHIDl yrneBO.UOB. ,[l,pyrne MOHOcaxapH.!lhl' ITOCTyrraIOwHe H3 KHWe'-IHHKaB npouecce Mern6oJIH3Ma, MOryT npeBpalll,aTbC51 B fJIIOK03y HJTH npO.UYKThI ee MeTa60JIH3Ma. l.JaCTb rJIIOK03b1 B ne'-!em1nenOHH- pyeTC5f B BH,LI,e fJIHKOreHa, a .upyra51 '-laCTb '-lepe3 o6lll,HH KpOBOTOK 1lOCTaBJT51eTC51 H HCnOJib3yeTC51 pa3HhIMH TKaH51MH H oprattaMH. CTpH HopMaJihHOM pauHOHe nHTaHH51 KOHUeHTpauHH fJIJOK03bl B KpOBH no,Ll,1lep)!(HBaeTC51 Ha ypOBHe 3,3-5,5 MMOJTb/JI (60-100 Mr/M). AB nepHOJl nmueBapeHH51 ee KOHueHTpaUH51 MO)!(eT nOBbIWaThC51 npHMepHO ,Ll,O 150 Mf/,LI,JI (8 MMOJib/JI).

A. <l>oc<1>0PHJIHPOBAHHE rnJOK03hI

B .UaJihHeti:wHx npeBpaweHH51X B KJieTKax rnJOK03a H ,LI,pyme MOHOcaxapHLI,bI y'-laCTBYJOT TOJTbKO B BH.Ue cPOCcPOPHhIX 3cPHPOB. <Doc<PopHJIHPOBaHHe CB060L(Hh!X MOHOCaxapHLI,OB - o6513aTeJTbHa51 peaKUH51 Ha OYTH HX HCnOJTb30BaHH51, OHa npHBOL(HT

K o6pa30BaHHIO 6oJTee peaKUHOHHO-cnoco6HbIX coe.u1rneHHH H n03TOMY MO)!(eT paccMaTpHBaTbC51 KaK peaKUH51 aKTHBaUHH.

fJIJOK03a, nocTynaIOlll,a51 B KJieTKH opraHOB H TKaHeti, cpa3y )Ke nonBepraeTC51 cPOCcPOPHJIHpOBaHHIO c HcnoJTh30BaHHeM AT<D. 3Ty peaKUHIO Bo MHOrHX TKaHHX KaTaJIH3HpyeT <PepMeHT reKCOKH-

Ha3a,

a B ne'-!eHHH rroL()!(eJiy,LI,o'-!Hoti)!(eJie3e

- <Pep-

MeHT

fJIIOKOKHHa3a. <Doc<Pop11J1HPOBaHHe

fJIIOK0-

3bl - npaKTH'-leCKHHeo6paTHMa51 peaKUH51, TaK KaK OHa npoTeKaeT c HCITOJTb30BaHHeM 3HaY.HTeJTbHOro KOJTHY.ecrna 3HeprHH. 06pa30BaHHe rJIIOK030-6-

<Poc<Parn B KJTeTKe - cBoeo6pa3tta51 «JTOBywKa» ,LI,JIH rJIIOK03hI, TaK KaK MeM6paHa KJTeTKH HenpOHHuae-

Ma ,Ll,J151 cPOCcPOPHJIHpOBaHHOH fJIIOK03bl (HeT COOTBeTCTBYIOWHX TpattcnopTHbIX 6eJTKOB). KpoMe rnro, cPOCcPOPHJTHpoBaHHe yMeHbWaeT KOHUeHTpauHIO cBo6onHoti rnIOK03hI B u11TonJia3Me. B pe3yJThTaTe c03naIOTCH 6JTaronpH51THhie ycJTOBH51 ,LI,JI51 o6Jier'-leH- Hoii 1ll1cPcPY3HH rJIIOK03hI B KJTeTKH H3 KPOBH.

fJIIOKOKHHa3a. <Doc<PopwrnpoBaHHe fJIIOK03bl B

renaTOUHTax B nepHon rr11wenapeHH5! 06ecrreY.11BaeTC5l CBOHCTBaMH fJIIOKOKHHa3bl, KOTOpa51 HMeeT BbICOKoe 3HaY.eHHe Km - 10 MMOJTb/JI. B 3TOT ne-

pHon KOHUeHTpauH51 fJIIOK03bl B BOPOTHOH BeHe 6oJThwe, Y.eM B .upyrnx omeJTax KpOB51HOro pycJTa 11

MO)!(eT rrpeBbIWaTh I 0 MMOJib/JT, a cJienonaTeJThHO, aKTHBHOCTb fJIIOKOKHHa3bl B rerraTOUHTaX nOBbIWaeTC51. CJTe.uyeT OTMeTHTh, 'lTO aKTHBHOCTh rnIOKOKHHa3hI, B OTJIHY.He OT reKCOKHHa3hT, He HHrH6HpyeTC51 npo,LI,yKTOM KaTaJTH3HpyeMoti peaKUHH - rnIOK03o-6-<Poc<ParnM. 3rn o6cT051.TeJThCTBO o6ecne'lHBaeT nOBbTWeHHe KOHueHTpauHH fJIIOK03bl B KJieTKe B cPOCcPOPHJTHpOBaHHOH <PopMe, COOTBeTCTBeHHO ee YPOBHIO B KPOBH. KaK y)!(e ynoMHHaJIOCh, fJIIOK03a rrpOHHKaeT B renaTOUHTbl nyTeM o6JTerY.eHHOH nHcPcPY3HH rrp11 yqaCTHH TpaHcrropTepa fJllOT-2 (He3aBHCHMOfO OT HHCYJTHHa). fJllOT-2, TaK )!(e, KaK fJIIOKOKHHa3a, HMeeT Bb!COKYJO Km, Y.TO cnoco6cTByeT nOBhIUieHHJO CKOpocTH nocTynJieHH51. rJIIOK03hl B renaTOUHThl B nepHOll OHlll,eBapeHH}[, CJieLI,OBaTeJibHO, YCKOp51eT ee cPOCcPOPHJIHpOBaHHe H L(aJibHetiwee J1COOJib30BaHHe ,Ll,J151 ,LI,enoHHpOBaHH51.

Xorn HHCYJJHH H He BJT115leT Ha TPaHcnopT rnJOK03hI, OH ycHJTHBaeT npHTOK rJIIOK03bI B renaTOUMThI B nep11on IIHWeBapeHH51 KOCBeHHhIM TIYTeM, HHL1.yu11py51 CHHTe3 fJIIOKOKHHa3bl H yCKOp5151 TeM CaMbIM cPOCcPOPHJTHpOBaHHe rJIIOK03hl.

TipeHMylll,eCTBeHHOe TIOTpe6JTeHHe fJTIOK03bl renaTOUHTaMH, o6yCJTOBJTeHHOe CBOHCTBaMH fJTIO-

315

7-21).
>I 07 LI,,
IV).
7-20).
(Km <O, I

KOKl1Ha3hI, rrpe,nornpamaeT qpe3MepHoe rroBhillleH11e ee KOHLI,eHTpaLI,1111 B KpOBH B a6cop6Tl1BHOM rrep110,ne. 3TO, B CBOIO oqepe,nb, CHm1<aeT I10CJie,n- CTBl151 rrpoTeKaHH51 He)l(eJiaTeJibHhTX peaKLI,11H c ycraCTl1eM fJIIOK03hl, Harrp11Mep fJIHK03HJil1POBaHH51 6eJIKOB.

feKCOKHHa3a OTJil1'IaeTC51 OT fJIIOKOKHHa3hl Bhl-

COKHM cpo,ncTBOM K rJIIOK03e MMOJih/JI).

CJie,noBaTeJihHO, 3TOT ¢epMeHT, B orn11crne OT rn10KOKHHa3hI, aKTHBeH rrpM Hl13KOM KOHU,eHTpau1111 rnIDK03hI B KPOBl1, 'ITO xapaKTepHo MH rrocrn6-

cop6TttBHoro COCT051.HH51. necreHh B 3TOT rrepH01l.

rrornomaeT ropa3,no MeHhllle rJIIOK03hI, TaK KaK CKOPOCTh ee BHYTPHKJieTO'IHOro¢oc¢opMJI11POBaHH51 rn10KoK11Ha3oi1 pe3KO CHmKaeTCH. Tor,na KaK I10Tpe6JieHtte fJIIOK03hl M03fOM, 3P11TpOLI,11TaMl1 11 1l.PYrHMl1 TKaH51.Ml1 o6ecrre'll1BaeTC51 aKTl1BHOM B 3THX ycJIOBH51.X reKCOKHHa30tt. <l>epMeHT reKCOKMHa3a MO)l(eT KaTaJil13HPOBaTh ¢oc¢opMJIHpOBaH11e He TOJihKO D-fJIIOK03hI, HO J1 ,npyrnx reKC03, XOT51 11 c MeHhWeH CKOPOCThlO. AKTMBHOCTh reKCOKHHa3bl H3MemieTC51 B 3aBHCHMOCTl1 OT rroTpe6HOCTeM K1leTKM B 3Hepmtt. B Kacrecrne peryJIHTopoB Bh1cryrra10T coornolllett11e AT<I>/ A,l],Cl> H BHYTPl1KJieTO'IHhIMypoBeHh fJIIOK030-6-¢oc¢arn (rrpo,nyKTa KaTaJIH3ttpyeMOH peaKU,HH). Ilp11 CHH)KeHl1H pacxo,na 3Hepr1111 B KJieTKe ITOBhilllaeTCH ypoBeHh AT<I> ( OTHOCHTeJihHO A,l],<I>) 11 rJIIOK030-6-¢oc¢a- Ta. B 3TOM CJiy<-iae aKTHBHOCTh reKCOKMHa3hI CHH- )l(aeTcH, H, CJie,UOBaTeJibHO, yMeHhWaeTC51 CKOPOCTh

TIOCTyrrJieHHH rJIIOK03hl B KJieTKy.

CJie11.yeT OTMeTHTh, 'ITOB pa3HhIX TKaHHX reKco-

KMHa3a npHCYTCTByeT B pa3JIH'IHhIX H3o¢opMax, OTJIH'JaIOlUHXCH BeJil1'1HHOH Km. f JIIOKOKHHa3a neqeHH (11 nocreK) 51.BJIHeTCH tt3o¢opMoii IV

(reKCOKHHa3a B KJieTKax MhillIU co,nep)KMTCH reKCOKHHa3a JI, a B KJleTKax orryxoJieBhIX TKaHeH npeo6JI3,L(aeT reKCOKHHa3a 111, c 6oJiee BhICOKHM, 'IeMy reKCOKHHa3hI 11, cpo,ncTBOM K rnIOK03e.

Ji. ,11,E<l>OC<l>OPRJIMPOBAHME I'JIIOK030-6-

<l>OC<l>ATA

flpeBpameHtte rn10Ko30-6-¢oc¢arn B rn10Ko3y B03MO)J(HO B ne'!eHH, TIO'!KaxH KJleTKax 3TIHTeJIH51 KHllle'IHHKa.B KJieTKax 3TMX oprattoB HMeeTcH ¢epMeHT rJTIOK030-6-¢oc¢aTa3a, KaTaJIH3HPYIOlUAA OTIUeITJieHHe ¢oc¢arnotf rpyrrrrhI fH1l.POJIMTM'IeCKHMnyreM:

fJIIOKo3o-6-ctiocctiaT +H 20 --+ f JIIOK03a + H3PO4

06pa3oBaBlllaHC51 cBo6o,nHM mIDK03a cnoco6Ha ,n11¢¢YH11.HPOBaTh 113 3T11x oprattoB B KPOBh. B ,npy-

rnx oprattax 11 TKaHHX rJIIOK03o-6-¢oc¢aTa3hI HeT, H I103TOMY ,ne¢oc¢opMJIHPOBaHHe fJIIOK030-6-¢oc- ¢arn HeB03MO)KHO. IlpttMep rro,no6Horo Heo6paTMMoro npoHMKHOBeHH51 fJIIOK03hI B KJieTKY - MhIW11,hI, r.ue rJIIOK030-6-¢oc¢aT MO)l(eT HCI10Jih30BaThC51 TOJihKO B MeTa60JIH3Me 3TOH KJieTKH.

B. METABOJIM3M rJIIOK030-6-<1>oc<1>ATA

fJIIOK030-6-¢oc¢aT MO)KeT MCTIOJih30BaTbC51 B KJieTKe B pa3JIH'IHhIXnpeBpameHH51.X, OCHOBHhIMl1 113 KOTOphIX 51BJI51IOTC51: Cl1HTe3 fJIHKOreHa, KaTa60JIH3M c 06pa30BaH11eM co2 11 H20 HJIH JiaKTaTa, c11HTe3 rreHT03. Pacna,n rnIOK03hI ,no KOHe'IHhIX npo,nyKTOB CJIY)KMT 11CTO'IHl1KOM3HeprHH 1l.JI5I opraHH3Ma. BMecTe c TeM B npou,ecce Mern6oJI113Ma fJIIOK03o-6-¢oc¢arn o6pa3YIQTCH npoMe)KYTO'IHhie rrpo,nyKThI, 11CTIOJih3yeMhie B ,11,aJibHetfweM ,UJI51 Cl1HTe3a aMHHOKHCJIOT' HyKJieOTl11l.OB, [Jil1Uep11Ha 11 )l(JAPHhIX KHCJIOT. TaKHM o6pa3oM, rn10Ko3o-6-¢oc- ¢aT - He TOJibKO cy6cTpaT 1l.JI5I OKHCJieHI151, HO H CTpOJATeJibHhIM MaTep11aJI ,UJI51 Cl1HTe3a HOBhIX co- e,n11HeHHM (p11c.

VI. META60Jllt13M rJlltlKOrEHA

Mttor11e TKaH11 c11HTe3ttpy10T B Ka'lecrne pe3epBHOH ¢opMhI fJIIOK03hI rn11KoreH. C11ttTe3 11 pacna,11, fJil1KOreHa o6ecne'll1BaIOT I10CT051.HCTBO KOHUeHTpau1111 fJIIOK03hl B KPOBH 11 C031l.aIOT 11.eno 1l.JI51 ee HCTIOJih30BaHl151 TKaH51Ml1 ITO Mepe Heo6X01l.HMOCTl1.

A. Crt>OEHME M <l>YHKQMM rJIMKOrEHA

fJIHKOreH - pa3BeTRJieHHhIH fOMOTIOJll1Mep rJIIOK03bl, B KOTOPOM OCTaTKM fJIIOK03hl coe,n11HeHbl B Jil1HeHHhIX y'iaCTKax a-1,4-mlfK03l1,1lHOH CB513hl0. B TO'IKaxBeTRJieHI151 MOHOMephI coe,UHHeHhl a- I ,6- [Jil1K03l1,llHhlMl1 CB51351.MH. 3n1 CB513H o6pa3yIOTC51 npHMepHO c K~hIM ,necHThIM OCTaTKOM fJIIOK03hl. CJie,noBaTeJihHO, TO'iKMBeTBJieHHH B rn11KoreHe BCTpe'lalOTC51rrpHMepHO qepe3 K~hJe ,nec51.Th ocTaTKOB mIOK03hI. TaK B03HttKaeT ,npeBoo6pa3HaH CTPYKTypa c MOJieKyJIHpHotl: Maccotr 'ITOCOOTBeTCTByeT rrptt6JIH3HTeJibHO 50 000 OCTaT-

KOB rnIOK03hI (p11c. TaKHM o6pa3oM, B MO-

JieKyJie fJI11KOfeHa HMeeTC51 TOJihKO 0,UHa CB06o,n- Ha51 aHOMepHaH OH-rpynrra H, cJie,noBaTeJihHO, TOJihKO 01l.HH BOCCTaHaBJIHBaIOlUl1H (pe,UyUHpyIOlUHH) KOHeu.

316

rnlOK03a ,,.~...

! !

<>i' ..J rrnoT

t

rmoKo:ia

neHT03bl

1

MOHOCaxap'1Abl

 

l

 

rerepononi.1-

 

caxapi.1Abl

>K1-1pbl

II

>K111pHble Kl<!CnOTbl

l

P111c. 7·20. MeTa6onM3M rnlOK03o·6·QioccpaTa.

317

7-22).

A

5

a·1,4-rm.1KO·

 

·OH

OH

OH

PMc. 7·21. CTPYKTYPa rnMKoreHa. A. CrpoeH1<1e MoneKynb1 rn1<1KoreHa: 1 -

ocraTK"1 rnlOK03bl, coeA1<1HeHHb1e a-1,4-rn"1K03"1AHOM cssi-

3bl0; 2 - OCTaTK"1 rnlOK03bl, C08A"1HSHHbl8 a-1,6-rn"1K03"1AHOM CBSl3blO; 3 -

Hepeoyl.l"1PYIOU1"18 KOHLj8Bbl8 MOHOM8pb1; 4 -

peoyl.l"1PYIO-

Ll1"1M KOHLj8BOM MOHOMep. 6. CrpoeHMe OTA8flbHOro qiparMeHTa MoneKynbl rnMKOreHa.

 

 

B KJJeTKaX )l(HBOTHhIX nrnKoreH - OCHOBHOH pe-

3epBHhIH no1111caxapH,I1,. Op11 rro1111Mep113au1111 rmoK03hT CHH)l(aeTCH pacrnopHMOCTh 06pa3y1orueti:c51 MOJTeKyJThl rJTHKOfeHa H, CJTe,IJ.OBaTeJTbHO, ee BJTHHHHe Ha OCMOTW:leCKOe ,IJ.aBJTeH11e B KJTeTKe. 3To o6CTOHTeJThCTBO o6bHCHHeT' rro1 1eMy B KJTeTKe n:e- IIOHHpyeTCH rJTHKOreH, a He CB060,IJ.H351 fJTIOK03a.

fJTHKOfeH Xp3HHTC5l B UHT030JTe K.IIeTKH B ¢lopMe rpaHyJT ,IJ.HaMeTpOM 10-40 HM. c rpaHyJTaMH CB5133Hbl H HeKOTOPhie ¢iepMeHThI, Y'laCTBYfOIUHe B MeTa60JTH3Me fJTHKoreHa, 1:1TO o6JTefl.laeT HX B3aHMO,IJ.eHCTBHe c cy6cTpaToM. Pa3Bern11eHttaH CTPYKTypa fJTHKoreHa o6ycJTOBJTHBaeT 60JTblllOe KOJTHqeCTBO KOHUeBhIX MOHOMepoB, qTo cnoco6cTByeT pa6oTe ¢iepMeHTOB, OTIUeIUIBIOIUHX HJTH rrp11coeIl,HHHIOIUHX MOHOMepbI rrp11 pacrra,IJ.e HJTH CHHTe3e fJTHKOfeHa, TaK KaK 3TH ¢iepMeHThI MOryT O,IJ.HOBpeMeHHO pa6oTaTh Ha HeCKOJlhKHX BeTBHX MOJTeKYJThl. r1111KoreH n:enoH11pyeTcH rnaBHhIM o6pa3oM B neqeHH 11 CKeJTeTHhlX Mhl1IIU3X.

flocJTe IlpMeMa IIHIUH, 6oraTOti: yrneBO,IJ.aMH, 33nac fJTHKOreHa B rreqeHH MO)l(eT COCT3BJT51Th IIPHMepHO 5% OT ee MaCCbI. B Mhllllll3X 33IIacaeTCH OKOJTO 1% fJTHKOreHa, Oil,H3KO Macca MbillleqHOH TKaHH 3HaqJITeJTbHO 6o11hwe 11 II03TOMY o6mee K01111qecrno fJIHKOfeHa B Mbllllll3X B 2 p33a 60JTbIIIe, qeM B rreqeHH. fJTHKOreH MO)l(eT CHHTe3HpOBaThC51 BO MHOfHX

KJTeTKax, Hanp11Mep B Hei1poHax, MaKpo¢iarax, KJTeTKax )l(HpOBOti: TK3HH, HO con:ep)l(3HHe ero B 3THX TKaHHX He3Ha1 111TeJibHO. B opraHH3Me MO)!(eT coLl,ep)!(aThcH AO 450 r rn11KoreHa.

Pacrra,IJ. fJTHKOreHa neqeHH CJiy)!(J1T B OCHOBHOM )lJTH IIO,II.Ll,ep)l(aHHH YPOBHH rJTIOK03hl B KPOBH B IIOCTa6cop6THBHOM nep110LI,e. Oo3TOMY co)lep)l(aH11e rn11KoreHa B neYeHH H3MeHHeTC51 B 33BHCHMOCTH OT pttTMa n11rnH11H. Op11 )lJTHTeJThHOM roJTOil.aHHH OHO CHH)!(aeTCH noqTJ1 Il.0 HYJTH. fJTHKoreH MhllllU CJTY- )!(HT pe3epBOM rJIIOK03bI - HCTOYHHKa 3HeprnH rrp11 MhiweqttoM coKpameH1111. Mb1weqHhii1 rn11KoreH He HCIIOJlb3YeTCH )lJl5I IIO,II.Ll,ep)l(3HHH ypOBHH fJTIOK03bl B KpOBH. KaK ~e yrroMHHMOCh pattee, B KJJeTKax Mbllllll HeT ¢iepMeHTa fJTIOK030-6-¢ioc¢iarn3hI, H o6pa30B3HHe cBo6o)lttoH. rJTIOK03hI HeB03MO)l(HO. PacXO)l fJTHKOreHa B MbIIIIU3X 33BHCHT B OCHOBHOM OT ¢lH3WieCKOH Harpy3KH (p11c.

B. CMHTE3 rnMKOrEHA (rnMKOrEHornHE3)

fJTHKoreH CHHTe3MpyeTC51 B nepHOil. ITHIUeBapeHHH (Yepe3 1-2 lJ IIOCJTe rrp11eMa yrneBOil,HOH rr11m11). C11en:yeT OTMeTHTh, qTo CHHTe3 rn11Koretta 113 fJTIOK03bl (pMC. 7-23), K3K H 11106oi1 aHa601111YeCKHH npouecc, HBJTHeTCH 3HI1,eproH11qecKHM, T.e. Tpe6ymIUHM 33TpaT 3Heprn11.

318

7-24).
KOI1Hp0<\loc€llopRJia3a.

PMC. 7-22. <l>yHKLIMM rnMKOreHa B neLteHM M MblWL.18X.

rJJJOK03a, IIOCTyIIaJOIUa5l B KJieTKy, cpoCcpOpHJIHpyeTC5l rrp11 yyacT1111 AT<D (peaKUHH l ). 3meM fJJIOK030-6-cpocQ:>aT B X0)1e o6paTl1MOH peaKU1111 rrpeBpawaeTc5l B rn10K010- l -cpocQ:>aT (peaKUH5l 2)

II0)1 )1eHCTBHeM Q:>epMeHTa Q:>occpormoKOMYTa3bl. fJJIOK030- l-Q:>ocQ:>aT ITO TepMO)lHHaMH'leCKOMYcoCTOHHHIO MOf 6bl CJJY)l(HTb cy6cTpaTOM )lJJH CHHTe3a rn11Koretta. Ho B CHJIY 06pan1MocT11 peaKUHH fJllOK030-6-¢>oc¢>aT H fJl!OK030- l-cpoccpaT CHHTe3 rn11Koretta 111 rnJOK010- l -Q:>oc¢>arn 11 ero pacrran OK33aJIHCb 6bI TaK)[(e o6paTHMb!Mll 11 II03TOMY HeKOHTpOJmpyeMbll\111. lfro6bl CHHTe3 fJlHKOreHa 6bJJI TepMO)lHH31\1111.J.eCKl1 Heo6paTHMblM, Heo6XO,lll1Ma

)10 IIOJIHHTeJI bH a5l CTa,11115! 06pa30Ball l15l ypl1)1l1HJ..IJ1 - cpoccpaTrnJOK03bI m YT<D 11 rnJOK010- l-cpoccparn (peaKllJUI 3). <I>epMeHT, KaTaJIH3llPYIOIUHM :ny pe3KUHIO, Ha3BaH no o6paTHOi1 peaKu1111: Y,IJ,<l>-rJIIO-

OuHaKo B KJieTKe o6paTHaH peaKUll5l He rrpoTeKaeT, IIOTOMy 'ITO o6pa30BaBlllllMC51 B xoue IlPJIMOM peaKUHltl 1111pocpoccpaT OlfeHb 6bICTpo pacwerrm1eTC5I m1po¢>oc¢>arn10i1 Ha 2 MOJieKyJibI cpocQ:>arn (p11c.

PeaKUAA o6paJoBaHHH YLl,<I>-rnIOKo3bI o6ycnoBJIHBaeT Heo6paTMMOCTb BCeti cepHM peaKU11H, rrpoTeKaIOIUHX np11 Cl1HTe3e fJI11KOreHa. 3T11M )J(e 06b5lCH51eTC5l HeB031\10)1(HOCTb rrpoTeKaHHH pacna-

~~-IJllOK03a@

npa~Mep 4

(om1rocaxapV1,q)

~

'~ Y,[l<P-l1110K03a

r------+Y,Q<l>

l11V1KoreH

PMc. 7-23. CMHTe3 rnMKoreHa. 1 - rntoKOKV1Ha3a vinvi reKCOKV1·

Ha3a; 2 -

cjJoccjJorntoKoMyTa3a; 3 -

Y,Q<P-rntoKonvipocjJoccjJopvi-

na3a;

4 -

rnviKOreHCV1HTa3a (rntoK03vimpaHccjJepa3a); 5 - cjJep-

MeHT

«BeTsneHvi~» (aMV1no-1,4--t1,6-rntoKo3vimpaHccjJepa3a),

CB8Tnbl8 ., 3awTpvixoeaHHbl8 Kpy>KKV1 -

rntoK03Hbl8 OCTaTKV1, 3aK-

paweHHbl8 KPY>KKV1 - rntoK03Hbl8 OCTaTKV1 B TOL!Ke B8TBn8HV1s:I.

319

3umpauc<1>epa3a).
7-23,
branching enzyme).

YpV1.Ql'IHAl'l¢oc¢aTrnt0K03a

(Y,QCl>-rJltOK03a)

P111c. 7-24. 06pa3oeaH111e YJl4>-rnt0K03bl.

,na rnttKoreHa nyTeM npocTora o6pamemrn npouecca era c11HTe3a.

06pa3oBaHHaH Y,Il;<l>-rnIOK03a ,naJiee 11cnoJih3YeTCH KaK ,UOHOp OCTaTKa fJIIOK03bl np11 Cl1HTe3e fJlHKoreHa (p11c. peaKU1151 4). 3Ty peaKU11!0

KaTaJI11311pyeT cpepMeHT rJIHKoreucuuTa3a (rn10K0-

flocKOJibKY B ,naHHOti peaKu1111 He 11cnOJih3yeTCH AT<t>, cpepMeHT Ha3bIBaJOT CHHTa3oti, a He CMHTeTa3oti. HyKJieoT11,nHaH tJaCTb YLl,<l>-rnJOK03bI HrpaeT cyiuecrneHHYJO pOJib B netiCTBHH fJIHKoreH Cl1HTa3bl, BbinOJIHHH cpyHKU11!0 «pyKOHTK11», npH noMOIU11 KOTOpoti cpepMeHT pacnoJiaraeT rnJOK03y B nom1caxapl1)1,HOi1 uenH B Hy)f(- HOM noJIO)!(eH1111. KpoMe Toro, HYKJieoT11nttaH l!aCTb YLl,<l>-rn10Ko3hI, no-BH)l.HMOMY, Heo6xonttMa ,UJIH y3HasaHHH cy6CTparn npH KaTaJil13e.

TaK KaK rnHKoreH B KJieTKe H11Kor,na He pacmenJIHeTC5I nOJTHOCTblO, C11HTe3 fJll1KOreHa ocymecTBJHleTCSI nyreM YMHHemur )')Ke 11Me10metic51 MOJieKYJibl noJI11caxapl1)1,a, Ha3bIBaeMoi1 «3aTpaBKa», HJIH «npaU:Mep». K «3arpasKe» rrocJienosaTeJibHO np11co- e,n11HH10TCH MOJJeKYJibI rnJOK03hI. Crpoett11eM MOneKYJibI «3aTpaBKH» KaK 6b1 npe,II,orrpe,II,eJIHeTcH THn CB5l3H, KOTOpb!H B03HHKaeT B peaKUHH TpaHCfJI11K03HJIHpOBaHmI. TaKHM o6pa30M, c11HTe311pyeTCH l10JI11Caxap11,II,, aHaJIOfW!Hblti no CTpOeHHIO c «3aTpaBOlJHbIM». B COCTaB «3aTpaBK11» MO)!(eT BX0)1.11Tb 6eJIOK rJIHKoreHHH, B KOTOPOM K OH-rpynne o,II,- Hora 113 THp03HHOBbIX OCTaTKOB npHCOe)l.HHetta OJIHrocaxapH,II,HaH uenolJKa (npHMepHO 8 OCTaTKOB fJllOK03bl). fnJOK03HhJe ocrnnor nepeHOCHTCH fJIHKOfeHC11HTa3oti Ha HepenyuttpyJOIUHH KOHeu OJiliracaxapl1)1,a 11 CB513bIBaJOTCH a-l ,4-rn11K0311,II,HblMH CBH3HMH. ITo OKOHlJaHHH CHHTe3a fJIHKOreHMH ocTaeTCH BKJIIOY.eHHblM B rpaHyJiy fJIHKOreHa.

Pa3BeTBJieHHaH CTPYKTYPa rn11Koretta 06pa3yeTCH npM yq:aCTHH aMl1JIO-l ,4~1,6-rmoK03HJITpattc- <t>epa3bl, Ha3bIBaeMoti cpepMeHTOM «BeTBJieHHH» (OT attrn. KaK TOJihKO rn11KoreHCHHrn3a YM11HH:eT JI11HetiHbiti ytJaCTOK npHMeptto no

11 fJIIOK03HblX OCTaTKOB, cpepMeHT BeTBJieHHH rrepeHOCHT ee KOHUeBOti 6JIOK, conep)f(alU11H 6-7 ocTaTKOB, Ha BHYTpeHHHti OCTaTOK fJllOK03bl 3TOH 11Jil1 ,II,pyroti ue1rn. B TOl.JKe BeTBJieH1151 KOHUeBOH OCTaTOK fJIIOK03hJ oJittracaxapl1)1,a coenttWieTc» c rH,II,- poKCHJihHOti rpynnoi1 B C 6 -nOJIO)!(eHHH c 06pa30Batt11eM a- l ,6-rn11K03H,II,Hoti CBH311. HosaH TOlJKa BeTBJieHHH MO)!(eT 6bITb o6pa30BaHa Ha paCCTOHHHH He Mettee 4 ocrnTKOB OT mo6oti )')Ke cymecrny10meti. TaKHM o6pa3oM, no Mepe CHHTe3a rnHKoretta MHoroKpaTHO B03pacTaeT lJHCJIO seTBJieHHH. KottUhI uenei1 CJI)')KaTTOlJKaMH poem MOJieKYJibl rrp11

eeCHHTe3e H HalJaJIOM TipH ee pacrra,n:e.

B.PACIIA,ll, fJIHKOfEHA (fJIHKOfEHOJIH3)

Pacrra,n: rn11KoreHa HJIH era Mo6HJitt3aUH51 npo11cxo,II,HT B OTBeT Ha TIOBblllieH11e noTpe6HOCTH opraH113Ma B rJIIOK03e. fJI11KoreH ne'-leHHpacnanaeTCSI B OCHOBHOM B I1HTepBaJiax Me){Qly rrp11eMaMH IIHIU11, KpOMe Toro, 3TOT npouecc B ne'-leHHH MblllIUaX yCKOpHeTCSI BO speMH cpi1311lfeCKOH pa60Thl.

Pacrra,n: rn11Koretta (p11c. 7-25) rrpottcxo,II,HT rryTeM IlOCJie,II,OBaTeJibHOfO onuerrJieHHH OCTaTKOB fJIIOK03bl B 811)1,e fJIIOK030- l-cpoccparn. fJIHK03H)],- HaH CB513b pacmerrJIH:eTCH c HCTIOJib30BaHHeM HeopraH11'-teCKoracpoccparn, IT03TOMY npouecc Ha3bIBaeTcH cpoccpopoJIH30M, a cpepMeHT rnHKoreH- <PoccpopwJia3oH.

TaK )l(e KaK 11 c11me3, pacmeruiett11e rnHKoretta Half11HaeTCH c HepeJlYllHPyJOIUefO KOHUa IIOJIHCa-

320

debranching enzyme).

xaptt)lHOM uentt. flptt 3TOM HaJil1'-ll1epa3BeTBJieH- HOM CTPYKTypbr rn HKoreHa o6nerqaeT 6brCTpoe BblCB060)!()],em1e rJIJOK03HblX OCTaTKOB, TaK KaK 4eM 60Jihllle KOHUOB l1MeeT MOJieKyJia rJII1KOreHa, TeM 6oJibllle MOJieKyJI rnttKorempoccpopHJia3hl MoryT )lei1cTBOBaTb O)lHOBpeMeHHO.

fJinKorenc(>occ(>opmia3a pacw,enm1eT TOJihKO a-

I ,4-rnttK03l1.Utthre CBH3l1 (peaKUHH 1). DocJie)loBa-

TeJibHOe OTW,enJieHHe rJIJOK03HblX OCTaTKOB npeKpaw,aeTCH, KOr)la )lO T04Kl1 BeTBJieHI15! OCTaeTCH 4

MoHoMepa. CTo)lo6HaH oco6ettttocTh n )leifcTBtttt rn11Korettcpoccpop1rna3b1 o6ycJIOBJieHa pa3MepoM 11 CTpOetttteM ee aKTl1BHOfO UeHTpa.

Ll,arrhtteiflllttif pacna)], rnttKoreHa Tpe6yeT yqacTHH )lByx )lpyrn:x cpepMeHTOB. CHa'-larraTptt ocrnBlllHXCH )lO TO'-lKl1 BeTBJieHI15! fJIIOK03HbIX OCTaTKa nepettocHTCH np11 yqacT1111 onttrocaxaptt)lTpaHccpepa3hI (peaKUttH 2) Ha Hepenyuttpy10w,ttif KOHeu coce)lHeif uentt, YMI1H5l5! ee l1 TaKl1M o6pa30M co- 3)laBa5! ycnoBttH )lJIH )leifcTBHH cpoccpop11na3hr. 0CTaBllll1MC5! B TO'!Ke BeTBJieHI15! rJIJOK03Hblll ocTaTOK f11)lpOJII1Tl1'-leCKI1 OTW,eTIJIHeTCH c TIOMOW,blO a-1,6-rnJOK03H)la3bl B Bl1).Ie CB060)lHOH rJIIOK03bI (peaKuHH 3), nocne 4ero Hepa3BeTBneHHhIM yqacTOK rJil1KOreHa MO)!(eT BHOBb aTaKOBaTbCH cpoccpoPl1Jla30H.

C4ttTaIOT, 4To nepeHoc Tpex ocrnTKOB rnJOK03hr l1 ynarreH11e MOHOMepa l13 T04Kl1 BeTBJieHI15I (peaKUI1l1 2 l1 3) KaTaJII13ttpyeT 0)ll1H l1 TOT )Ke cpepMeHT, KOTOpblH o6Jia)J,aeT )lByMH pa3HbIMI1 cpepMeHTaTl1BHblMl1 aKTI1BHOCT5IMl1 - TpaHccpepa3HOM l1 rJIHK0311)la3ttoi1. Ero Ha3bIBaIOT «)leBeTBHW,HM» cpepMeHTOM (oT attrn.

Dpo)lyKT )leifcTBttH rnttKorettcpoccpopl1Jla3bI - fJIIOK030- I -cpoccpaT - 3aTeM 1130Mep113yeTC5I B rn10Ko3o-6-cpoccpar cpoccpornJOKOMYTa3oti. LJ:arree rnJOKOJo-6-cpoccpaT BKJ1IO'-laeTCH B npou:ecc KaTa6oJitt3Ma HJIH n.pyrne Mern6onw1ecKtte TIYTtt. B ne'feHH (HO He B MbilllUax) fJIIOK030-6-cpoccpaT MmKeT fl1)lpOJII130BaTbC51 c o6pa30BaHtteM fJIIOK03bl, KOTOpaH BhJ)leJI51eTC51 B KPOBb. 3TY peaKUHIO Karnn111ttpyeT cpepMeHT rnroK03o-6-cpoccparn3a. PeaKLI,115I npoTeKaeT B npocBeTe 3P, KY)la c noMo- W,hIO cneLI,ttaJihHOro 6enKa TpaHcnopTttpyeTC51 rnro- K03o-6-cpoccpaT. <l>epMeHT JIOKaJll130BaH Ha MeM6paHe 3P TaKI1M o6pa30M, 4TO ero aKTI1BHhIM ueHTp o6paw,eH B npocBeT 3P. flpo)lyKTbt rtt)lpOJIH3a (rnroKo3a tt Heopratttt4eCKtttl cpoccpar) B03Bpaw,a- IOTC5I B U11TOTIJia3My TaJOKe c IIOMOW,bIO TpaHcrropTHbIX CHCTeM.

rm1KOreH

1

}lee j

,~~axrrxxJ_ ___________ _

'1 On111rocaxap111gTpaHccpepa3a -2

~---------

.~ 3 a1 ,6-rnt0K03111ga3a rntaK03a - l

t~i

H3P04 ~ lm1KoreHcpoccpopV1naaa

~ 1

y''

n rnlOK030-1-cpoccpaT

'

'

'

...''

n rntaK030-6-cj)occj)aT

P111c. 7-25. PacnaA rn111KoreHa. B paMKe - cpparMeHT rm1KoreHa c TO'lKOliisernneHl'l5l.3aKpaweHHblli1 Kpy)l(OK - rnt0K03Hblli1 ocraTOK, CB5l38HHblli1 a-1,6-rnl'IK031'1JlHOli1CB5l3btO; CBeTnble l'I38WTpl'l- XOB8HHble KP~Kl'I - rnt0K03Hb1e ocraTKlll B nl'IHeli1HblXy'laCTKax l'I60KOBblX BeTB5lX, CB5l38HHble a-1,4-rnl'IK031'1JlHOli1CB5l3bt0. 1 - rnl'IKoreHcf>occpopl'lnasa;2 - onl'lrocaxap111orpaHccpepasa;3 - a- 1,6-rnt0K031'1Jla3a.

321

11-2128

f. DllOJIOfHqECKOE 3HAqEHllE OBMEHA fJillKOfEHA B rrnqEHH II MhIIIIQAX

Ha p11cyttKe 7-26 rrp11Be.IJ.etta o6rn:M cxeMa c11tt - Te3a 11 pacrra).l,a rn11Koretta 11 perymrum1 3Tl1X rrpoueccos ropMOHaM11.

CpaBHett11e 3T11x rrpoueccoB rro3so;rneT C.IJ.eJiaTh CJie.IJ.y:iorn:11e BbIBO).l,bJ:

c11ttTe3 11 pacrra.IJ. rn11Koretta rrpoTeKalOT rro pa3HhIM Mern6on11qecK11M11 rryrn:M;

rreqeHb 3arracaeT rJilOK03y B Bl1.Il.e rn11KoreHa

He CTOJibKO llllil co6CTBeHHbIX Hy)J(,[(, CKOJibKO llllil IIOMep)l(aHJUI IIOCT05IHHOH KOHUeHTpau1111 fJIJOK03bl B KPOBl1, 11, CJie,n:oBaTeJihHO, o6ecrre1Il1BaeT IIOCTyIIJieH11e rJIJOK03bI B ,n:pyr11e TKaHl1. CTp11CYTCTB11e B rreqeH11 rJIJOK03o-6-¢oc- ¢aTa3hI o6yCJIOBJil1BaeT 3TY rnaBHYJO <PYHKlll1lO rre1Iett11 B o6Mette rn11KoreHa;

QlYHKUl15I MbIIIIe1IHOro fJI11KOretta 3aKJil01IaeTC5I B OCB060)l(Jl:eHl111 rJIIOK030-6-<Poc¢arn, rroTpe6JI5IeMOf0 B caMOH MblIIIUe llllil OICHCJieHl151 11 11CIIOJib30BaHl15I 3Hepr1111;

c11me3 rn11Koretta - rrpouecc 3H;:i:eprott111IecKHH. TaK Ha BKJIJ0qett11e o,n:Horo ocrnTKa rnroK03hI B IIOJI11caxapl1).l,HYJO uerrh 11CIIOJib3yeTC5I

1MOJib AT<l> 11 1 MOJib YT<l>;

pacrrM rn11Koretta ,n:o rn10Ko3o-6-<Poc<Parn He TPe6yeT :rnepm11;

Heo6paT11MOCTh rrpoueccos Cl1HTe3a 11 pacrrMa rn11KoreHa 06ecrreq11BaeTC5I l1X peryAAu11ei1.

VII. PErYflSIU14SI META60fll43MA

rnl4KOrEHA

CTpoueccbl HaKoIIJieHM rnIOK03ht B s11,n:e rn11KoreHa 11 ero pacrra.IJ.a .IJ.OJI)l(HbI 6bITb cornacoBaHbI c I10Tpe6HOCT51Ml1 opraHl13Ma B fJIIOK03e KaK 11cTOqH11Ke 3Hepr1111. 0.IJ.HOBpeMeHHOe rrpoTeKaH11e 3Tl1X MeTa60JI11qecK11X rryrei1 HeB03MO)J(H0, TaK KaK B 3TOM cnyqae 06pa3yeTC5l «XOJIOCTOH» U11KJI, cy- w,eCTBOBaH11e KOToporo rrp11BO).l,11T TOJibKO K 6ecITOJie3HOH TpaTe AT<l>.

113MeHeH11e HarrpaBJieHl15I rrpoueccoB B Mern6oJil13Me rn11KoreHa 06ecrreq11BaIOT peryJI51TOpHble MexaHl13MbI, B KOTOpbIX yqaCTBYJOT ropMOHbl. CTepeKJI10qeH11e rrpoueccoB c11ttTe3a 11 M0611JI113au1111 rn11Koretta rrpo11cxo,n:11T rrp11 cMette a6cop6T11Bttoro rrep110).l,a Ha rrocrn6cop6Tl1BHbIM HJil1 COCT05IHl15I IIOK051 opraHH3Ma Ha pe)l(J1M Qll13111IeCKOH pa60TbI.

B rrepeKJiroqeH1111 3Tl1X Mern6on11qecKHx rryrei1 B

rreqemr Y'IaCTBYJOTropMOHbI 11Hcyn11H, rJIJOKarott 11

a).l,peHaJil1H, a B MbIIIIUax - 11HCYJil1H 11 MpeHaJil1H.

A. XAPAKTEPllCTllKA fOPMOHOB,

PEI'YJlllPYIOIIJ,llX OBMEH fJillKOrEHA

CTeps11qHhIM c11rHaJIOM llllil Cl1HTe3a 11 ceKpeu1111 11HCYJil1Ha 11 fJIIOKarotta 5IBJI51eTC5I 113MeHeHl1e ypOBH51 rnroK03hI B Kpos11. B ttopMe KOHUeHTPaUM rnroKo3hI B KPOBM COOTBeTCTByeT 3,3-5,5 MMOJib/JI (60-

100 Mr/.IJ.JJ).

11ucyJIHH - 6eJIKOBbIH ropMOH, CMHTe311pyeTC5I 11 ceKpeT11pyeTC5I B KpOBb ~-KJieTKaMH OCTPOBKOB JlattrepxaHca rro,Il;)l(eny,n:oqHoi1 )l(eJie3bI. ~-KJieTKM

1IYBCTBl1TeJibHbl K 113MeHeHl151M CO.IJ.ep)l(aHmI fJIJOK03bl B KPOBl1 11 ceKpeT11PYJOT MHCyJil1H B OTBeT Ha IIOBbIIIIeH11e ee COL(ep)l(aHH5I ITOCJie rrp11eMa I1HW:11. TpattcrropTHbIH 6enoK (fJIIOT-2), 06ecrre1IMBa10- w,11i1 IIOCTyIIJieH11e fJilOK03bI B ~-KJieTKH, OTJI11qaeTC5I HM3KHM cpo.IJ.CTBOM K Hett. Cne,n:oBaTeJibHO, 3TOT 6enoK TPaHcrropT11pyeT rn10Ko3y B KJieTKY rro,n:- )l(eJiy.IJ.oqttoi1 )KeJie3bl Jil1IIIb ITOCJie TOfO, KaK ee CO).l,ep)l(aHMe B KPOBl1 6y.IJ.eT BbIIIIe HOPMaJibHOfO ypOBH5I (6onee 5,5 MMOJib/JI).

B ~-KJieTKax rnroKo3a ¢oc¢op11JI11pyeTC5l rn10KOK11Ha3ol1, 11Me10rnei1 TaK)l(e BbICOKYJO Km llllil rnro-

K03bI - 12 MMOJib/JI. CKOPOCTb <Poc¢op11JI11p0BaHl15l rJIJOK03bI rJIIOKOKHHa30H B ~-KJieTKax I1P51MO

rrpOITOPUMOHaJibHa ee KOHUettrpaUl111 B KpOBl1. C11ttTe3 11HCYJIMHa peryn11pyeTc5I rnroKo3ol1.

fJIIOK03a (11JI11 ee MeTa60Jil1Tbl), I10-Bl1).l,11MOMy, HeIIOCpe).l,CTBeHHO yqaCTBYJOT B peryJI5IU1111 3KcrrpecCl111 retta MHcyn11Ha. CeKpeuM 11HCyJIMHa M rnroKaroHa TaK)l(e peryJIMpyeTC5I rJIIOK030H, KOTOpa5I CTl1MymrpyeT ceKpeu,1110 l1HCyJI11Ha 113 ~-KJieTOK 11 rro.IJ.aBJI5IeT ceKpeu1110 rn10Kar0Ha 113 a-KJieToK. KpoMe Toro, caM 11Hcyn11H CHl1)l(aeT ceKpeu,1110 rn10Karotta (cM. pa3.IJ.eJI 11).

rmoKal'OH- «rOpMOH fOJIOL(a», Bbipa6aThIBaeMbIH 1X-KJieTKaM11 ITO,Il;)l(eJIY).l,OqHOH )l(eJie3bl B OTBeT Ha CHJ1)l(eH11e ypOBH5I fJilOK03bl B KpOBM. flo XMM11qecKoi1 rrp11po.IJ.e rn10KaroH - rreITTl1.Il..

A,npeHaJIHH BbI,n:eJI5IeTC5I lf3 KJieTOK M03fOBOro Bew,ecTBa Ha).l,rroqeqHl1KOB B OTBeT Ha Cl1fHaJibl HepBHOH Cl1CTeMbl, 11,n:yrn:11e 113 M03ra rrp11 B03Hl1KHOBeHl111 3KCTpeMaJibHbIX c1nyau,ui1 (Harrp11Mep, 6ercrno MJIM 6opb6a), Tpe6y10rn:11x BHe3arrHoi1 MbirneqHoH ,n:e51TeJibHOCTH. ALJ:peHaJIMH 51BJI51eTC51 c11rttanoM <<Tpesor11». Ott ,n:OJI)KeH MrttoBeHHO 06ecrreq11Tb MbIIIIUbl 11 M03r 11CTOqHl1KOM 3HeprM11.

322

323
Ras-

 

 

 

 

f1111KOreH

~'y;~----------HcynMH

 

rnt0KaroH

------~

5

4

 

Ap,peH81111H

e

 

 

 

 

 

 

(ne'leHb)

 

 

 

 

 

YA<l>-rnt0Koaa

 

 

 

 

 

 

 

 

 

 

? e PPi

 

 

 

 

 

6

 

 

 

 

~

YT<l>

 

 

 

 

 

f11tOK030-1-Q>occpaT

 

 

 

 

 

 

 

 

lT

 

/

f1111K011113

 

 

 

 

 

 

 

 

 

)t

 

 

 

f 1110K030-6-Q>occpaT - - - - - - - - - - - -<~- -t

neHT030cpoccpaTHbllii

 

 

.~

 

 

 

1)::_ AQ<l>

','»

nyTb

 

 

 

 

 

Apyrne nYTl1

 

 

P1

 

 

 

 

 

AT<l>

 

 

 

 

 

ne'l9Hb

rrnoKoaa

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

L\11T03011b

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

MeM6paHa K119TKl1

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

f11IOK038

 

 

 

 

 

 

 

 

Kpoeb

 

 

 

 

Pitc. 7-26. CMHTe3 M pacnaA rnMKOreHa.

1 -

reKCOKHHaaa 11n11 rnt0KOKHHasa (neljSHb); 2 - YJl<l>-rnt0Kon11pocpoccpop11nasa;

3 - rn11KoreHCHHrasa; 4- aM11no-1,4~1.6-rnt0K0311nrpaHccj:lepasa (cpepMeHT sersneH11s:r); 5 - rn11KoreHcpoccpop11nasa; 6- "Aeserss:r111111i» cpepMeHr; 7 - rnt0Koso-6-cpoccparasa (neljeHb); 8- rpaHcnoprHble c11creMb1 rfllOT.

6. PErYJUIIJJUI AKTHBHOCTH

fJIHKOfEH<l>OC<l>OPHJIA3hl

H fJIHKOrEHCHHTA3hl

CTOCKOJihKY CJIHTe3 JI pacrra.n, fJIJIKOreHa rrpOTeKaIOT ITO pa3JIWIHhIM MeTa60JIWieCKMM rryrn:M, 3TJI rrpouecchl MOryr KOHTPOJIJlpOBaThCH peumipOKHO. BJIJ1HHJ1e ropMOHOB Ha CJIHTe3 JI pacrra.u rJIJIKOretta ocyw.ecTBJIHeTCH rryreM Jl3MeHeHMH B rrpOTJIBOIIOJIO)l(}{bIX HarrpaB.Jiemrnx aKTMBHOCTM .unyx KJIIOqeBhIX <PepMeHToB: fJIJIKOreHCllHTa3hI JI fJIJIKOfeH<t>oc- <PopllJia3hl c IIOMOIUhIO JIX <Poc<t>opllJIJIPOBaHMH JI

1.1.e<t>oc<t>opllJIJIPOBaHMH (pJIC.

7-27).

 

fJJHKoreul(>Occj>opu.mua cyw.ecrnyeT B 2 <PopMax:

1)

<Poc<t>opllJIJIPOBaHHllil -

aKTJIBHllil (<l>opMa a);

2)

1.1.ecPoccPoPJIJIBPOBaHHIDI -

HeaKTMBHIDI (<l>opMa B).

<I>oc<PopMIDlpoBaHJt:e ocyw,ecrn.rrn:eTCH rryreM rrepeHoca <Poc<Pamoro ocraTKa c ATCI> Ha rn.rq>OKCllJihHYIO rpyrr- rry O,ll.Horo Jl3 cepMHOBhIX ocTaTKOB <l>epMema. CJieLJ:- CTBJle 3TOfO - KOHcPoPMfill}fO:HHhie Jl3MeHeHMH MOJieKYJlhI <l>epMema JI ero a.KI11B£lUIDI.

B3aJIMorrpenpameHMH 2 <PopM rJIJ1Korett<Poc<Pop1rna3hI 06ecrreqJ1BaIOTCH LJ:eHCTBJleM <PepMeHTOB KJt:HaJhI <Poc<t>opllJiaJhI JI <Poc<l>orrpoTeJ1HcPOCcPaTa3h1 (<l>epMeHT, CTpYKTYPHO CBH3aHHhltt c MOJieicyJiaMJI fJIJIKoreHa). B cno10 oqepeLJ:h, aKTJIBHOCTh KJt:- HaJhI <Poc¢>opJ1Jia3hI JI <Poc<PorrpOTeJ1HcPOCcPaTa3hI TaIOKe peryJIJ1pyeTCH rryreM <Poc<t>oprurnponaHMH JI LJ:e<Poc<t>opJ1JIJ1ponaHMH.

Aimmau,mi KHIUl3bl cl>occJ>opH.Tia3bl IIpoJICXOJ1:JIT IIOLJ: LJ:ei1:cTBJ1eM rrpOTeJIHKJt:Ha3hI A - II.KA (uAMCl>- 3aBJICJIMOi1:). uAMCI> CHaqan:a aKTJIBMpyeT rrpoTellHKMHa3Y A, KOTOpllil <Poc<PopllJillpyeT Kl1Ha3Y <Poc- <l>opllJia3hl, rrepeBOM ee B aKTJIBHOe COCTOHHJle, a Ta, B cno10 oqepe)J,h, <Poc<PopJ1JIJ1pyeT rnJ1Korett<Poc- <l>opJ1Jia3y. CJ1me3 uAMCI> CTJ1MYJIJ1pyeTCH a.n,pettaJillHOM JI fJIIOKarOHOM (CM. pa3,ll,eJI 5).

Alcruua~Jr cj>oc<JmnpoTeuucj>occj>aTaJbI rrpoJ1cxo-

.mrr B pe3yJibTaTe peaKUJIJI <Poc<l>opllJIMPOBaHMH, KaTaJIJl3JlpyeMOH crreuJ1cPJ1qecKOH rrpoTeJIHKJIHa30H, KOTOpaH, B CBOIO oqepe,Uh, aKTMBJlpyeTCH MHCYJIJlHOM rrocpeLJ:CTBOM KaCKa.n,a peaKUJt:tt c yqacTMeM

Соседние файлы в папке підручники. біохімія