підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin
.pdfrm1KoreH¢oc¢op1..maaa «8» HeaKT.
Pi |
AT<l> |
|
K111Haaa |
|
cpoccpo- |
|
p111naab1 |
|
aKT. |
rm1KoreH¢oc¢op111naaa «A» 8KT.
rn111KOreHCll1HT83a
8KT.
Pi
AT<l>
<l>n-¢oc¢aTaaa (rP)
A,Q<l>
rn111KoreHCl11HTa3a
HeaKT.
PMC. 7-27. ll13MeHeHMe 8KTMBHOCTM rnMKOreH<PocQ>opMna3bl M rnMKOreHCMHTa3i.1. Kpy>KKaM111 o6oaHaYeHbl MoneKynb1 cpepMeHra: aKrneHi.1e - yepHb1e, HeaKrnBHb1e - 6enb1e. <lln-cpoccpara- aa (rP) - cpoccponpoTe111Hcpoccparaaa rpaHyn rn111KoreHa.
6eJIKa, a TaJOKe npyrnx 6eJIKOB 11 <t>epMeHTOB ( c11rHaJihHb1tl: Ras-nyrh, CM. pa1neJI 11). AKT11B11pyeMM 11HCYJIHHOM npoTeHHKl1Ha3a <t>oc<t>op11JI11pyeT H TeM caMhIM aKTHB11pyeT <t>oc<t>orrp0Te11tt<Poc<t>arn1y. AK-
T11BHM <t>oc<t>onp0Te11tt<t>oc<t>arn1a ne<t>oc<t>op11JI11pyeT 11, CJie.IJ.OBaTeJibHO, 11HaKTl1B11pyeT Kl1Ha3y <t>oc- <t>opHJia3bl 11 rn11KoreH<t>oc<t>op11Jia3y (p11c.
AKTHBHOCTh rJIHKOfeHCHHTa3hl TaIOKe H3MemJeTCH B pe1yJihTaTe <t>oc<t>op11JI11poBaHH5l H ne<t>oc<t>o- p11JittpoBaHH5l (cM. Bhillle p11c. 7-27). OnttaKo eCTb CYllJeCTBeHHbie pa3Jil1'111HB peryJI51U1111 fJil1KOreH- <t>oc<t>op11Jia3bl H fJil1KOreHCl1HTa3bI:
• <t>oc<t>op11JI11poBaH11e fJil1KOreHC11HTa3bl KaTaJil1311pyeT nK A 11 Bbl3bJBaeT ee 11HaKTl1Bau1110;
• ne<t>oc<t>op11JI11p0Batt11e rn11KoreHCHHTa3hI no)l netl:cTBHeM <t>oc<t>orrp0Te11tt<Poc<t>ma1b1, ttao6opoT, ee aKTl1B11pyeT.
B. PEI'YJUlll,HH METAI>OJHI3MA fJUfKOfEHA
B TIEqEHH
KaK y)Ke OTMe'laJIOCh, nepBH'IHhIHcJ.ffHaJI .IJ.JIH
Cl1HTe3a HHCYJil1Ha 11 fJIIOKaroHa - H3MettettHe
KottuettTpautt11 rnIOK03hI B KPOBH. HHcyJIHH H fJIIOKarott IlOCTOHHHO npHCYTCTBYIOT B KpOBH, HO rrp11 cMeHe a6cop6THBHoro rrep11ona Ha rrocTa6cop6THBHhIM H3MeHHeTCH HX OTHOCHTeJibHaH KOHueHTpau.1rn, 'ITOHBJI51eTCH rnaBHhIM <t>aKTOPOM, rrepeKJIIOlJaIOWHM MeTa60JIH3M fJIHKOreHa B neqeHH. 0THOllleHHe KOHUeHTpauHH HHCYJIHHa B KpOBH K KOHUeHTpaum1 fJIIOKarmrn Ha3hIBaJOT «HHCYJIHHrmoKarOHOBhlH HH.IJ.eKC». 8 nocTa6cop6THBHOM
nep110ne HHCYJIHH-fJIIOKafOHOBbIM HH.IJ.eKC CHH- )KaeTCH, 11 pelllaIOwee 3HalJeH11e B peryJIHU.HH KOH- u.eHTpaUHH fJIIOK03bl B KPOBH nptto6perneT KOH-
u.eHTpaUHH fJIIOKaroHa.
fJIIOKarou .IJ.JIH renaTOU.11TOB CJIY)!(HT BHeWHHM CHfHaJIOM 0 Heo6XO.IJ.HMOCTH BbUleJieHHH B KpOBb
rnJOK03ht 1a cqfa pacna)la rnttKoreHa (rnttKoreHoJIH3a) 11Jll1 CHHTe3a fJIIOK03bl H3 .IJ.PYfHX sewecTB - rnIOKOHeorette1a (3TOT npou.ecc 6yneT H3JIO)!(eH no1nttee). fopMOH CBH3bIBaeTcH c peuenrnpoM Ha nJia3MaTHlJeCKOM MeM6paHe H aKTHBHpyeT npH nocpe.IJ.HH'leCTBe G-6eJIKa aneHHJiaTU.HKJia1y, KOTOpm1 KaTaJIH3HpyeT 06pa10Batt11e u.AM<I> 111 A T<I>
(cM. pa3.IJ.eJI 5). Ll,anee cJienyeT KacKan peaKUHH, npHBO.IJ.5IlIIHM B ne'IeHH K aKTHBaUHH fJil1KOreH- <t>oc<t>opHJia3bl 11 HHfH6HpOBaHHIO fJIHKOfeHCHH-
Ta3bl (pHC. 3TOT MeXaHH3M npHBO.IJ.HT K BbICB060)!(.IJ.eHHIO 113 fJIHKoreHa fJIIOK030- l -<Poc<t>arn, KOTOpbIM npeBpawaeTCH B fJIIOK030-6-<Poc<t>aT. 3aTeM non BJIH51HHeM rJIIOK010-6-<t>oc<t>arn1h1 06pa1yeTCH CB060.IJ.H351 fJIIOK03a, cnoco6HM BbIHTH 111 KJieTK11 B KpOBh. TaKHM 06pa10M, rnJOKaroH B ne'!ett11, CTl1M)'JIHPYH pacnan rn11Koretta, cnoco6CTByeT no.IJ..IJ.ep)!(aHHIO fJIIOK03bl B KPOBH Ha noCTOHHHOM ypOBHe.
A,n,peHaJIHH CTHM)'JIHpyeTBbIBe.IJ.eHHe fJIIOK03bl 113 ne'IeHHB KpOBb, .IJ.JIH Toro 'IT06bICHa6.IJ.HTb TKaHH (B OCHOBHOM M03f 11 MhlllIUhI) «TOnJIHBOM» B 3KCTpeMaJibHOM Cl1Tyau.HH. 3<P<PeKT anpeHaJIHHa B ne'!eHHo6ycJIOBJieH <t>oc<t>opHJIHpOBaHHeM (H aKTHBau.11etl:) fJIHKOreH<t>oc<t>opHJia3bl. A,upeHaJIHH HMeeT CXO.IJ.HblH c fJIIOKarOHOM MexaHH3M netl:CTBH51 (p11c. Ho B03MO)!(HO BKJIIOlfetttte H npyrotf 3<P<PeKTOPHOM CHCTeMbl nepenalfH Cl1rHaJia B KJieTKY neY:eHl1 (pHC.
KaK35l CHCTeMa nepe.IJ.a'IHCHTHaJia B KJleTKY 6y-
.IJ.eT HCIIOJib30BaHa, 3aBHCHT OT Tl1na peuenTopoB, c
324
|
|
|
|
|
|
|
® |
|
|
|
|
|
|
<Pn-cpoccparnaa (rP) |
|
|
|
|
|||||||
|
|
|
|
|
|
|||||||
|
|
|
|
|
|
|
|
|
|
|
||
|
HeaKT. |
|
|
|
rn11KOreHCl1HTa38 |
|
|
|
|
|
||
|
|
|
|
|
|
HeaKT. |
|
|
|
|
|
|
V1Hcym1H ~~~n~~~(pp~~~~)-----•fAAAT: |
|
|
|
|
|
|
|
|
||||
|
|
|
|
|
|
|
|
|||||
11::--------------• t- Pi |
|
|
|
|
|
|||||||
'+' |
|
|
|
rm1KoreHCl1HT838 |
||||||||
|
|
|
|
|
|
8KT. |
|
|
|
|
|
|
~---~® |
I |
|
|
|
|
|
|
|
|
|||
|
|
|
|
|
|
|
|
|||||
I |
|
|
|
|
|
|
|
|
||||
|
<Pn-ct:>occparnaa (rP) - - - - - - |
1 |
|
|
|
|
|
|
|
|
|
|
|
8KT. |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
® |
|
|
|||
|
|
|
|
|
|
|
|
|
||||
|
|
|
|
|
|
|
|
|||||
|
|
|
|
|
|
|
|
|
||||
|
|
|
|
|
|
K11Haaa cpoccpop11naab1 |
|
|
||||
|
|
|
|
|
|
8KT. |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
--------------' |
|
· |
Pi |
||||||
|
|
|
|
|
|
|
||||||
K11Haaa tj:>octj:>op11naab1
HeaKT.
P11c. 7-28. Bn11siH11e 11Hcyn11Ha Ha aKTl1BHOCTb rn11KoreHCl1HTaab1 11 K11Haab1 cpoccpop11naab1. <l>n-cpoccpara3a (rP) - cpoccponpore-
111Hcjloc¢arasa rpaHyn rn111KoreHa. nK (pp90S6) - npore111HK111Ha3a, aKr111s111pyeMa5i 111Hcyn111HOM.
KOTOphIMM B3a11Mo,!lei1cTByeT a,n,peHaJIMH. TaK, B3a11Monei1cTB11e an,peHaJI11Ha c p2-peuerrrnpaM11 KJieTOK rre'IeHH rrpMBO).J,11T 13 nei1crn11e a,n,eHMJTaTl(MKJia3HYlO cttcTeMy. B3a11Monei1crn11e )Ke a;::i:pettaJIHHa c a,-peuerrTopaMH «BKJIIO'IaeT» l1H03l1TOJI(p0CcPaTHblH MexaH113M TPaHcMeM6paHHOi1 rrepenaq11 ropMOHaJihHoro cttrHana. Pe3y.11hTaT ;::i:eti.CTBM51 06e11x CMCTeM - cfloccflopH.11MpOBaH11e K.IJIO'!eBbIXcflepMeHTOB 11 rrepeKJTIO'IeH11errpoueccoB c CHHTe3a r.1111KoreHa Ha ero pacna;::i:. C.11e,!lyeT OTMeTl1Th, 'ITO TMrr pel(ernopoB, KOTOPhill B Ha1160JibWeti. CTerreHl1 BOB-
.11eKaeTC5I B OTBeT K.JJeTK11 Ha aLipeHaJIHH, 3aBHCl1T OT KOHUeHTPaU11I1 ero B KpOBH.
B nepuo,11, numesapeum1 rrpeo6.11aLiaeT BJIH5IHMe MHcyJI11Ha, TaK KaK 11HCYJIMH-f.11IOKaroHOBb!H HH,!leKC B 3TOM c.11yqae rroBhiwaeTCH. B ue.110M 11Hcy.11HH B.JJMHeT Ha o6MeH fJU1KOreHa npOTMBOilOJIO)J(HO fJIIOKaroHy. 11HCYJil1H CHIDKaeT KOHUeHTPaUHIO f.1110K03bl B KPOBH B rrepHO)J, rrmueBapeHHH, ;::i:ei1cTBy5I Ha MeTa60JII13M rre'!eHHC.11eL!YIOW11M o6pa3oM:
• CHl1)1(aeT ypoBeHb uAM<I> B K.JJeTKax, <Poc<Popillrnpy5I (onocpenoBaHHO 4epe3 Ras-ITYTh) M TeM caMhIM aKTttB11py51 rrpoTeMHKttHa3y B
(uAM<l>-He3aBHC11My10). IlpoTeHHKHHa3a B, B CBOIO O'Iepejj,h,cfloccflopttJTHpyeT l1 aKTl1BMpyeT
cfloccflo,!1.113cTepa3y uAM<D - cflepMeHT, nm- pOJII13YIOIU11H uAM<I> c o6pa30BaH11eM AM<l>. MexaHtt3M B.11H5IHH5I HHcyJIHHa Ha ypoBeHh uAM<I> B KJieTKe I10,!lp06Hee 6y,!leT l13JIO)l(eH B pa3,!le.11e 11;
• aKTttBwpyeT (qepe3 Ras-IIYTh) cfloccflorrpoTeMHcPOCcParn3y rpaHyJT f.1111KOreHa, KOTopa5I ,!lecfloccflop11JT11pyeT f.11MKOreHCl1HTa3y 11 TaKl1M o6pa30M ee aKTHB11pyeT. KpoMe TOro, cfloccflorrpoTettH<Poc<Parn3a ,!le<Poccflop11n11pyeT 11, CJie,!l.OBaTeJibHO, HHaKTHBHpyeT Kl1Ha3y cfloccflopI1.11a3hl M rnttKoreHcfloccflopHJia3y;
• HH)J.yUMpyeT CHHTe3 f.11lOKOKHHa3bl, TeM caMhIM ycKop5I5I <PoccflopHJI11poBaH11e f.11IOK03hI B KJieTKe. C.11e,!lyeT HarroMHl1Th, 'ITO pery.115ITOPHhIM cflaKTOPOM B MeTa60JII13Me fJIHKOreHa 51BJT5IeTC5I TaK)l(e BeJiwrnHa Km f.11lOKOKHHa3bI, KOTopmi. MHOro Bhillle, '!eMKm reKCOKI1Ha3hI. CMhICJI 3TIIX pa3IIH'IHHIlOH5ITeH: rre'IeHbHe ,!l.OJI)l(Ha IlOTPe6JI5ITh fJilOK03y )JJl5l Cl1HTe3a f.IJHKOfeHa, eC.1111 ee KOJIH'IeCTBOB KPOBH B rrpe,!leJTax HOpMbl.
Bee 3TO BMecTe rrp11Bo;::i:11T K ToMy, 'ITOMHCyJIHH O,!l.HOBpeMeHHO aKTMBttpyeT rJIHKOreHCMHTa3y l1
11ttr11611pyeT rn11KoreH<PoccflopttJia3y, rrepeKJIIO'IaH rrpouecc Mo6mrn3au11w rnMKoreHa Ha ero CMHTe3.
325
rmoKaroH
\
G-6e.noK
---;;{
r.Q<i> |
~M<l>----~ |
AT<l> |
• |
|
:G> |
3 |
|
j |
|
|
|
|
|
|
|
|
|
|
|
|
|
:G> |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
|
|
|
|
|
|
|
|
|
|
|
|
|||
|
00 |
|
|
|
|
|
|
|
|
|
|
|
||||
|
|
nKA |
|
|
|
|
|
|
|
|
|
|
|
|
||
|
|
8KTVIBH8Sl |
|
|
|
|
|
|
|
|
|
|
|
|||
|
|
I |
|
I |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
|
I |
|
|
|
|
|
|
|
|
|
|
|
|
rnvtKOreHCVIHT838 |
|
I |
|
I |
|
|
|
|
|
|
|
KvtHa3a |
|
|
|
|
|
I |
|
I |
|
|
|
|
|
|
|
|
|||||
8KT. |
|
I |
|
I |
|
|
|
|
|
¢oc¢op1.-ma3b1 |
|
|||||
|
|
I |
|
I |
|
|
|
|
|
|
|
HeaKT. |
|
|
|
|
|
|
I |
|
I |
|
|
|
|
|
|
|
|
||||
|
|
I |
|
I |
|
|
|
|
|
|
|
|
|
|
|
|
6 ~---------------' |
|
'----------- ----------fl 4 |
||||||||||||||
~---~® |
|
|
|
|
® |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
KvtHa3a |
|
|
|
||||
rnvtKOreHCVIHT838 |
|
|
|
|
|
|
|
|
|
|
|
|||||
HeaKT. |
|
|
|
|
|
|
|
|
|
¢oc¢opvtna3bl |
|
|||||
|
|
|
|
|
|
|
|
|
|
|
|
8KT. |
|
|||
|
|
|
|
|
|
|
|
|
|
|||||||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
® |
|
|
|
|
||
|
|
|
|
|
|
|
|
|
II |
|
|
|
|
|||
|
|
|
|
|
|
|
|
|
I |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
1 |
|
|
|
|
|
|
|
||
|
|
|
|
|
|
|
|
|
rnvtKOreH¢loc¢lopvtna3a |
|||||||
|
|
rnvtKOreHcPOCcPOpvtn8381--_•____. |
||||||||||||||
|
|
|
|
|
HeaKT. |
5 |
|
|
|
8KT. |
||||||
|
|
|
|
|
|
---- |
|
|
|
|
|
|
|
|||
|
|
|
|
|
|
|
|
|
|
|
|
|
||||
|
rnvtKoreH ~-i=-n-;;K~30-1-P-----+ rmoK030-6-P-----+ rnlOK03a |
|||||||||||||||
|
|
|
|
|
7 |
|
|
|
|
|
|
|
|
|
|
|
PMC. 7-29. Perynsn"'MSICMHTe3a ... paCn8A8 rnMKOreHa B ne<teHM rnt0KarOHOM ... aApeHanMHOM. 1 - rmoKaroH Iii aAPeHanlilH B3alilMOAeVicTBYIOT co cne1..1111cpw-1ecK111M111 MeM6paHHblMlil pe1..1emopaM111. KoMnneKc ropMOH-pe1..1emop 0n111sier Ha KOH<f>OPMal.llillO G-6enKa, Bbl3b10aS1 AlilCCOl..llilal..llillO ero Ha npOTOMepbl Iii 3aMeHy B a.-cy6beAlllHlill..le rJ:l<f:> Ha rT<f:>; 2 - a.-cy6beAlilHlill..la, CBSl3aHHaSIC rT<f:>, aKTlilBlilpyeT aAeH111nar1..1111K11a3y, Karan1113111pyt01Uyt0 c111Hre3 l..IAM<t> 1113 AT<t>; 3 - B np111cyrcr0111111 l..IAM<t> npore111HK111Ha3a A (l.1AM<t>-sa0111c111MaS1) o6paTlilMO A111cco1..1111111pyer, oc0060JKAaS1 06naAat0U1111e Karan111rn4ecK0Vi aKrnBHOCTblO cy6beAlilHlill..lbl C; 4 - npore111HK111Ha3a A cpoccpop111n111pyer 111 aKrn0111pyer Kli1Ha3y cpoccpop111naab1; 5 - Kli1Ha3a cpoccpop111naab1 q:iocq:iop111n111pyer rn111KoreH<f>occpop111na3y, nepeBOASI ee B aKrnBHYIO
cpopMy; 6 - npore111HK111Haaa A cpoccpop111n111pyer ratoKe rn111KoreHc111Hraay, nepeBOASI ee B HeaKrnBHoe cocTOS1H111e; 7 - |
B peaynbrare |
111Hr1116111po0aH111S1 rn111KoreHClilHTa3bl 111 aKrn0a1..1111111 rn111KoreH<f>occpop111na3b1 rn111KoreH BKnt04aercS1 B npo1..1ecc pacnaAa; 8 - |
cpoc<f>oA1113cre- |
pa3a Karan111a111pyer pacnaA l..IAM<t> 111 reM caMblM npepb10aer AeVicr0111e ropMoHanbHOro c111rHana. KoMnneKc a.-cy6beAlilHlilL1a-rT<t> 3areM pacnaAaercsi, a.-, ~- 111 y-cy6beAlilHlill..lbl G-6enKa peacco1..1111111pyt0rcsi.
326
A,QpeHa111<1H
l |
|
|
|
|
e |
a1-Peu,emop |
<l>oc¢0111-1na3a C |
npOTel<1HKl<1HB3B C |
|
|
||||
|
|
|
|
|
|
G-6e110K |
|
|
|
|
-----1--,1 |
|
|
MeM6paHa |
|
|
|
|
|
|
I |
|
|
|
|
|
|
|
'--------~ |
|
||
I, 4_C_a_2+___K_a_,~-b-M_o_A_Y_11_1-1___H |
L |
|
|
|
7 |
|
|
|
Ka11bMO,Qy111-1H- |
|
|
||||
__.I |
-- - -;-- --. ~-3_a_e_1-1_c_1<1_M_a_R_n_K__, |
- - - - - - .. |
|
||||
I |
|
|
|
|
7 |
|
|
: 5 |
|
|
|
|
|
|
|
I |
|
|
|
|
/// /-- |
|
|
I |
|
|
|
|
|
||
w |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
K1<1Ha3a |
|
|
|
|
|
// |
|
¢oc¢op1-111a3b1 |
|
|
|
/// |
|
|
|
|
|
|
----- |
|
~r-11_1-1_K_o_re-H~c-1<1-HT_a_3_a_ |
||
HeaKT. |
|
|
|
|
|||
|
|
|
////// |
|
aKT. |
||
|
|
|
|
|
|||
p |
|
// |
7 |
|
|
|
|
K1-1Ha3a ¢oc¢op1-111a3b1 aKT.
///
//
//
///
I |
|
I |
6 |
I |
I
rJ11<1KOreHcpoccpop1<111a3a HeaKT.
I w
r111<1KOreHcpocQ:>op1<111a3a
aKT.
|
~/ |
|
|
r1111KoreH---.. rmoK030-1-P---.. |
rmoK030-6-P---.. rmoK03a |
P14c. 7-30. |
Perymn~1411 Cl4HTeaa 14 pacnaAa rnl4KoreHa e ne'leHl4aApeHanl4HOM 14 Ca2•. |
<t>V1<l>2 - cpoccparns:1vm11Hos11ron611ccpoc- |
cpaT; V1<ll3 - |
11H0311TOn-1,4,5-Tp11cpoccpaT; .QAr - s:111a411nrn114epon; 9P - SHAOnnasMaT11LtecK11~ peT11KynyM; <DCcpoccpos:111T11nce- |
|
p11H. 1 - esa11Mos:1e~cTe11e ai:1peHan11Ha c a,-pe4emopoM TpaHccpopM11pyeT c11rHan Ltepes aKrnea41110 G-6enKa Ha cpoccpon11nasy C, nepeeos:1si ee e aKrneHoe cocTOS1H11e; 2 - cpoccpon11nasa C rns:1pon11syeT <till1<ti2 Ha lll<ti, 11 .QAr; 3 -111¢3 aKrne11pyeT Mo611n11sa41110
2 |
9P; 4 - |
2 |
.QAr 11 cpoccpos:111T11ncep11H aKrne11py10T np0Te11HK11Hasy C. np0Te11HK11Hasa C cpoccpop1·m11pyeTrn11KoreHCl1HTa- |
Ca • 113 |
Ca •, |
sy, nepeBOASI ee 8 HeaKTl1BH00 COCTOSIHl1e; 5 - KOMnneKC 4Ca2•-KanbMOAyn11H aKT11811pyeT Kl1Ha3y cpoccpop11nasbr 11 KanbMOAyn11Hsae11C11Mble np0Te11HK11Hasb1; 6 - K11Hasa cpoccpop11nasbr cpoccpop11n11pyeT rn11KoreHcpoccpop11nasy 11 TeM caMblM ee aKrne11pyeT; 7 - aKTl1BHble cpopMbr Tpex cpepMeHTOB (KanbMos:1yn11Hsae11c11Masi np0Te11HK11Hasa, K11Hasa cpoccpop11nasbr 11 np0Te11HK11Hasa C) cpoccpop11n11py10T rn11KoreHCl1HTasy 8 pa3nl1'iHblX40HTpax, nepeBOASI ee 8 H0aKTl1BHOe COCTOSIHl10.
327
B neqeH11 cyruecTByeT 11 arrJiocTep11qecKa5l peiym1um1 rn11Korempoc¢opttna3hI, 06ecneq11BmomaH BHYTPl1KJieTOqHbie noTpe6HOCTl1 B rmOK03e, HO ropMOHarihHhie Cl1rHarihl 11MelOT np11opHTeT HarI BHYTp11KJieTOqHhIMl1 11 npecJieJJ.yIOT JJ.pyrne ¢113110JiornqecK11e ueJIH. Pattee (cM. pa3,!1.eJJ 6) paccMaTp11Barroch 3Haqett11e 113MeHett11H B KJieTKe ypoBHetl: AT<l>, A,ll<l> 11 AM<l> KaK noKa3aTeJIH, OTpIDKaiomero noTpe6ttoCTM KJieTKM B ::mepm11. 3aMe,!J.Jlett11e YTMM3aU1111 AT<l> conpooo)l(llaeTCH CHIDKeH11eM aKT11BHOCT11 rn11KOreH¢oc¢opMJia3hI l1 yMeHhUlett11eM CKOpOCTl1 pacnaua rn11KoreHa. HanpoT11B, YBeJIHqem1e pacxo,!J.o- BaH11H AT<l> BeJJ.{h K nOBhrwett1110 ypoBHH AM<l>, aKnrnauirn rn11Korett¢oc¢opttna3hI 11 ycKopeH1110 pacnaua rn11KoreHa. AT<l> 11 AM<l> HBJIHIOTCH aJIJIOCTep11qecK11M11 3¢¢eKTOpaMM no OTHOWeHl1lO K rn11KoreH¢oc¢opMJia3e. CymecrnyeT TaIOKe 11 MeTa60JI11qecK11ti: KOHTpOJih aKTl1BHOCTl1 rJIMKOreH¢oc¢o- PMJia3hl. TaK, np11 nOBhIUleH1111 KOHUeHTpaUMM rmo- K03o-6-¢oc¢arn aKTl1BHOCTh 3TOro ¢epMeHTa B KJieTKax neqeHM CHIDKaeTCH.
r. PErYJrnuIDI METABoJIH3MA rnHKornHA
B MhllllUAX
PeiymruMH o6MeHa rn11Koretta B Mhrwuax 06ecneq11BaeT 3HepreT11qecKMM MaTepMaJIOM KaK l1HTeHCl1BHylO pa6oTy MhIWLI, (6er 11Jil1 6oph6a), TaK 11 3Hepro3aTpaTbl B COCTOHHl111 nOKOH.
B 3KCTpeMa.rthHbIX CH'fYaum1xB MhillieqHhIX KJieTKax M06HJIM33UMH rJIMKOreHa ycKOp51eTCH aupettaJil1HOM. CBH3hIBaH11e a,upettaJIMHa c P-peuenrnpa- M11, accou1111poBaHHhlMl1 c a,!1.eH11JiaTU11KJia3HOH Cl1CTeMOH, npl1BO,!J.11T K o6pa30BaHl1lO uAM<l> B KJieTKe, a 3aTeM ¢oc¢op11m1p0Batt1110 11 aKTMBau1111 Kl1Ha3hI ¢oc¢op1vrn3h1 11 rn11Korett¢oc¢op11Jia3hI (p11c.
06pa30Batt11e uAM<l>, cT11MyJI11poBaHHoe MpeHaJIMHOM, CJIY:)l<HT CMfHarIOM K yBeJI11qeHl1lO npo113BOJJ,CTBa 3Hepr1111 B pe3yJihTaTe ycKopeHMH pacmeTIJiett11H rn11Koretta. l1MeHHO B xo,!J.e pacnaua, o6pa3oBattttoro 113 rn11Koretta rn10Ko3o-6-¢oc¢a- Ta, c11me311pyeTcH A T<l>.
l1ttaKT11Bauiu1 rn11KoreHCl1HTa3hI no,LJ. BJIMHHHeM aupeHaJil1H3 B MhiweqHhIX KJieTKax npOXO,Ll.11T TaK )[(e, KaK 11 B neqeHM.
B COCTOBHHH UOKOB np11 HH3KHX KOHUeHTpaUH51X a,!1.peHarr11Ha B KpOBM rJIHKOrett¢oc¢opHJia3a MhllllU HaxO,!J.HTCH B LJ,e¢oc¢opttn11poBaHHOM - HeaKTl1BHOM COCTOHHl1l1 (¢opMa B), HO pacna,!J. rJil1KoreHa BCe-TaKl1 npOMCXO,Ll.MT. 3To o6bHCHHeTCH TeM, qTO
rn11Korett¢oc¢opH.Jia3a aKT11B11pyeTCH cnoco6oM, He CBH3aHHhIM C ee cPOCcPOPl1JI11pOBaHl1eM, TaK KaK ypoBeHh uAM<l> B KJieTKe Hl13Kl1i1. B JJ.aHHotl cHTyau1111 np011CXO,Ll.11T aJIJIOCTep11qecKa5l aKT11BaUl1H rn11KOreH¢oc¢op11Jia3bl B. AKT11BaTopaMl1 ¢epMema cJI~aT AM<l> 11 H 3P04 , 06pa3y10ml1ecH B KJieTKe np11 pacnaue AT<l> (p11c. 7-31, TIYTh 1).
Ilpu )'MepeHHhIXMhimequbIX c0Kpa111euusx, T.e. B
c11Tyau1111, He Tpe6yioweti: yqaCTMH B peryJIHU1111 uAM<l>, aJIJIOCTepl1qecKl1M cnoco60M aKTMBl1pyeTCH Kl1Ha3a ¢oc¢opH.Jia3hI (pttc. TIYTh 2). B ,!J.aH-
HOM CJiyqae aJIJIOCTep11qecKl1Ml1 3¢¢eKTopaM11 CJiy- )!(aT 110Hhl Ca2+, KOHUeHTpau11H KOTOpblX pe3KO 803pacrneT npl1 COKpaweHMl1 MhIWU B OTBeT Ha Cl1rtta.JI OT ,LJ.BMraTeJihHOro ttepBa. AKTl1BHOCTh ¢epMeHrn CHl1)[(3eTCH cpa3y )[(e, KaK TOJihKO KOHUeHTpaUMH Ca2+ B KJieTKe yMeHhrnaeTcH nocJie nocryTIJiett11H c11rttana K paccna6Jiettl1IO MhillIU. TaK11M o6pa30M, poJih 110HOB Ca2+ 3aKJiioqaeTCH He TOJihKO B 11H11Ul1au11l1 Mhlllieqttoro COKpaweHl1H, HO TaK)[(e B 06ecneqeH1111 ero 3Hepro3aTpaT.
AKTl1BauH51 KHHa3hI ¢oc¢op11Jia3hI c noMOlllhIO MOHOB Ca2+ onocpe,!J.oBaHa KarihMOL\YJil1HOM. Kanh-
MO,LJ.YJIMH B ,!J.aHHOM CJiyqae - npoqHo CBH3aHHaH
cy6beJJ.l1H11ua ¢epMema (p11c. MhrweqHaH
Kl1Ha3a ¢oc¢opttna3hl COCTOl1T 113 cy6be,!J.11H11U 4 Tl1TIOB: a, p, y l1 0, 06be,!J.11HeHHhIX B KOMn.JieKC. <t>epMeHT BKJIIOqaeT 4 TaKHX KOMrIJieKca. KaTarrl1T11qecKOH aKTl1BHOCThIO 06.Jia,!J.aeT y-cy6be,!J.11Hl1ua. Cy6be- ,LJ.HH11UhI a 11 pBhinOJIHHlOT peiyJIHTOPHYIO cPYHKU11lO. Ott11 co,!J.ep)[(aT ocrnTKM cep11Ha, ¢oc¢opHJI11pyeMb1e
CT K A. O-Cy6be,!J.MHl1LJ,a CBH3bIBaeT 4 110Ha KaJihUl1H; otta HD,ettT11qHa 6e.JIKY KMhMO,!J.yJI11Hy. CBH3hrnaH11e MOHOB KarihLl,MH Bbl3bIBaer KOH¢opMaU110HHhie 113MeHeHl1H, qTo npMBO,!J.HT K aKTHBaUHI1 KaTaJil1T11qecKOro ueHTpa y-cy6be,!J.11Hl1Uhl, XOTH MOJieKYJia OCTaeTCH B ,LJ.e¢oc¢opMJIMpOBaHHOM COCTOHHl1l1.
B ecJJ11 OH co-
Bna,!J.aeT c COCTOHHMeM TIOKOH, np011CXO,!J.11T CTl1MYJIHLJ,HH Cl1HTe3a rnMKoreHa. MbrIIIe<rnaH pa6orn Bo BpeMH n11meBapett115J 3aMe,!J.JIHeT npouecc c11ttTe3a rJIMKOreHa, TaK KaK np11 3TOM MhIWUhl 11CnOJih3YIOT ,!J.JIH OKMCJieHMH rJilOK03Y KpOBl1, nocTyna10wy10 113 KMrneqttnKa.
B nepeKJI10qett1111 Mo611JI113aun11 rn11KoreHa Ha 3anacaHMe rnIOK03hI yqacrnyeT 11HcyJI11H. KaK y)[(e rOBOpMJIOCh, rJilOK03a TIOCTynaeT B MhIWeqHbie l1 )[(MpOBbie KJieTKM c TIOMOlllhlO rJilOK030-Tpattcnop- TepOB [ J1 lOT-4. TpattcnopTepbI B OTCYTCTBl1e 11Hcy.JI11Ha ttaXOMTCH B u11TonJia3Me KJieToK, 11 rnioKo3a KJieTKaMl1 He HCTIOJih3yeTCH, TaK KaK B MeM6-
328
®
|
|
|
( H~p;~b~~-~'1 |
|
|
|
|
/ --------- ....... \ |
||||||||||
|
|
|
|
|
|
|
|
( A,QpeH8I111H : |
||||||||||
|
|
|
|
11Mnynbc |
1 |
|
|
|
|
|
|
|
|
|
|
|||
CapKonnaaMaT114eCK11~ |
|
|
|
|
|
|
|
|
|
|
|
'------ r-- I |
||||||
|
'-------- |
|
|
|
|
|
|
|
|
|
|
|
||||||
pen1KynyM |
Ca2• |
|
|
|
|
|
|
|
|
|
|
1 |
|
|
||||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
||||
|
|
|
|
|
|
|
|
|
|
|
|
|
" |
|
|
|||
|
|
|
|
\ |
|
|
|
|
|
|
|
|
|
|
|
|
||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
|||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
1..1AMct> |
||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
I |
||
|
|
|
|
Ca2• |
|
|
|
|
|
I |
||||||||
|
|
|
|
|
\ |
|
|
|
|
|
|
|
../ |
|
|
|||
|
|
|
|
|
|
|
|
|
/ |
/ |
|
|
|
|||||
|
|
|
|
|
|
|
|
|
|
|
..I |
|
|
|
nKA |
|||
|
|
|
ATC!> |
( |
4 Ca2• ~KaJ1bMO.Qyn11H ) |
I |
|
|
|
|
||||||||
|
|
|
|
|
|
|
|
|
|
|
|
I |
|
|
|
|
||
|
|
|
|
|
|
|
|
|
|
|
I |
|
|
|
|
|||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
K11H838 |
|
|
|
® |
|
|
||
|
|
|
|
|
|
|
|
cpoccpop11naab1 |
|
|
||||||||
|
|
|
|
|
|
|
|
|
|
8KT. |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
ATC!> |
A,Qct> |
|
|
|
|
|||
CoKpaL1.1eH11e |
|
|
|
|
|
|
||||||||||||
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|||
MblWLI |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
||
|
|
|
|
|
|
|
ln11KoreH- |
|
|
|
|
ln11KoreH- |
||||||
|
|
|
|
|
|
|
cpoccpop11naaa B |
|
|
cpoccpop11naaa A |
||||||||
|
|
|
|
|
|
|
HeaKT. |
|
|
|
|
|
|
8KT. |
|
|||
Pi
PMc. 7-31. AKTMB81..1MSI rnMKoreH<j>oc<j>opMnaab1 Mb1w1..1. 1 - annocrep1114ecKasi aKr111sa1..1111si rn111KoreHcjJoccjJop11naab1 B. B npo1..1ecce Mb1We4Horo coKpa111eH111S1 npo111cxoi:1111r paapyweH111e AT(J) c 06paaoBaH111eM AM(J), Koropb1~ sisnsiercsi annocrep1114ecK111M aKTlllBaropoM rn111KoreHcjJoccjJop111nasb1 B; 2 - HepBHbl~ 111Mnynbc 111H1111..1111111pyer ocso6o)KJ:1eH111e Ca2+ 1113 capKonnasMar1114ecKoro pernKynyMa. Ca2+ 06paayer KOMnneKc c KanbMoi:1yn111HOM, cnoco6Hbl~ aKr111s111posarb KlllHasy cjJoccjJop111nasb1; 3 - aKr111sa1..1111si rn111KoreHcjJoccjJop111nasb1 ai:ipeHan111HOM 4epe3 ai:ieH111naTL1111Kna3HYIO ClllCTeMy.
paHe HeT 6eJIKOB-rrepeHOC1-111KOB. HHcymrn cn1- M)'IIttpyeTrrepeMeW,eHtte fJUOT-4 l1 BCTpattBaHHe ttx B MeM6paHy KJieTOK. MexaHtt3M rrono6Horo BJIH -
5!Hl151 l1HCYJIHHa l13y'!eHHe,UOCTaTO'IHO, HO orrpe-
,UeJieHbI ero OCHOBHbie 3TaIThI. Uerrh co6bITHH rrpH
CTl1MYJIS:lLl,Hl1 HHCYJIHHOM rroTpe6JieHHH fJIIOK03bI
MblllIU,aMH l1 )l(HPOBblMH KJieTKaMtt BblfJIH,llHT CJie-
JlYIOW,l1M o6pa3oM:
• |
peu.emop |
l1HCYJIHHa (IR) - l1HCYJil1HCTl1M)'- |
|
Jil1pyeMa51 THp03l1HOBa51 rrpoTel1HKIIHa3a - o6H- |
|
|
3aTeJibHbltt rrocpe,llHHK BCex netl:CTBHH l1HCym1- |
|
|
Ha (cM. pa3neJI 5); |
|
• |
aKTHBttpoBaHHhIH HHCYJIHHOM IR cpoccpop11JI11- |
|
|
pyeT crre11,ttcptt'leCKtte U.ttTorrJia3MaTtt'lecKtte |
|
|
6eJIKH - |
cy6cTPaTbl l1HCYJIHHa (IRS); |
•cpoccpopl11II1pOBaHHbltt cy6CTPaT (B OCHOBHOM
IRS- I) coe,UHHHeTC51 c cpoccpaTI1,UI1JIHH0311TOJI-
3-KI1Ha30ll (<I>H-3-KHHa3a) l1 aKTHBHpyeT 3TOT
cpepMeHT;
€aKTl1BHaH <I>H-3-K11Ha3a KaTaJIH3ttpyeT cpoccpopttJIHpoBaHHe ITO IT03I111,l1l1 3 PH!l KOMITOHeHTOB I1H03HTOJicpoccpaTHOtt CHfHaJihHOtt CHCTe-
MhI, rrpHBo,UHw,etl: K CTHMYJIHU:HH TPaHcJI0Kau;1111 f JIIOT I13 U,I1T030JIH B ITJia3MaT11'1eCKYIOMeM6patty;
• fJIIOK03a c ITOMOW,b!O fJilOT-4 rroczyrraeT B MbIllle'IHbieKJieTKH l1 BKJIIO'!aeTCHB CI1HTe3 fJIHKOretta.
BJIHHH11e 11ttcyJI11tta Ha cKopocTh CHHTe3a rnttKoreHa B MhIIII11,ax ocyw,ecTBJIHeTC51 rrocpe,UCTBOM I13MeHeHl15l aKTHBHOCTH fJil1KOreHCl1HTa3hl l1 fJII1KoreHcpoccpopHJia3bl - KJIJO'leBbIX cpepMeHTOB, 0 qeM ~e fOBOPHJIOCb rrpM o6cy)l(JleHl1l1 BJil1HHl1H HHCYJIHHa Ha Mera60JIH3M fJIHKoreHa B rre'leHH.
329
|
cpoc¢opi-ma3bl |
|
|
H08KT"1BH8~ |
|
|
Ca2+ |
ca2+ca2• |
|
~,g~~ |
|
|
~~ |
|
K1-1Ha3a ¢occpop11111aab1 |
K11Ha3a cpoc¢opKna3bl |
|
cpoc¢opHJ1111poaaHHa~ |
Hecpoc¢op111111o1poeaHHa~ |
|
3Kn1BHas! |
aKT1o101o1poaaHa Ca2+ |
|
A |
6 |
|
P11c. 7-32. Perymn-111si aKTllBHOCTll KllH83bl cj>occj>op11na3bl. <l>epMeHT COCTOlllT 1113 4 111i:1eHTl'1'iHblX6enKOBblX KOMnneKCOB. KIDK,Qbll1 KOMnneKc coi:iep:>K111T 4 pa3HblX cy6bei:1111Hllll..lbl o:, ~. y, S. Ha p111cyHKe noKa3aH oi:1111H 1113 TeTpaMepos. Karan111Tw-1ecKoi1 aKTlllBHOCTbto oonai:iaeT y-cy6beAlllHlllL1a. a- Ill ~- npOTOMepbl BblnOnH5llOT peryn51TOpHyto <PYHKl..lllllO, OHlll cpoccpop111n111pytoTC5I np111 y'iaCTllllllnK A. Kanb-
Moi:1yn111H - |
S-cy6bei:1111H1111..1a, npO'iHOCB513aHHa51 c cjlepMeHTOM. A - aKT111sa1..111151 KlllHa3bl cpoccpop111nasb1 B pesynbrnre cpoccpop111n111posa- |
Hlll5I; 6 - |
aKrnsa1..111151 KlllHa3bl cpoccpop111nasb1 nocne np111coei:1111HeH1115I Ca2• K KanbMOi:1yn111Hy. |
,II;. IIAPYIIIEHHH OBMEHA rJIHKOrEHA
fJIHKOreHOBbie 6oJie3HH - rpyrrrra HaCJie,ll;CTBeHHhIX Hapyrnem1ti, B OCHOBe KOTOPhIX Jie:)IGff CHMJKem1e MJIH OTCYfCTBI-le aKTHBHOCTH cpepMeHTOB, KaTaJIH3HPYJOmMX peaKUHH CHHTe3a MJIH pacrra,ll;a rnrrKoretta, m160 ttapyrnettrre peryJUIUHH 3TMX cpepMeHTOB.
I. I'AuKozeuoJbl - 3a6oneBamrn, o6ycnoBJieHHhie ,ll;ecpeKTOM cpepMeHTOB, yqacrnyIOII(HX B pacna.ue fJIHKOretta. 0HH Ilp051BJUIIOTC51 MJIH Heo6h1q- 'ttotiCTPYKTYPOM rm1:Koretta, HJIH ero H36bITOY:HbIM HaKOIIJieHHeM B rreqeHH, cep,ll;eqHOM HJIM CKeJieTHhIX MhIIIIUax, rroqKax, JiefKMX H Ll:PYfMX oprattax.
B Ta6JIHUe 7-3 OIIHCaHbI HeKOTOpbie THIIbI fJIHKOreH030B, pa3JIJiqaIOII(HXC51 xapaKTepoM H JIOKaJIH3autteti cpepMeHTHoro L1:e$eKTa.
Cne,ll;yeT OTMeTHTh, qTo TepMHH «fJIMKoretto3» 6hm BrrepBhie rrpe,IJ,JioJKeH K.<I>. Kopw H LT. Kopw. Otttt )J(e rrpe,IJ,JIOJKHJIM CMCTeMY ttyMepauwtt 3TMX 6one3Heti. 0,ll;HaKo B HaCTOHmee BpeMH rrpeo6na- ,ll;aeT ,ll;eJiettwe rnwKoreH030B Ha 2 rpyrrrrh1: rreqeHOY:Hhie H MhIIIIelJ:Hbie.
Ile11eHO'IHbiect>opMbl fJIHKOfeH030B Be,ll;yr K ttapyIIIeHHIO HCI10Jih30BaHM51 fJIHKOretta Ll:JUI IIOMep- )J(aHH51 ypoBH51 rnIOK03hI B KpOBM. Tio3TOMY 06rnwti CHMIITOM ,IJ,JI51 3THX cpopM - fHI10fJIMKeMH51
B rrocrn6cop6THBHhIM rrepMO,ll;.
EoJie3Hb fupKe (THII I) OTMeqa10T ttaw6onee qacTo. OrrttcaHHe OCHOBHhIX CMMIITOMOB 3T0f0 THrra fJIHKOfeH03a H MX rrpttqJiH MO)J(eT CJIY- )J(lfTb OCHOBaHMeM Ll:JUI I10HMMaHM51 CMMIITOMOB Bcex OCTaJihHhIX TMIIOB. TipttqMHa 3T0f0 3a6oJieBaHH51 - HaCJie,ll;CTBeHHhIM ,ll;e$eKT fJIIO- K030-6-cpocct>aTa3hI - cpepMeHTa, 06ecrreY:H- Ba10mero BhIXO,ll; fJIIOK03hl B KpOBOTOK IIOCJie ee BbICB060)J(Ll:eHM51 H3 fJIMKOfeHa KJieTOK rre- Y:eHH. Eone3Hb fttpKe rrpo51BJUieTCH rwrrornwKeMweti, rttrrepTpMaUHJirJIMUeponeM11eti (rroBhIrneHMeM CO,ll;ep)J(aHMH TpMaUMJifJIMU,epoJIOB), r11rrepypMKeMtteti (IIOBhilIIeHMeM CO,ll;ep)J(aHH51 MOqeBOM KHCJIOTbl).
fHIIorJIHKeMHH - cne,ll;CTBMe ttapyrneHH51 peaKUMH o6pa30BaHM51 CB060,ll;HOM fJIIOK03hl H3 rnIOK03o-6-<l>oc<t>arn. KpoMe Toro, BCJie,ll;cTBrre ,ll;e<t>eKTa fJIIOK030-6-cpoc<t>aTa3hI rrpOMCXO,ll;HT
330
Ta6m11.1a 7-3. XapaKTep11cT11Ka HeKOTOpblX rn11KoreHOBblX 6one3HeM
|
|
|
|
r JIHKOreH03bl |
|
|
|
|
|
|
|
|
clJopMa |
i:.eclJeKTHblH clJepMeHT |
npOHBJieHHH 6oJie3HH |
Tun, uaJeauue |
|
|
rJIHKoreuoJa |
|
|
|
6oJie3HH |
|
|
|
|
|
|
|
IleqeHO'IHaH |
f'moKOJo-6-<l:>oc<l:>aTaJa |
f'ImornHKeMHH,rm1epa- |
I |
|
|
|
|
|
QHITfITHQeporreMHH,rH- |
Eorre3Hh f'ttpKe |
|
|
|
|
rrepypHKeMHH,aQH,[(03 |
|
|
|
|
|
(BCITe,[(CTBHe HaKorrrreHHH |
|
|
|
|
|
rraKTaTa),xapaKTepHoe |
|
|
|
|
|
Bhipa)KeHne ITHQa («ITHQO |
|
|
|
|
|
|
|
|
|
|
|
KHTaHCKOH KYKIThI» ). |
|
|
|
AMHrro- l ,6-rnioK03H,[(a3a |
HaKorrrreHne rrrnKoreHa c |
III |
|
|
|
(«,[(eBeTBHmHii:» qiep- |
KOpOTKHMH BHellIHHMH |
Eorre3Hh <l>op6ca-KopH, ITHMHTO- |
|
|
|
||||
|
|
MeHT) |
BeTBHMH (rrHMHTO- |
,[(eKCTPHH03 |
|
|
|
|
|
,[(eKcTpnH). OcTaJihHhie |
|
|
|
|
|
rrpoHBITeHHH MeHee |
|
|
|
|
|
Bhipa)KeHhl, qeM rrpH THITe |
|
|
|
|
|
I. |
|
|
|
AMHIT0-1,4~1,6 |
HaKorrrreHHe cTpyKTypHo |
IV |
|
|
|
||||
|
|
rrr10KOJHITTPaHc<l:>epa3a |
H3MeHeHHOro rJIHKOreHa c |
Eorre3Hh AH):(epceHa |
|
|
|
(«BeTBHmHH» qiepMeHT) |
O'leHh,[(ITHHHhlMH |
|
|
|
|
|
|
Hapy)KHhlMH BeTBHMH H |
|
|
|
|
|
pe,[(KHMH TO'IKaMH |
|
|
|
|
|
BeTBITeHHH. |
|
|
|
<l>oc<l:>opnrraJa |
HaKorrrreHne rrrHKoreHa |
VI |
|
|
|
|
|
HOpMa.JihHOH CTPYKTYPhI. |
Eorre3Hh Xepca |
|
|
|
|
y MepeHHaH rHITOfITHKe- |
|
|
|
|
|
MHH, rerraToMera.rrHH, |
|
|
|
|
|
KITHHH'leCKHerrpOHBITeHHH |
|
|
|
|
|
ITOXO)KH, HO MeHee |
|
|
|
|
|
Bhipa)KeHhI, 'leMrrpH |
|
|
|
|
|
rJIHKoreHmax I H III |
|
|
|
|
|
THITOB. |
|
|
|
|
|
|
|
|
|
KnHaJa <l:>oc<l:>opnrraJhI |
AHa.JIOrH'IHhlVI THIIY |
IX |
|
|
|
TipoTeHHKHHa3a A |
AHa.JIOrH'IHhlVI THIIY |
x |
|
|
|
||||
|
|
|
|
v |
|
|
Mb1111e'IHh1e |
f'rrnKoreH<I:>oc<I:>opttrraJa |
Eorrn B MhillIQax, |
||
|
|
|
|
cy):(oporH rrpH |
Eorre3Hh |
|
|
|
|
<l:>H3H'leCKOHHarpy3Ke |
MaKAp):(rra |
|
|
|
|
():(a)Ke yMepeHHOH). |
|
|
|
|
|
HaKorrrreHne B MhIIIIQax |
|
|
|
|
|
rrrnKoreHa HOpMarrhHOH |
|
|
|
|
|
CTPYKTYPhl. |
|
|
|
<l>oc<l:>o<l:>pyKTOKHHa3a |
AHarrom'IHhIV Tnrry |
VII |
|
|
|
<l>oc<l:>orrrnQepoMyrn3a |
AHarrorn'IHhIV Tnrry |
|
|
|
|
JiaKTaT,[(em,[(poreHaJa |
AHarrorn'IHhIV Tnrry |
|
|
|
|
(M-rrpOTOMep) |
|
|
|
|
CMe111aHHh1e |
J1H30COMHaH a-1,4- |
f'eHeparrtt30BaHHOe |
II |
|
|
|
rITHK03H,[(aJa |
HaKorrrreHne rrrHKOreHa B |
Eorre3Hh IloMrre |
|
|
|
|
|
ITH30COMaX, a JaTeM B |
|
|
|
|
|
QHT030ITe |
|
|
|
|
|
|
|
331
HaKoTUlemre n KJieTKax neqeHH cy6cTJ)aTa - fJUOK030-6-¢oc¢arn, KOTOpbIH BOBJieKaeTC5I B npouecc Karn6oJIH3Ma, r,LJ,e OH npeBpail.i;aeTcH B nttpyBaT H JiaKTaT. B KPOBH nOBhilllaeTCH KOJIWieCTBO JiaKTaTa, II03TOMY B03MO)J(eH auH- ,11,03. B rn)J(eJihIX cnyqaHX pe3yJihTaTOM rnnorJI11KeM1111 MOryT 6bITh cy,nopon1. fMIIOfJil1KeMI15I conpOBO)K)J,aeTC5I yMeHhllleHtteM CO,LI,ep- )J(aHH5I J1HCYJil1Ha l1 CHl1)J(eH1IeM OTHOllleH1rn 11HCYJlHH/rmoKaroH, qTo, B CBOIO oqepe,LJ,h, Be,LJ,eT K ycKopeHHIO JII1IlOJII13a )KHpOBOH TKaHH B pe3yJihTaTe ,LJ,eHCTBl15I fJIIOKarotta 11 BhlXO,LJ,y B KPOBb )J(l1PHhlX KHCJIOT (CM. pa3,QeJI 8). f1UieJ>TPmmn.rrr.IIllUepOJieMID1 B03HHKaeT B pe3yJib-
TaTe CHH)J(eHl15I aKTHBHOCTH JITI-JIMTia3hI POBOH TKaHtt - cpepMeHTa, aKTHBttpyeMoro ttttcymmoM 11 06ecrreq11Ba10mero ycBoetttte
TAf KJieTKaMM )J(MpOBoti TKaHVl (cM. pa3,L1,eJI 8). funepypuKeMUH B03HHKaeT B pe3yJihTaTe CJie-
,ll,YIOlll,11X co6b!Tl1VI:
•YBeJI11q11BaIOTC5I co,n:ep)J(attMe B KJieTKax rJIIO- K03o-6-¢oc¢arn 11 ero 11crroJihJOBattwe B nettT03ocpoc¢arnoM rryrn c o6pa3oBattweM p116030-5-¢oc<Parn -- cy6cTparn ,LI,JI5I CMHTeJa nypl1HOBhIX HyKJieOTH,L\OB;
•yBeJI11q11BaeTcH o6pa30Bam1e MoqeBoti KHCJIOThI BCJie,Ll,CTBHe H36blTOqHoro CHHTe3a, a CJie,LJ,OBaTeJlhHO, 11 KaTa60JIM3Ma nypHHOBhIX HyKJieOTH,Ll,OB, KOHeqHblM rrpO,Ll,YKTOM KOTOporo 5IBJI5IeTC5I MO'-leBaHK11CJIOTa.
•CHJ1)J(aeTC5I BhIBe,LJ,eHHe MOlJeBOH KHCJIOThl
BCJie,LJ,crn11e YBeJl11lJemrn npOJlYKUM11 JiaKTaTa 11 113MeHeHl15I pH MOlJl1 B KHCJIYIO CTOpoHy, qro 3aTpy,LJ,H5IeT BhIBe,L1,ett11e ypaTOB - TPY,LJ,- ttopacrnopMMhIX COJieti MOlJeBOH KHCJIOThl.
Tip11 ,L1,11arnocT11Ke ,LJ,attttoti naToJiorn11 orrpe,LJ,e- AAIOT aKTI1BHOCTb fJIIOK030-6-¢occpaTa3bl B 6110ITTaTax rreqemi:. KpoMe Toro, 11cn0Jlh3YIOT TecT co CTl1MYAAU11eti fJIIOKarOHOM 11Jll1 a,Ll,peHaJIMHOM, KOTOpbIH B CJlyqae 6oJie3Hl1 ,LJ,aeT O'fP11UaTeJihHhlH pe3yJihTaT, T.e. ITOCJie l1HbeKU1111 ropMOHa ypoBeHb fJIIOK03bl B KPOB11 113MeH5IeTC5I He3Haq11TeJihHO.
JJeqeH11e COCTOl1T B orpaH11qeH1111 ynoTpe6JieHl15I rrpO,LI,YKTOB, co,L1,ep)J(am11x rnIOK03y. PeKoMett,LJ,yeTcH 11CKJlIOlJVlTb 113 LlI1eThl rrpo,LJ,yKTbl, co,L1,ep)J(am11e caxapo3y 11 JiaKTOJy, rnK xax o6pa3yIOm11ec51 113 H11X ranaKT03a 11 ¢PYKT03a rrocJie rrpeBpawett115I B rnIOK03o-6-¢occpaT Be- ,LJ,YT K ,Ll,aJlhHetiweMy HaKOITJieHl1IO fJIHKOreHa. Ll,JIH npe,LJ,OTBpamemrn rnnorn11KeM1111 11cnoJih-
3YIOT MeTO,Ll, qacrnro KOPl\1JieHl1H. 3THM MO)[(- HO rrpenyrrpe,L1,l1Tb C11MITTOMhl rnnorn11KeMl1l1. fJittKoretto3 I nma HacJienyeTc51 rro aYTOCOMHepeuecc11BHOMY nmy. Y)Ke B paHHeM nep11one Ha1160Jiee 3aMeTHhJti rrpH3HaK - renaToMeraJil15I. y 60JihHhlX ,LJ,eTeti KOpOTKOe TyJIOBHlll,e, 6oJihWOH )J(HBOT, yBeJittlJetthI noqK11. BonhHhre
,LJ,eTH OTCTa!OT B cpH3J1L!eCKOM pa3Bl1Tl111. On11cattHoe 3a6oJieBattHe Httorna o603ttalJaIOT
KaK rn11KoreH03 Tttrra Ia, TaK KaK cywecrnyeT ero pa3HOBl1Jl.HOCTh - Tl1II lb. fJIHKOrett03 lb rrpe,LJ,CTaBJI51eT co6oi1 penKo BCTpe'IaIOmy10c51 rraTOJIOrHIO, KOTOpa51 xapaKTepH3yeTC51 TeM, lJTO ,LJ,e<PeKTeH cpepMeHT TpaHCJlOKa3a fJIIOK030-6- ¢occpaTa, 06ecneq11Ba10m11ti TpattcnopT ¢oc- ¢op11J111p0Battttoti rn10K03hI B 3P. Tio3TOMy, HeCMOTp51 Ha ,11,0CTaTO<J.HYIO aKTl1BHOCTb fJIIO- K030-6-¢oc¢arn3hl, OTlllenne1-rne HeopraHtt- <J.ecKoro ¢oc¢arn 11 BhlXO,LI fJIIOK03hl B KPOBh HapyweH. KilttHH<J.eCKa51 KapTHHa fJIHKOreH03a Tttna lb rnKaH )J(e, KaK np11 rnHKoreH03e la.
EoJie3Hb Kopu (THD Ill) BechMa pacnpocTpattetta. Otta cocTaBJIHeT 1/4 Bcex CJ1yqaeB rreqetto<J.HhIX rn11KoreH030B. HaKaTIJIHBaeMhIH rnHKoreH attoMaJieH no C'fPyKType, TaK KaK necpeKTeH cpepMem aMI1JlO- l ,6-rnIOK0311,Ll,a3a, f11,L1,pOJil13YIOWl1H fJIMK0311.llHbie CBH3l1 B MeCTaX pa3BeTBJiett11ti («JJ.e- BeTBHlll,11H cpepMeHT», OT attrn. debranching
He,LJ,ocrnTOK rnIOK03hI B KpOBH npoHBJJHeTCH 6hIC'fPO, IIOCKOJibKY fJI11KOreHOJil13 B03MO)J(eH, HOB tte3Ha'mTeJihHOM06-WMe. B om11- <J.11e OT rn11KoreH03a I THrra, naKToau11no3 w rnnepyp11KeMHH He OTMeqa10Tc5!. 6one3Hh OTJJ11qaeTC5I 6onee JierK11M Te<J.ett11eM.
EOJie3Hb ~epceu (rnn IV) - Kpatitte pe)l.Koe aYTOCOMHO-peuecc11Bttoe 3a60JieBatt11e, B03HHKaIOmee BcJiencrn11e ,LJ,ecpeKrn BernHmero cpepMema - aM11JI0-1,4-1,6-rn10Ko311J1'fPattc<Pepa3hI. Co,n:ep)KaHtte rn11KoreHa B neqeH11 He CHJihtto yBeJIWieHo, HO CTPYKTypa ero 113:vieHeHa, 11 3TO npenHTcrnyeT ero pacnany. MmICKYJia rn11Koretta HMeeT MaJIO TO<J.eK BeTBJleH115l, a TaK)[(e O<J.eHb ,n:JIMHHhre H pe,n:KHe 6oKOBbie BeTB11. B TO )J(e BpeMH r11norn11KeMl1H Bbipa)KeHa yMepeHHO. bOJie3Hh pa3Bl1BaeTC5I 6hICTPO, ornrowaeTCH paHHl1M ll}tpp030M neqeHl1 l1 npaKTtt<J.eCKl1 He IIOJJ.- ,LJ,aeTC5I neqeHHIO. Ll,ecpeKT cpepMema BeTBJiemui o6ttapy)J(l1BaeTCH He T0,1hKO B rret:IeHH, HO TaK)Ke B JJeHKOU11TaX, MhllllUaX, ¢u6po6JiacTax, HO paHHHe H rrpeo6JianaIOLUHe npOHBJ1eHl151 6oJie3HH o6ycJIOBJieHhT HapywetmeM cpyHKUHH rreqeHtt.
332
lioJie3Hb Xepca (THU VI) TaK>Ke npoHBJrneTcH
CHMnTOMaMH, o6ycJTOBJleHHblMH nOp(l)l(emrnM ne'-leHtt.llaHHb!H fJllfKOreH03 - CJTe)J:CTBHe ,ri:e- <PeKTa rn11KoreH<Poc<Popirna3bI. B renaTOUHTax HaKanmrnaeTCH rm1KoreH HOpMaJTbHOti: cTpyK-
TypbI. Te'-lemie 6oJTe3HH cxo;:i:Ho c rn11KoreH030M I T11na, HO Cl1MTIT0Mbl Bbipa)!(eHbl B MeHhrnei1 cTeneH11. Ctt11)!(eHHa5r aKTl1BHOCTh n111Korempoc<Pop11JTa3ht 06Hapy)!(11BaeTCH TaK- )!(e B JTeti:Kou11rnx. 6oJTe3Hb Xepca - pe;:i:K11ti: THIT rJTHKOfeH03a; HacJTe;:i:yeTCH ITO ayTOCOM- HO-peuecCl1BHOMY Tl1rry.
)le<l>eKT KHHa3bI <t>oc<t>opHJia3bI (Tuu IX) BCTpe-
'-!aeTCHTOJlbKO y MaJlb'-!11KOB,TaK KaK 3TOT np113HaK cuenJieH c X-xpoMocoMoti:.
)le<l>eKT upOTeHHKHHa3bI A (Tun X), TaK )!(e KaK 11
;:i:e<PeKT Kl1Ha3bl <Poc<Pop11Jla3bI, npOHBJJHeTCH c11MnT0MaM11, cxo;:i:HhIMl1 c 6oJTe3HbIO Xepca.
Mblmequble <t>opMbI rJIHKoreuo30B xapaKTep1ny-
10TCH ttapyrnetttteM B 3Heproctta6)!(eH1111 CKeneTHbIX MbllllU. 3Tl1 6oJTe3HJf npOHBJIHIOTC51 np11 <PH3Jf'-!eCKJfX Harpy3Kax 11 corrpOBO)l(L!alOTC51 6oJTHMH 11 cy;:i:oporaM11 B Mh1rnuax, cna6oCTbIO 11 6btCTpoti: YTOMJTHeMOCThIO.
aYTOCOMHO-pe- ueccttBHaH naTOJTOfJfH, np11 KOTopoti: noJTHOCTblO OTCYTCTByeT B CKeJleTHbIX MbllllUax aKTJfBHOCTb fJll1KOreH<Poc<Pop11Jla3bl. TiocKOJlhKY aKTHBHOCTb 3T0f0 <PepMeHTa B renaTOUJfTaX HOPMaJTbHaH, TO rnrrorn11KeMHH He Ha6JTIO)J;a- eTC51 (CTPOeH11e <PepMeHTa B ne<reHH 11 MhilllUaX Kon11py10TCH pa3HhIMl1 reHaM11). TH)l(eJThie <PH3wrecK11e Harpy3Kl1 TIJlOXO nepeHOCHTCH 11 MOryr conpOBO)l(L!aThCH cynoporaMH, OllHaKO nptt <Ptt3Jf'-leCKHX Harpy3Kax rnrreprrpOJJ:YKUHH JlaKTaTa He Ha6JTI01laeTC51, lfTO ITOll'-!epKHBaeT3Ha- '-leHl1eBHeMbIWe'-!HhIXHCTO'-!HHKOB3HeprHJf 1I)I5I
COKpaweHmr MhllllU, HarrpHMep, TaKl1X KaK )i(11pHhie Kl1CJ10TbI, 3aMeIUaIOwtte rrptt ;:i:aHHOH naTo,1orn11 rnIOK03Y (cM. pa311,eJl 8). Xorn 6oJTe3Hb He cueITJietta c noJioM, 6oJThrnaH qacToTa 3a6oJleBaHl1H xapaKTepHa MH M~HH.
)le<t>eKT !J>oc!J>o!J>pyKTOKHHa3bl xapaKTepeH LIJTH
rn11Koretto3a VII T11na. nOJlhHbte MOryr BhIITOJTHHTh yMepeHHb1e <P113Jf'-leCKHeHarpy3KH. Te'-le- Htte 6oJie3Hl1 CXO)J:HO c I'JIMKoreH030Mv TMna, HO OCHOBHble npOHBJleHl151 Mettee Bblp(l)l(eHbI.
)le!J>eKT !J>oc!J>omuuepoMyra3bI u ne!J>eKT M-cyfu.e-
)1,HHHilbI JI)lf (HettyMepoBaHHhie no KJiacc11<P11Kau1m Kop11, CM. Ta6JT. 7-3) xapaKTepHhI MH
MhIIIIe'-IHhIX<PopM fJTHKOreH030B. Tipo51BJTeHI15l
3Tl1x narnJTornti: attanorn'lHbI6oJTe3HH MaKApMa. ,lle<PeKT <Poc<PornttuepOMYTa3hI B Mhrrnuax OITJfCaH TOJTbKO y 01lHOro 60JThHOfO.
2. Al.tluKozeno3bl
Arn11Koretto3 (rnttKoreH03 0 no KJiacctt<P11Ka-
u1111) - 3a60JleBaHl1e, B03HHKaIOIUee B pe3yJlbTaTe Lie¢eKTa rn11KorettcHHTa3hI. B rre'-1em111 Lipyrnx TKaHHX 60JlbHbIX Ha6Jll01laIOT O'leHbHH3KOe COL1,ep- )!(att11e rJTl1KOreHa. 3TO np05IBJ15leTCH pe3KO Bblpa- )!(eHHOti: r11rrornttKeMtteti: B nocTa6cop6nrnttOM nep11o;:i:e. XapaKTepHhiti: c11MnTOM - cy;:i:oporn, npoHBJlHIOIUttecH oco6eHHO no yTpaM. 6oJie3Hh COBMeCTHMa c )1(113Hhl0, HO 60JTbHbJe neTl1 Hy)l(L!a- IOTCH B l.JaCTOM KOpMJieH11Jf.
VIII. KATA60fllf13M rntOK03bl
Karn6oJIJf3M rnIOK03hI - ocHOBHoi1 nocrnBIUHK
3HeprttH M5l npouecCOB )i(113He;:i:eHTeJTbHOCTl1 opra-
HH3Ma.
A. 0CHOBHhIE IIYfll KATAEOJill3MA rJIIOK03hl
OKHcJieuue rJIIOK03bI no C02 u H 20 (a3po6ttblH
pacuan). A3po6Hhitt pacna;:i: rnIOK03hI MO)!(HO BhIpa311Th cyMMapHhlM ypaBHeH11eM:
C6H1P6 + 6 02--:> 6 co2 +Hp+ 2820 K,[1,)1(/MOJTb.
3TOT npouecc BKJTIOlfaeT HeCKOJTbKO CTa1lHH (p11c. 7-33).
•A3po6Hhiti: rnttKOJlM3 - npou,ecc oKHcJTeHHH rJTIOK03hI c o6pa30BaHl1eM 1lBYX MOJTecyJT ITl1pyBarn;
•06m11ti: nyYh Karn6oJT113Ma, BKJlIO'-laIOIUlillrrpe- BpameH11e rr11pYBaTa B aueT11JTKoA 11 ero 1laJTbtteti:wee OKJfCJleHtte B UHTpaTHOM UHKJTe;
•UTI3 tta KHCJTopo;:i:, corrpmKeHttaH c peaKuttHMH ;:i:erMp11poBaH11H, npOHCX01l511Ul1MI1 B rrpouecce pacrra;:i:a rnIOK03hl.
Aua3po6ttbIH pacuan
B onpe;:i:eJieHHhtx cttryau11Hx 06ecne'-lett11eKHc- Jiopo.L1,oM TKaHett MO)!(eT He COOTBeTCTBOBaTb 11X noTPe6ttocrnM. HanpttMep, Ha Ha'laJThHhIXCTMHHX l1HTeHCHBHOti: MhIWe'-!HOHpa60Tbl rrpJf CTPecce cep-
.Ll,e'lHbieCOKPaIUeHI1H MOryr He 1lOCTJffaTh tty)KHOH qacTOTbl, a IlOTpe6HOCTl1 MhllllU B KJfCJlOPOLie 1I)I5I a3po6Horo pacrra;:i:a rJIIOK03hI Bem1KH. B no;:i:o6HhIX CJIY'-IMXBKJTIO'!aeTCHnpouecc, KOTOpb!H npoTeKaeT 6e3 KHCJTOPOLia H 3aKaH'-IJfBaeTC51 o6pa30BaHHeM JlaKTaTa Jf3 n11pOBl1HOfPMHOti: KJfCJTOTbl. 3ToT npo-
333