підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

pa3BMTH10'rraTOJIOfWieCKMX COCTOHHMM, Ha3hIBaeMhIX fMI10BMTaMMH03aMM.

OcHOBHhie npWIHHbI nmosmaMuuo3os

•He.uocrnTOK BMTaMMHOB B rrwme;

•Hapyrnetttte Bcach!BaHIDI B .IKKT;

•Bpm1q:i:etttth1e .ue<t>eKThI <t>epMeHTOB, yqacTByIOIUMX B rrpeBpameHIDIX BMTaMMHOB;

•)],e:H:cTBtte CTPYKT)'PHhIXaHanoroB BHTaMMHOB ( aHTMBMTaMMHhI).

CToTPe6HOCTh qenoBeKa B BMTaMMHax JaBMCMT OT rrona, B03pacrn, <l>tt3MOJIOfJflleCKOro COCTOHHIDI 11 MHTeHCMBHOCTM Tpy.ua. CymecTBeHHoe BJIIDIHMe Ha 110Tpe6HOCTh qeJIOBeKa B BMTaMMHax OKa3hIBaIOT xapaKTep 11HIUM (rrpeo6na.uaHMe yrJieBO,UOB MJIH 6eJIKOB B ,UMeTe, KOJIJflleCTBO M KaqecTBO xmpoB),

a TaIOKe KJIMMaTJflleCKHe ycJIOBIDI.

. .

KllACClllC!>lll KAUlllSI BlllTAMlllHOB

no XMMJflleCKOMY CTPOeHMIO M <l>tt3MKO-XMMM- qecKMM CBOHCTBaM (B qacTHOCTM, 110 paCTBOpMMOCTM) BMTaMMHhl ,UeJIHT Ha 2 rpyrr11hl.

A. Bo,JJ,OPACTBOPHMbIE

BttrnMMH B, (TttaMtttt); BttTaMHH B 2 (ptt6o<t>naBttH);

BMTaMMH pp (HMKOTMHOBaH KMCJIOTa, HMKOTMHaMM,U, BMTaMMH B);

CTaHToTettoBaH KMCJIOTa (BMTaMMH B 5);

BttTaMMH B 6 (mipw.uoKCHtt);

EwoTMH (BMTaMMH H);

<l>o;meBaH mcnorn (BMTaMMH B 0 , B 9); BMTaMMH 1312 (Ko6anaMMtt);

BMTaMMH c (acKop6MHOBaH KMCJIOTa); BttTaMMH P (6wo<t>naBoHoM,UhI).

Ji. X<llPOPACTBOPHMbIE

BMTaMMH A (peTMHOJI);

BHTaMMH D (xoneKaJihUH<l:>epon); BMTaMHH E (ToKo<t>epon); BMTaMMH K (<l:>MJIJIOXMHOH).

Bo)J,opaCTBopHMwe BHTaMMHhI rrptt MX tt36hIToqHOM ITOCTyrrJieHMM B oprattM3M, 6y.uyqw xoporno paCTBOpHMhlMM B BO,Ue, 6hICTPO BhlBO)J,HTCH M3 opraHM3Ma.

JKupopaCTBOPHMbie BMTaMMHhl xoporno paCTBOpMMhl B :xrnpax M JierKO HaKaITJIMBaJOTCH B opraHM3Me rrpM lfX H36hITOqHoM ITOCTyrrJieHMM c ITH-

me:H:. Mx ttaKoITJietttte B oprattH3Me MO)K:eT Bhl3-

BaTh paCCTPOMCTBO o6MeHa BemecTB, Ha3hIBaeMoe rwrrepBMTaMMHOJOM, M .u~e rn6enh opraHM3Ma.

A. Bo,JJ,oPACTBOPHMbIE BHTAMHHbI

1. Burno.Mun B 1 (mull.Mun). CTPYKTYPa BMTaMMHa BKJIIOqaeT ITMPMMM,UMHOBOe M TMa30JIOBOe KOJih-

ua, coe,UMHeHHhie MeTHHOBhlM MOCTMKOM.

1- n lllPlllMlllAlllHOBOe KOilbl.lO; 2 - Tlll830110BOe KOilbl.lO

BMTBMMH 8 1 (TMBMMH)

llCTO'lllllKH. BHTaMMH B 1 - nepBhltt BMTaMHH, BhI,UeJieHHhIM B KpMCTaJIJIJflleCKOM BM,Ue K. <l>ytt-

KOM B 1912 r. Ott lllHpOKO pacnpOCTPaHeH B npo.uyKTax paCTMTeJihHOfO npOMCXO)l(JleHMH ( o6onoqKa ceMHH XJie6HbIX 3JiaKOB M pttca, ropox, <t>acOJib, COH M .up.). B oprattM3Max )IGIBOTHhIX BHTaMMH B, co.uep)IGITCH rrpeHMymeCTBeHHO B BM,Ue .UH<l:>oc<t>opttoro 3<l>ttpa TMaMMHa (T)],<l>); OH o6pa3yeTCH B rreqeHM, rroqKax, M03re, cep,aeqtto:H: Mhnnue nyTeM <t>oc<t>opMJittpoBaHIDI TMaMMHa IIPM yqaCTMM TMaMHHKMHa3bl M AT<l>.

B3pocJioro qeJioBeKa B cpe.utteM cocTaBJIHeT 2-3 Mr BMTaMMHa B,. Ho IIOTpe6HOCTh B HeM B oqeHh 6oJihlIIOM CTeneHM 3aBHCMT OT COCTaBa M o6me:H: KaJIOpMMHOCTH 11Mlll.M, HHTeHCHBHOCTH o6MeHa BemecTB 11 HHTeHCHBHOCTM pa60Tbl. Tipeo6na.uaHHe yrJieBO,UOB B 11Hll.J.e IIOBhilllaeT IIOTPe6HOCTh opraHH3Ma B BHTaMHHe; )K:Mphl, Hao6opoT, pe3KO YMeHhlllaJOT 3TY IIOTPe6ttocTh.

BHTaMHHa B 1 orrpe,ae.JUieT-

CH TeM, qTo B BM,Ue T)],<l> OH BXO,UMT B COCTaB KaK MMHMMYM Tpex <t>epMeHTOB H <t>epMeHTHhlX KOMITJieKCOB: B COCTaBe IlHPYBaT- H cxKeTOrJIYTapaT.uerH,D;poreHa3HhlX KOMI1JieKCOB OH yqacTByeT B OKHCJIHTeJihHOM .ueKap60KCHJIHpoBaHHH IIHpyBaTa H CX-KeTOfJIYTaparn; B cocTaBe TpattcKeTonaJhI T)],<l> yqacTByeT B

rreHT03ocpoccpaTHOM rryrn: rrpeBpamemrn: yrJieBO.UOB.

OcttOBHOH, Han6oJiee xapaKTepHhIH H crreuncpwi:ecKlfH rrpli3HaK He,UOCTaTO'IHOCTHBHTaMHHa B, - rromrneBpHT, B OCHOBe KOTOporo Jie)l(aT .uereHepaTHBHhie H3MeHeHHH HepBOB. Btta'laJiepa3B11saeTcH 6oJie3HeHHOCTh B.UOJih HepBHhIX CTBOJTOB, 3aTeM - IlOTepH KO)f(HOll 'IYBCTBl1TeJihHOCTH H HacTyrraeT napaJil1'1 (6epn-6ep11). Brnpoi1: Ba)l(Hei1:nrnii npH3HaK 3a60JieBaHlfH - ttapyrnett11e cep.ue'IHOH.ueHTeJihHOCT11, 'ITO Bbipa)l(aeTCH B HapyrneHHH cep;:i;e'IHoro pMTMa, yBeJIH'leHHH pa3MepoB cep.uua H B IIOHBJieHHH 6oJieM B o6JiaCTH cep.uua. K xapaKTeptthIM rrpH3HaKaM 3a60JieBaHHH, CBH3aHHOro c He.uocTaTO'IHOCTblO BlfTaMHHa BI'OTHOCHT TaK)l(e HapyrneHHH ceKpeTOpHOM H MOTOpHOM cpyHKUl1H ){(KT; tta6mo.uaIOT CHH)l(eH11e Kl1CJIOTHOCTH )l(eJiy- )l;O'IHOro COKa, IIOTepIO arrrreT11Ta, aTOH11IO Klfllie'IHl1Ka.

B octtose CTPYKTYPhI BlfTaM11tta B 2 Jie)l(HT CTPYKTYPa lf30aJIJIOKca3HHa, coe.u11HeHHOro co CIIlipTOM p11611TOJIOM.

Pi.t6o¢naBHHKHH03a FMN-a,QeHHnHmpaHc¢epaaa

KocpepMeHThI FAD 11 FMN Bxo;:i;HT B cocTaB cpJiaBlfHOBblX cpepMeHTOB, rrp11H11MaIOII1,HX yqaCTl1e B 0KlfCJil1TeJihHO-BOCCTaHOBl1TeJihHhIX peaKUHHX (CM. pa3,a:eJihl 2, 6, 9, 10).

KrlHHH'leCKHenpm1BJieHHJ1 ue.uocTaTO'IHOCTHp116ocpJiaB11Ha Bhip(l)J(aIOTCH B OCTaHOBKe poem y MOJIO.UhIX opraHlf3MOB. lfacTo pa3Bl1BaIOTCH BOCIIaJil1TeJihHhie rrpoueCCbl Ha CJIH311CTOM o6oJioqKe pOTOBOM IIOJIOCTl1, IIOHBJIHIOTCH MHTeJihHO He3a)l(HBaIOIIl,He Tpelll,11Hhl B yrnax pTa,

.uepMaT11T ttocory6ttoii CKJia.UKH. T11rrHqHo BOCIIaJieHHe rJia3: KOH'blOHKTHBl1Thl,BaCKYAAp113aUHH poroBHllhI, KaTapaKTa. KpoMe Toro, np11 aBl1TaMHH03e B 2 pa3B1rnaIOTCH o6maH MhIrne'IHaH CJia6ocTh H cna6oCTh cep;:i:eqttoii MhlllIUhl.

3. Buma.Mun PP (nlllwmunoBaa KUC.lloma, nu1eomuna.MuiJ, Buma.Mun B)

0

fJiaBHbie HCTO'IHHKH BHTaM11Ha B2 - rreqeHh, rroqKH, Hiiua, MOJIOKO, .upo)l()l(H. BHTaMl1H co-

.uep)l(HTCH TaK)l(e B llII111HaTe, rrrnett11ue, p)f(lf.

qacT11qHo qeJioBeK rron:yqaeT BHTaM11H B 2 KaK npo,uyKT )f(lf3He.UeHTeJihHOCTl1 KHrneqHOH Ml1KpocpJIOphl.

Cyro11Hrui: nOTpe6uoCTb B Bl1TaM11He B 2 B3pocJioro qeJIOBeKa COCTaBJIHeT 1,8-2,6 Mr.

liHOJIOI'll'lecKHe4JYffKU,HH. B CJil1311CTOM o6oJioqKe KHllie'IHl1KaIIOCJie BCaChIBaHl1H Bl1TaMlfHa rrpo11cxo.u11T 06pa30BaH11e KocpepMeHTOB FMN 11 FAD no cxeMe:

paHeH B paCTl1TeJihHhIX npo.uyKTaX, BhICOKO ero co.uep)l(att11e B pHCOBbIX 11 nrneH11qHhlX 0Tpy6Hx, .UPO)l()l(aX, MHOro Bl1TaM11Ha B ne- 'leHHH rroqKax Kpynttoro poraToro CKOTa H CB11Hei1. B11rnM11H PP MO)l(eT o6pa30BhIBaTh- cH 113 TpHrrTOcpaHa (113 60 MOJieKyJI Tp11IITOcpaHa MO)l(eT o6pa30BaThC51 1 MOJieKyJia Hl1KOTl1HaM11.ua), qTo CHl1)l(aeT IlOTpe6HOCTb B BMTaMI1He pp rrpH yBeJI11qemm KOJil1'leCTBa TPl1IITOcpaHa B rrrrme.

CyT011ua}I noTpe6uocTL B 3TOM BHTaMMHe coCTaBJIHeT .um1 B3POCJiblX 15-25 Mr, .UJIH .ueTeii - 15 Mr.

liHOJIOI'll'lecKHe4Jyt1KIU1n. H:ttKOTHHOBaH KHCJIOTa B opraHH3Me BXO)lJfT B cocrnB NAD H NADP, BbIIIOJIHHIOIIl,MX cl>YHKUHH KoQ:>epMeHTOB pa3JUI'IHhIX.uern.uporeHa3 (CM. pa3,ueJI 2). CMHTe3 NAO B opraHH3Me rrpoTeKaeT B 2 3Tarra:

NADP 06pa3yeTcH M3 NAD rryreM cpoccpoprr1rnpoBaHHH IIO)l )leMCTBHeM UMTOIUla3MaTWiecKOH NAD-KHHa3hI.

NAD+ + AT<l> ~ NADP+ + A,Il,<1>

He.11.ocTaT011uocTb BHTaMHHa PP rrpMBO)lMT K 3a6oJieBaHMIO «IIeJIJiarpa», )lJIH KOToporo xapaKTepHhI 3 OCHOBHbIX rrpM3HaKa: ,UepMaTMT, )lHapeH, )leMeHUHH («TJJM LI,»). CTeJIJiarpa rrpOHBJIHeTCH B BH)le CMMMeTpMqHoro )lepManna Ha yqacTKax KO)l(J.1., )lOCTYfIHbIX )leHCTBMIO COJIHeqHbIX nyqeii:, paCCTJ)OHCTB )l(}(T (,UttapeH) M BOCIIaJIHTeJibHbIX rroproKeHMH CJIM3MCThlX o6oJioqeK pra M H3h1Ka. B )laJieKo 3arne,UrnMX cJiyqaHx rreJIJiarpbI tta6JIIO)laIOT paccrpoii:crna ll,HC (,UeMeHUHH): IIOTepH rrawrrn, faJIJIIOUMHall,MM H 6pe)l.

4. IlanmomenooaR KUCAoma (8UmllMUH B)

CTaHTOTeHOBaH KHCJIOTa COCTOMT H3 OCTaTKOB 0-

2,4-)lHfH)lpOKCH-3 ,3-)lMMeTH/IMaCJIHHOH KHCJIOThl H

~-aJiaHHHa, coe)lHHeHHhIX Me)f()zy co6oif aMH)lHOH

CBH3hIO:

CH3 OH

IlaHTOTeHOBaH KHCJIOTa - 6eJibrn MeJIKOKPHCTaJI-

JIHllecKHif rropOI.IIOK, xoporno paCTBOPIIMhIB B BO)le. Otta CMHTe3HpyeTCH pacreHHHMM H MMKpooprattH3MaMH, CO)lep)l(HTCH BO MHOfMX rrpo)lyKTax :xrn:BOTHOro H pacnrre.JThHoro rrpottcxoJK,IJ,eHAA (Hii:u,o, rreqeHh, M.SICO, pb16a, MOJIOKO, )lpo)l()l(H, KaprocpeJib, MOPKOBh, IIIlleHHUa, H6JioKH). B KJ.1.Ille'IHHKeqeJIOBeKa naHToTeHOBaH KHCJIOTa B HeOOJibllIMX KOJIHllecTBaX npo)lyUHPYeTC.SI KHUie'IHoifrrano~oif. IlaHTOTeHOBaH KHCJIOTa - yttMBepcaJihHhIH BHTaMHH, B HeH IDIH ee IIpoH3BO)lHhJX H)')IQJ,aIOTC.SIqeJIOBeK, )!(HBOTHbie, paCTeHM.SI H MHKpOOpraHM3MhI.

Cyro11uag noTpe6uOCTb qeJioBeKa B rraHTOTeHoBOH KHCJIOTe COCTarurneT 10-12 Mr.

IlaHTOTeHOBaH KHCJIOTa HCIIOJlh3YeTCH B KJieTKaX)lJIH CMHTe3a KocpepMeHTOB: 4-cpoccporrattToTettHa M KoA (pttc. 3-1). 4-cpoccporraHTOTeHH - KOcpepMeHT IIaJihMHTOMJICMHTa3bI. KoA yqacrnyer B rrepettoce auMJibHhJX pa,IU1KaJIOB B peaKUHHX o6mero flYTH KaTa6oJIH3Ma (CM. pa3)leJI 6), aKTHBaUHH )KllpHbIX KHCJIOT'CMHTe3a XOJiecrepMHa M KeTOHOBbIX TeJI (CM. pa3,UeJI 8), CHHTe3a auenuITJIIOK03aMHHOB (cM. pa3)leJI 15), o6e3Bpe)KHBaHM51 lJY)Kepo)lHhrx Bern,ecTB B rreqeHH (cM. pa3,UeJI 12).

Kmtull'leCKHe npoBBJieHHJI He,ll,OCTaTO'IHOCTH BHTaMHua. y qeJIOBeKa H )KHBOTHhIX pa3BMBaIOTCH )lepMaTHThl, )lHCTpOcpMqecKHe H3MeHeHH.SI )J(eJie3 BttyTpeHHeH ceKpeUHH (HarrpttMep, Ha,u- rroqeqHHKOB), HapYUieHtte )le51TeJihHOCTH HepBHOH CHCTeMhl (HeBpHThI, rrapannqrr), )lHCTJ)O- cpttqecKHe H3MeHeHHH B cep,Uue, rroqKax, )leIIHrMeHTaUHH H Bbma,ueHHe BOJIOC H rnepCTH y )J(HBOTHhIX, IIOTepH arrrreTHTa, MCTOI.IIeHHe. Htt3KHH ypoBeHh rraHTOTettaTa B KpOBH y JIIO)leH qacTO coqeTaeTCH c ,UpyrHMH IBIIOBHTaMHH03aMM (Bl'B 2) M rrpoHBJIHeTc51 KaK KOM611.ttttpoBaHHaH cpopMa fHIIOBHTaMHH03a.

5. BumaMun B 6 (nupuik>Kcun, nupuOoKCaAb, nupuiJoKcaMun)

B ocHoBe CTJJYKTYPhI BHTaMHHa B 6 Jie)KHT rrHPMHHOBoe KOJihUO. l13BeCTHhI 3 cpopMhI BHTaMHHa B 6 , oT1mqa10mnecH crpoeHneM 3aMema10meif rpyrrrrhl y aTOMa yrnepo,Ua B II-IlOJIOXeHHH K aTO-

MY a3oTa. Bee OHM xapaKTepH3YJOTCH o)lttHaKOBOH 6HOJI0fHqeCKOH aKTHBHOCThlO.

#0

3

I 1

Bee 3 ¢>opMbI BHTaMHHa - 6ecu;BeTHhie KpHcTaJIJibI, xoporno pacrnopHMhie B Bo.n;e.

B6 Alli qeJioBeKa - TaKHe rrpoayKTbl IIHTaHHH, KaK HHUa, rreqeHb, MOJIOKO, JeJieHhIH rrepeu, MOPKOBb, IIIlleHHUa, JlPOX-

)l(H. HeKoTopoe KonmecTBO BHTaMHHa CHHTeJHpyeTCH KHrneqHoH <t>JiopoH.

CYTO'IHIUImrrpe6uoCTb cocTaBJIHeT 2-3 Mr.

EuOJIOill'leCKHe<i>YffKU,HH. Bee ¢>oPMhI BHTaMHHa B 6

HCIIOJib3yioTCH B opraHHJMe ,llIDI CHHTeJa Ko¢>epMeHTOB: IIHpH,ll,OKC3.Jlb¢>oc¢>aTa H IlHpH,!J,OKcaMHH- <t>oc<t>aTa. Ko¢>epMeHTu1 o6paJyrorcH rryreM <Poc-

¢>opH.Jrn1x1BaHIDI ITO rn,n,poKCHMeTH.JlbHOM rpynne B IIHTOM IIOJIOXeHHH IIHpHMH,!J,HHOBOro KOJlbUa rrptt yqaCTHH ¢>epMema rrnpH,ll,OKCaJibKHHaJhI H AT<I> KaK HCTO~ <t>oc<t>aTa.

IlHpH)lOKCaJieBble ¢>epMeHTbl HrpaIOT KJIIOqeBYIO poJib B o6Mette aMHHOKHCJIOT: KaTaJIHJHPYIOT peaKUHH TpattcaMHHHpoBaHHH H )leKap6oKcIDmpoBaHHH aMHHOKHCJIOT, yqaCTBYIOT B crreumlmqecKHx peaKUHHX MeTa60JIHJMa oTJleJihHbIX aMHHOKHCJIOT: cepHHa, TpeOHHHa, TPHIIToq>aHa, cepoco.n;epxaIUHX aMHHOKHCJIOT, a TaKXe B CHHTeJe reMa (CM. paJ;i:eJihI 9, 12).

KIIHHH'leCKHenpoBBJieHIDI ue)l.OCTaro11uocm su-

TaMHHa. ABHTaMHHOJ B 6 y )leTeli rrpoHBJIHeTcH IIOBbIIlleHHOH BOJ6YJlHMOCTbIO UHC, rrepHoJJ.HqecKHMH cy;i:oporaMH, qTo CB513aHO, BOJMO)f(HO, c He)lOCTaTOqHbIM o6pa30BaHHeM TOpMOJHOrO Me)lHaTOpa fAMK (CM. paJ;J;eJI 9), crreutt<t>WieCKHMH )lepMaTHTaMH. y BJpOCJibIX rrpHJHaKH rHIIOBHTaMHHOJa B 6 tta6JIIOJlaIOT rrpH JlJIHTeJibHOM JieqeHHH zy6epKYJieJa HJOHHaJHJJ.OM (aHTaroHHCT BHTaMHHa B 6). IlpH 3TOM BoJHHKa10T rropaxeHHH HepBHOH CHCTeMbl (rroJIHHeBpHTbl), )lepMaTHTbl.

6• .&uomuu (BumaMuH H)

B ocHoBe CTPOeHHH 6HoTHHa Jie)l(HT THo<l>ettoBOe KOJibUO, K KOTOPOMY IIPHCOe;J;HHeHa MOJieicyna MOqeBHHbl, a 60KOBaH uerrb rrpe)lCTaBJieHa BaJiepbHHOBOM KHCJIOTOM.

MCTO'IHHKll. farnnrn conep:xrn:TCJI IIO'ITMBO Bcex rrpo,Uyi<TaX )KMBOTHOro M paCTMTeJibHOro rrpoMCXO)K):(eHMJI. Haw6oJiee 6oraThI 3TMM BMTaMMHOM rreqeHh, noqKM, MOJIOKo, )KeJITOK Hilua. B o6hI'IHhIXycJIOBIDIX IIeJioBeK rronyqaeT nocTaToqHoe KOJIMqecTBO 6MOTMHa B pe3yJibTaTe 6aKTepMaJibHOro CMHTe3a B KMIIIe'IHMKe.

CyTO'IHaJI noTpe6uocTh 6MOTMHa y qenoBeKa He

npeBbIIIIaeT 10 MKr.

BuoJiollf'lecKaJI po.rib. BMOTMH BbIIIOJIHHeT Ko<l>epMeHTHYJO <l>YHKn:MIO B COCTaBe Kap60KCMJia3: OH yqacrnyeT B o6paJOBaHIDi aKTMBHOH <l>opMhI C02•

B opraHMJMe 6MOTMH MCIIOJib3yeTCH B 06pa30BaHw11 MaJIOHl1JI-KoA M3 au,eTl1JI-KoA (CM. pa3,UeJI 8), B Cl1HTe3e rrypMHOBOro KOJibua (CM. paJneJI 10), a TaIOKe B peaKLI,1111 Kap60KCMJIMpoBaH11J1 n11pyBaTa c o6pa30BaHMeM OKCaJIOaueTaTa (cM. pa3neJI 6).

K.JnmuqecKHe npoSIBJleHIUI He,UOCTaTO'IHOCTJ1:6MOTMHa y qeJIOBeKa 113yI.JeHbl MaJIO, IIOCKOJibKY 6aKTep1rn KHIIIe'l.HHKa 06Jia,UaIOT crroco6HOCTb10 CMHTe3HPOBaTb 3TOT BHTaMHH B He06XO,UHMbIX KOJIMY.ecrnax. Il03TOMY KapTHHa aBHTaMHH03a npOHBIDJ:eTCJI rrp11 m1c6aKTepH03ax KMUieqHMKa, HarrpuMep, nocJie rrpueMa 6oJihIIIMX KOJIM'Iecrn aHTM6HOTHKOB mm cyJib<l>aMttnHhIX rrperrapaTOB, Bbl3bIBa10uurx rn6eJib Ml1KPO¢mopbl KHIIIe<JHHKa, JIH6o nocJie BBeneHMH B pauuoH 60JihIIIOro KOJIH'IecrnaCbiporo HH'IHoro6eJIKa. B HH'IHOM 6eJIKe conep)KHTCJI rJIHKOIIpOTeMH aBM,UMH, KOTOpbIH coe,UMHJleTCSI c 6MOTMHOM M rrperrSITcTByeT scachIBaHMJO rroCJienttero m KMIIIe'!HMKa.ABMMH (MOJieKYAAPHM Macca 70 000 K,[1,) COCTOHT M3 'IeTbipex11,ll,eHTWIHbIX cy6be,UMHMU, conep- )KaIUMX ITO 128 aMMHOKMCJIOT; KID!QlM cy6ben11HMUa CB513bIBaeT ITO 0)1,HOM MOJieKYJie 6110THHa.

IlpM He,UOCTaTOqHOCTM 6MOT11Ha y qeJIOBeKa pa3BJrnaJOTC51 HBJieHJrn crreuw<l>tt<JecKoro nepMaTMTa, xapaKTepM3yIOIUerocH rroKpacHeH11eM M IIIeJIYllieHMeM KO)l(Jf, a TaIOKe o6MJibHOH ceKpeu,MeM CaJibHbIX )!{eJie3 (ce6opeSI). fipH aBM-

TaMHH03e BHTaMMHa H Ha6JI10na10T TaK)!{e BbIIIa,UeHMe BOJIOC M IIIepCTM y )KMBOTHbIX, rropa)KeHMe HOrTeM, 'IaCTO OTMe'IaIOT 60Jil1 B MblIIIUax, yCTaJIOCTb, COHJIMBOCTb M nerrpeCCMIO.

7. <Po.!lue6aJI KUCAoma (6Ul1UlMUH Be, 6Uma.MUH Be)

Cl>oJIMeBM KHcJiorn cocTOMT M3 Tpex CTPYKTYPHhix e,UMHMu: OCTaTKa rrTepH,UHHa (I), rrapaaMMH06eH30MHOH (II) M rnyTaMMHOBOH (III) KHCJIOT.

BmaMMH, rronyqeHHhIM M3 pa3HbIX wcrnqHMKOB, MO)!{eT conep)!{aTb 3-6 OCTaTKOB rnyTaMMHOBOH KHCJIOTbl. Cl>oJIMeBM KMCJIOTa 6bIJia BbI,UeJieHa B 1941 r. M3 3eJieHbIX JIMCTbeB pacTeHMM, B CB513M c qeM M IIOJiyqMJia CBOe Ha3BaHMe (OT JiaT. folium - JIMCT).

McTO'IHHKH. 3HaqwTeJihHoe KOJIMqecrno 3Toro

BMTaMMHa conep)!{MTCJI B npo)IQl(ax, a Ta.JOKe B rreqeHH, rroqKax, MJ1ce M .upyrwx rrponyKTax )KMBOTHOro rrpOMCXO)K,L(eHMJI.

CyT011urur noTpe6uocrh B <l>oJIMeBoif KMCJIOTe KoJie6JieTcH OT 50 no 200 MKr; O,UHaKO BCJie,U- CTBMe IIJIOXOM BCaChIBaeMOCTH 3TOro BHTaMM-

Ha peKOMettnyeMM CYTOqHaJI ,U03a - 400 MKr. liHOJIOflllleCKaJI poJlb <l>oJIMeBOH KHCJIOTbl onpe-

neJIHeTCSI TeM, qTo OHa CJIY)KMT cy6cTpaTOM ,UJI51 CMHTe3a Ko<l>epMeHTOB, yqacTByIOIUMX B peaKUMJIX rrepeHoca onHoyrneponHhIX PMHKaJIOB pa3JIH'IHOH CTerreHH OKMCJieHHOCTM: MeTMJibHbIX, OKCMMeTMJibHblX, <l>opMMJibHbIX M npyrux. 3TH Ko<l>epMeHTbl yqaCTBYJOT B CHHTe3e pa3JIM<JHbIX BeruecTB: rrypMHOBblX HyKJieOTl1,ll,OB, rrpeBparueHHH dYMCI> B dTMCI>, B o6MeHe rJIH-

UMHa M cepMHa (CM. pa3,UeJibl 9, 10).

llau6onee xapaKTepHb1e npu3uaKH aeuTaMHHOla

<l>oJIHeBOH KMCJIOTbl - HapYUieHMe KPOBeTBOpeHMJI M CB513aHHhie c 3THM paJJIH'IHbie<l>opMbl MaJIOKPOBMJI (MaKpoumapHM aHeMMH), JieMKOrreHMJI H Janep)!{Ka pocTa. Ilpw r11rroBHTaMMH03e <l>oJIHeBOM KHCJIOTbl Ha6JIIO,UaJOT HapYUieHHJI pereHepauHM 3IIHTeJIM51, oco6eHHO B )Kl(T,

129

o6yCJIOBJieHHhie He,IJ,OCTaTKOM rrypHHOB H PHMl1,ll,HHOB ,ll,JI51 CHHTe3a ,IlHK B IIOCT051HHO ,IJ,eJI51Ill,HXC51 KJieTKax CJIH3HCTOM o60JIO'IKH.

AinrraMHH03 <t>onHeBOM KHCJIOThl pe,IJ,KO rrp051BJI51eTC51 y qeJIOBeKa H )1(1180THhIX, TaK KaK 3TOT BHTaMHH B ,IJ,OCTaTO'lHOMCTerreHH CHHTe3HpyeTC51 Kmne'lttoM. MttKpo<t>nopoM.. OnttaKo 11cIIOJih30Batt11e cynh<l>attHJiaMl1,ll,HhIX rrperrapaToB

,lJ,JI5I Jie'leHIDIp51,I1,a 3a6oJieBaHHM MO)l(eT Bhl3BaTh pa3BITTHe aBttTaMHH030B. 3TH rrperrapaThl - CTPYKTYPHhie attanor.11 rrapaaMtttto6ett30ti.ttoM. KHCJIOThl, HHr11611pyIOill,He CHHTe3 <t>onHeBOM KHCJIOThl y MHKpoopraHH3MOB (CM. pa3nen 2). HeKoTOphre rrpoH3BOJJ.Hhie rrTep.11JJ.ttHa (aMHHOIITepttH 11 MeTOTpeKcaT) TOpMo351T POCT IIO'ITH BCex opraHH3MOB, Hy)l(,ll,aIOIll,HXC51 B <t>onHeBOM KHCJIOTe. 3TH rrperrapaThl Haxo,IJ,HT rrp11Mettett11e B neqe6ttoM. rrpaKTHKe

,lJ,JI5I rronaBJieHIDI orryxoneBoro pocTa y OHKOnorwrncKHx 6oJihHhIX.

8. Ruma.Mun B12 («ooOAa.MUH)

BmaMHH B 12 6hm BhI,IJ,enett H3 rre'leHHB KpttcTaJIJimecKOM Bl1,ll,e B 1948 r. B 1955 r. ,Iloporn Xon- )l(Kett c IIOMOill,hIO peHTreHO-CTPYKTYPHOfO aHaJIH3a pacum<t>poBana crpyicrypy 3TOro BHTaMHHa. 3a 3TY pa6oTY B 1964 r. eM. 6bma rrpHCy)l(,ll,etta Ho6eneBCKM rrpeMM. BHTaMHH B 12 - e.a;HHCTBeHHhIM BHTaMHH, co.a;ep)l(alll,HM B CBOeM COCTaBe MeTaJIJI K06aJihT (pHC.

lICTO'IHllKll. H.11 )1(11BOTHhie, HH pacTeHM He crroco6Hhl cHHTe311poBaTh BttTaMHH B 12. 3To e,IJ,HHCTBeHHhlM BHTaMHH, CHHTe3HpyeMhIM IIO'ITH HCKJIIO'lITTeJihHOMHKpoopraHH3MaMtt: 6aKTeptt51MH, aKTHHOMHUeTaMH H CHHe-3eJieHhIMH BOJJ.OPOCMMH. 113 )1(11BOTHhIX TKaHeM tta116onee 6oraThI BHTaMHHOM B 12 rre'leHhH IIO'IKH. HenocTaTO'IHOCThBHTaMHHa B TKaH51X )1(1180THhIX CB513aHa c HapyrneHHeM BCaChIBaHM K06anaMHHa H3-3a HapyrneHH51 CHHTe3a BttyTPeHHero <i>aKTopa Kacna, B coenHHeHHH c KOTOphIM oH H BCaChIBaeTC51. <l>aKTOp Kacna CHHTe3ttpyeTC51 o6KJiallO'IHhIMHKJieTKaMH )l(eJIY,ll,Ka. 3To - fJIHKOITpOTeHH C MOJieKyMpHOM MaCCOM 93 000 ,Il. Ott coe,IJ,HH51eTC51 c BHTaMHHOM B 12 rrp.11 yqacTHH ttottoB KaJihUHH. f11rroaBttTaMttH03 B 12 o6hl'IHOCO'leTaeTC51c ITOHH)l(eHHeM KHCJIOTHOCTH )l(eJIYJJ.O'lHOfO COKa, 'ITO MO)l(eT 6hITh pe3YJihTaTOM ITOBpe)l(,ll,eHIDI CJIH3HCTOM o60JIO'IKH )l(eJIY,IJ,Ka. r11rroaBITTaMHH03 B,2 MO)l(eT pa3BHThC51 TaK)l(e rrocne TOTaJihttoro ynaneHH51 )l(eJIYJJ.Ka rrp.11 XHpypmqecKHX orrepaUIDIX.

Cyro'lnas m>Tpe6uOCTL B BHTaMHHe B 12 KpaM.tte MaJia H COCTaBJI51eT BCero 1-2 MKf.

BHTaMHH B 12 CJIY)l(l1T HCTO'IHHKOMo6pa3oBaHM

JJ.BYX Ko<t>epMeHTOB: MeTHJIK06aJiaMHHa B UH-

TOIIJia3Me H ,IJ,e30KCHfil{eH03HJIK06aJiaMHHa B

MHTOXOH,ll,pH51X (pHC.

•MeTHJI-B12 - Ko<l>epMeHT, yqacTByIOill,HM B o6pa3oBaHHH MeTHOHHHa 113 fOMOUHCTeHHa.

KpoMe Toro, MeTHJI-B 12 rrpttHttMaeT yqacTHe B rrpeBpalll,eHH51X rrpOH3BO,ll,HhIX <t>onHeBOM KHC-

JIOThl, He06XO,IJ,HMhIX ,lJ,JI5I CHHTe3a ttyKJieorn- ,ll,OB - rrpenrnecrneHHHKOB ,IlHK H PHK.

•,Ile30KCHa,IJ,eH03HJIK06aJiaMHH B Ka11ecrne KO- <t>epMeHTa yqacrnyeT B MeTa60JIH3Me )1(11pHhIX KHCJIOT c He'leTHhIM'IHCJIOMyrnepO,ll,HhIX aToMOB H aMHHOKHCJIOT c pa3BeTBJieHHOM yrneBO-

JJ.OPOJJ.HOM uerrhIO (CM. pa3,ll,eJihl 8, 9).

Ocuoeuoii npu3HaK a&HTil.MHH03a MaKPQUHTaPHM (Merano6naCTHa51) atteMM. ,IlJI5I 3TOfO 3a6oneBaHH51 xapaKTepHhI ysenH'leHHepaJMepoB 3PHTPOUITTOB, CHH)l(eHHe KOJIH'leCTBa3PHTPOUHTOB B KPOBOTOKe, CHH)l(etttte KOHUeHTPauHH reMorno6HHa B KPOBH. HapyrnettHe KpOBeTBO- peHM CB513aHO B rrepByIO O'lepe,ll,bc HapyrneHHeM o6MeHa ttyKJieHHOBhIX KHCJIOT, B 'iaCTHOCTH CHHTe3a .IlHK B 6blCTPQ,IJ,eJI5Illl,HXC51 KJieTKaX KpoBeTBOpHOM c11cTeMhI. OoMHMO ttapyrnett.1151 KpoBeTBopttoM. <i>YHKUHH, nM aBmaMMH03a B 12 crreUH<i>H'IHO TaK)l(e paCCTPOMCTBO ,IJ,e51TeJihHOCTH HepBHOM CHCTeMhl, 06o5ICHSieMoe TOKCH'lHOCThIO MeTHJIMaJIOHOBOM KHCJIOThI'HaKaIIJIHBaIOill,eHCSI B opraHH3Me rrpH pacrraue )l(llpHhIX KHCJIOT c HeqfaHbIM 'IHCJIOM yrnepo,ll,HhIX aTOMOB, a TaK)l(e HeKOTOPhIX aMHHOKHCJIOT c pa.3BeTBJieHHOM ueIThIO.

9. BumaMuH C (ac«op6uu08aa KUCAoma)

AcKop6HHOBa51 KHCJIOTa - naKTOH KttCJIOThl, 6JIH3KOM rro CTPYKTYPe K rJIIOK03e. CyrnecrnyeT B ,IJ,Byx <t>opMax: BOCCTaHOBJieHHOH (AK) H OKHCJieHHOM (.a;emnpoacKop6HHOBOM KHCJIOTOM, ,UAK).

06e 3TH ¢opMbl acKop6HHOBOH KHCJIOTbl 6hICTpo H o6paTHMO rrepeXOMT JJ.pyr B JJ.pyra 11 B KalleCTBe KO<})epMeHTOB yqacTBYlOT B OKHCJIHTeJibHOBOCCTaHOBHTeJibHblX peaKu»Hx. AcKop6HHOBaH KHCJIOTa MO)((eT OKHCJIHTbCH KHCJIOPOJJ.OM B03)],yxa, rrepOKCH)J.OM H JJ.PYfHMH OKHCJIHTeJIHMH. )].AK JierKO BOCCTattaBJIHBaeTCH UHCTeHHOM, fJIYTaTHOHOM, cepoBOJJ.OPOJJ.OM. B cJia6omeJiollHOH cpeJJ.e rrpottcXOMT pa3pyrneHHe JiaKTOHOBOfO KOJihUa H IIOTepH 6.uoJioIWiecKoli aKTHBHOCTH. Ilp.u KYJIHHapttoi1 o6pa6oTKe IIHI.Ulll: B IIPHCYTCTBHH OKHCJIHTeJieH qacTb BHTaMHHa c pa3pyrnaeTCH.

HCTO'IHHKH BHTaMHHa c - CBe)((He <PPYKTbI' OBOIUH, 3eJieHh (Ta6JI.

Cyro'IHaJImrrpe6eocn. qeJioBeKa B BHTaMHHe C

COCTaBJUier Mf.

1iHOJIOlll'lecKHe cl>YJIKllHH. f JiaBHOe CBOHCTBO acKop6HHOBOH KHCJIOTbl - crroco6HOCTb JierKo

Ta6nM48 3-1. COAep>KeHMe acKop6MHoeoi KMGnOTbl e HeKoTOpblx OM11'8BblXnpoiiyKTBX M pacTeHMtlX

OKHCJUITbCH H BOCCTaHaBJIHBaTbCH. BMeCTe c llAK OHa o6pa3yeT B KJieTKax OKHCJIHTeJibHO-

BOCCTaHOBHTeJibHylO rrapy c pe,UOKC-IIOTeHUH- aJIOM +0,139 B. JiTiaro,n:apH 3TOH crroco6HOCTH

acKop6HHOBaH KHCJIOTa yqacTByeT BO MHOfHX peaKUHHX m,n:pOKCHJIHPOBaHHH: OCTaTKOB Ilpo 11 JlH3 rrp11 CHHTe3e KOJIJiareHa (OCHOBHOfO 6eJIKa coe.mrn11TeJihHOH TKaHH), rrp11 fHJl:POKCHJIHpoBaHHH noqmMHHa, Cl1HTe3e CTepOHJl:HbIX rop-

MOHOB B Kope HMIIO'le'IHHKOB(CM. pa3,n:eJihl

9, 11).

B KHIIIe'IHHKeacKop6HHOBaH KHCJIOTa BOCCTaHaBJIHBaeT Fe3+ B Fe2+, crroco6cTByH ero BcaChIBaHmo, yCKOpHeT OCB060)!(,lleH11e )l(eJie3a H3 <t>eppHTHHa (cM. pa3,n:en 13), crroco6cTByeT rrpeBpmueHmo <t>onarn B Ko¢epMeHTHhie ¢opMhl. AcKop611HOByID KHCJIOTY OTHOCHT K rrp11POJl:HhIM aHTHOKcH,n:aHTaM (cM. pa3,n:en 8). oOJihIIIoe 3Ha'leH11e 3TOH pon11 Bl1TaMl1Ha C rrpH,UaBaJI H3BeCTHbIH aMepHKaHCKHH yqeHhIH

JI. IloJIHHr, ,n:Ba)!(,llhI naypeaT Ho6eneBcKott rrpeM11H. OH peKoMeH,UoBan HcrroJih30BaTh MH rrpo¢HJiaKT11KH 11 ne'leHHHpH,Ua 3a6oneBaH11tt (HaIIpHMep, rrpOCT)'Jl:HbIX) 60JihIIIl1e Jl:03bl acKOp6HHOBOH KHCJIOTbl (2-3 r).

K.rIHllll'lecKHeDpOSUIJleHHJI ne,!J,OC'faTO'IHOCTHBHTaMHHa C. HenocTaTO'IHOCThacKop611HoBott KHCJIOThI rrpHBOJl:HT K 3a6oneBaHHIO, Ha3hIBaeMOMY UHHrott (cKop6YT). U11ttra, B03HHKa10maH y qenoBeKa rrpH He,n:ocTaTO'IHOM co,n:eplK:aHHH B IIHIUeBOM pau110He CBe)l(HX <l>PYKTOB H OBomett, orrttcaHa 6onee 300 neT Ha3M, co BpeMeHH rrpoBe.neHHH Jl:)IHTeJibHbIX MOPCKHX IIJiaBaHHH H ceBepHbIX 3KCIIe.II,HUHH. 3To 3a6oJieBaHHe CBH3aHO c He,UOCTaTKOM B IIHIUe BHTaMHHa c. DOJieIOT UHHfOH TOJibKO qeJIOBeK, rrpHMaThl H MOPCKHe CBl1HKH. f JiaBHbJe rrpOHBJieHHH aBHTaMHH03a o6ycJIOBJieHhl B OCHOBHOM HapyrneHHeM o6pa30BaHmr KOJIJiareHa B coe,n:HHHTeJihHOH TKaHH. Bcne,n:cTBHe 3TOro Ha6n10,n:a10T pa3phIXJieHHe ,UeCeH, pacIIIaTbIBaHHe 3y60B, HapyrneHHe UeJIOCTHOCTH KaIIHJIJIHpOB ( corrpOBO)!(,llaIOIUeecH rro,n:KO)l(HhIMH KPOBOH3JIHHHHHMH). Bo3HHKaIOT oTeKH, 6oJih B cycrnBax, atteMHH. AHeMHH rrpH UHHre MO)l(eT 6bJTb CBH3aHa c HapyrneHl1eM CIIOco6HOCTH HCIIOJib30BaTh 3aIIaChl )l(eJie3a, a TaKlK:e c HapyrneHHHMH MeTa60JIH3Ma <t>onHeBOH KHCJIOTbI.

10. BumllMun P (6uoefJ11a8011oudbt)

B HaCTmrmee BpeMH H3BeCTHO, 'ITO IIOHHTHe «BHTaMHH P» 06be,1J,HHHeT ceMelicTBo 6tto<l>JiaBo-

HOH,UOB (KaTeXHHhl, <t>naBOHOHhl, <t>naBOHbl). 3TO oqetth pa3Hoo6pa3HaH rpyrrrra pacTHTeJihHhIX rroJIH<l>eHOJihHhIX coe,IJ,HHeHHH, BJil1HIOIUHX Ha rrpoHHuaeMOCTb cocy,n:oB CXOJl:HhIM o6pa30M c Bl1TaMHHOM C.

0

O)o

0

<t>naBOH

Hatt6onee 6oraThI B11TaMHHOM P Jil1MOHhI, rpe- 'IHXa,qepHOIIJIOJl:HaH pH6HHa, qepHaH CMOpO,ll,HHa, Jil1CTbH 'laH,IIJIO,IJ,bl IIIl1IIOBHHKa.

Cyro'llllUImrrpe6uoCTb MH qenoBeKa TO'IHOHe

ycrnHoBJieHa.

BuoJiont'lecuspoJIL <l>JiaBOHOHJl:OB 3aKJI10qaeTCH B CTa6HJil13aUHl1 Me)l(KJieTO'IHOfOMaTPl1KCa coe,n:HHHTeJihHOH TKaHH H YMeHbIIIeHH11 rrpoH11uaeMocT11 KaIIHJIJIHPOB. MHortte rrpe,n:crnBHTeJIH rpyrrIIhI BHTaMHHa p 06na,n:a10T rnrroTeH3HBHhIM nettCTBHeM.

fHIIOaBHTaMHH03a BHTaMHHa p xapaKTepH3yeTCH IIOBhJIIIeHHOH KPOBOTO'IHBOCTbIO,n:eceH H TO'le'IHbIMHIIO,ll,KO)l(- HbIMH KpOBOH3JIHHHHHMH, o6mett CJia60CThIO, 6bICTpott YTOMJIHeMOCThIO H 60JIHMH B KOHe'l- HOCTHX.

B Ta6JIHUe 3-2 rrepe'IHCJieHhI cyTO'IHhre rroTpe6HOCTH, Ko<t>epMeHTHbie <t>opMbI, OCHOBHbJe 6HOJIOfH'leCKHe<l>YHKUHH BO,ll,opacTBOpHMbIX BHTaMl1HOB, a TaKlK:e xapaKTepHhie rrpH3HaKH aBHTaMHH030B.

Ji. )J{JfPOPACTBOPHMhIE BHTAMHHhl

UHKJIH'lecKHtt,He-

HaChIIUeHHhIH, O,IJ,HOaTOMHhlll CTIHpT.

:Ucro'lllHKH. BHTaMHH A co,1J,eplK:HTCH TOJihKO B lK:HBOTHhIX rrpo,UYKTax: rre'leHHKPYIIHOro poraToro CKOTa H CBHHett, HH'IHOM)f(eJITKe, MOJIO'l- HhIX rrpo,UYKTax; oco6eHHO 6oraT 3THM BHTaMHHOM ph16Htt lK:Hp. B pacTHTeJihHbIX rrpo,UYKTax (MOPKOBh, TOMaThl, nepeu, canaT H .np.) CO,ll,eplK:aTCH KapOTHHOH,IJ,bl, HBJIHIOIUHeCH rrpOBHTaMHHaMH A. B cJIH3HCTOH o6ono'IKeKHrne'IHHKaH KJieTKax ne'leHHco,n:ep)f(HTCH cneutt<l>H'lecKHtt <t>epMeHT KapOTl1HJl:HOKCHreHa3a, npeBpama10-