підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

6one3HH, rrpoTeKaIDlll,He c paJJiwrno:U cTerreHhID T5DKeCTH, BIIJIOTb LlO JieTaJibHOfO HCXO):la elll,e LlO po)l(LleHHH HJIH s rrepsb1e MeCHUhI rrocne po)l(UeHHH.

B. IlPHOBPETEHHblE IIPOTEHHOIIATHH

JI106aH 6one3Hb corrpOBO)l(UaeTCH H3MeHeHHeM 6eJIKOBOf0 COCTaBa opraHH3Ma, T.e. pa3BHBaeTCH rrpHo6peTeHHaH rrpoTeHHOIIaTHH. IlpH 3TOM nepBHlJ:HaH CTPYKTYPa 6eJIKOB He ttapyrnaeTcH, a o6bIIJ:- Ho rrpOHCXOLlHT KOJIHIJ:eCTBeHHOe H3MeHeHHe 6eJIKOB, oco6eHHO B Tex opraHax H TKaHHX, B KOTOpblX pa3BHBaeTcH rraTonornqecKH:H rrpouecc. HarrpHMep, rrpH rraHKpeaTHTax CHIDKaeTCH Bbipa6oTKa <i>epMeH-

TOB, Heo6XOLlHMbIX LlJlH rrepesapHBaHHH IIHlll,eBbIX Belll,eCTB B )J(KT.

B HeKoTOPhIX cnyqaHx rrpHo6peTeHHhie rrpOTeHHorramH pa3BHBa10TCH B pe3yJihTaTe H3MeHeHHH ycnosH:U, B KOTOphIX <t>yma..i:HoHHPYIDT 6enKH. TaK, rrpH H3MetteHHH pH cpeLlhI s 1UeJIOIJ:Hy10 cTopotty (anKaJI03hI paJJIHIJ:Ho:U rrpHPOLlhI) lf3MeHHeTCH KOH- <t>opMaUHH reMorno6Htta, yseJIHIJ:HsaeTcH ero cpoLlcrno K 02 H CHIDKaeTCH LlOCTaBKa 02 TKaHHM (rHIIOKCHH TKaHe:U).

Ylttor):la B pe3yJihTaTe 6one3HH rroBhIIIIaeTcH ypoBeHh MeTa60JilfTOB B KJieTKax H CbIBOPOTKe KPOBH, lJ:TO rrpHBOLlHT K MOLlH<l>HKaUHH HeKOTOpblX 6enKOB H HapyrneHHIO HX <l>YHKUHH. TaK, IIOBbIIIIeHHbie KOHUeHTpaUHH fJIIOK03bl B KpOBH rrpH caxapHOM LlHa6eTe rrpHBOLlHT K He<t>epMeHTaTHBHOMY rrpHCOe):lHHeHHIO ee K 6eJIKaM (fJIHK03HJilfpOBaHH10

6eJIKOB). IIpHMepoM MO)l(eT CJIY)l(HTh rrosb1IIIeHHe ypoBHH rnHK03HJilfposattttoro reMorno6Htta a 3pHTpoumax, IJ:TO ysenHIJ:HsaeT ero cpOLlCTBo K 0 2 H CHIDKaeT TPaHCIIOPT 02 B TKaHlf. fJIHK03HJIHposaHHe 6eJIKOB xpycTaJIHKa rJia3a (KPHCTaJIJIHHOB) rrpHBOLllfT K ero IIOMYTHeHHIO H pa3BHTH10 KaTapaKTbl.

KpoMe Toro, H3 KJieTOK rrospe)l(Llettttoro opratta B KpOBb MOryT BbIXOLlHTh 6eJIKH, KOTOPbie B HOpMe orrpe):leJIHIOTCH TaM JIHIIIb B CJie):lOBbIX KOJIHIJ:eCTBax. IIpH paJJIHIJ:HhIX Ja6onesattHHx IJ:acTo Hcrronb3YIOT 6JfOXHMHIJ:eCKHe lfCCJie):loBaHHH 6eJIKOBOfO COCTasa KPOBH LlJlH YTOIJ:HeHHH KJIHHHIJ:ecKoro LlHarHo3a. HarrpHMep, rrpH rrattKpeaTHTe s KPOBH noBhIIIIaeTcH aKTHBHOCTb rraHKpeaTHIJ:eCKOM aMHJia3bl (<t>epMeHTa, yqacTBYIOIUero B paclll,eIIJieHHlf KpaxMana), KOTOpaH B HOpMe He LlOJI)l(Ha IIOIIa):laTb B KPOBh, a no rrpOTOKY IIO):l)l(eJiy):lOlJ:HOM )l(eJie3bl Bbl):leJIHeTCH rrpH IIHlll,eBapeHHH B LlBeHMuamrrepCTHYID KHIIIKY (CM. Pa3LleJI 2).

B HeKOTOPhIX cnyIJ:aHx 6HOXHMHIJ:eCKHe LlaHHhie 06 H3MeHeHHH 6eJIKOBOfO COCTasa KpoBH HJIH MOlJ:H MOryT CTaTb Be):lylll,HMH rrpH IIOCTaHOBKe LlHafH03a. HanplfMep, rrpH MHenoMe (3JioKaIJ:eCTBeHHOM rrepepo)l(UeHHH IIJia3MaTHIJ:eCKHX KJieTOK, CHHTe3HPYIOIUHX lfMMYJIOfJI06yJilfHbl) B KpoBH H MOIJ:e rroHBJIHIDTCH 6emrn Bettc-,ll,)l(Ottca, KOTOpb1e B HH3KHX KOHUeHTPauHHX IIPlfCYTCTBYIOT H B KPOBH 3LlOpOBbIX JIIO):leM. 3TH 6eJIKH rrpe):lCTaBJIHIOT co6o:U nerKHe uerrH HMMyttorno6ynHHa G, ClfHTe3 KoTophIX ycHJIHsaeTcH s 3JIOKaIJ:ecrnetttto rrepepo)l(LleHHhIX KJieTKax.

I. 06lUASI XAPAKTEPVICTVIKA

<l>EPMEHTOB

KAK 6VIOfl0fVILIECKVIX KATAflVl3ATOPOB

<t>epMeHThI, KaI< 6bmo yCTaHORJleHO ern,e B 1922 r., 51BJUllOTC51 6eJIKaMM. l1x poJib )'HMKaJibHa:OHM )'Bemt-

trnBalOT CKOpOCTb rrpOTeKaHH51 XHMWieCKOfi pea~lll,

OJIHaKo rrpM 3TOM He pacxO)lYJOTCH. B 1926 r. 6bIJI BrrepBbie O'llilll,eHH Bbl)leJieH B BH)le 6eJIKOBbIX KpHCTaruIOB ¢epMeHT ypea3a, KaTaJUi3MPYIOIIJ;I-IB pea.KIJllll pacw:eITJieHl151 MoqeBMHbl )lO aMMHaKa M )lHOKCH)la yrnepo)la. K HaCTOHW:eMY BpeMeHH B KpMcrnnm1qecKOM BH)le rronyqeHbI COTHH Pa3.JIWIHbIX ¢epMeHTOB, pacurn¢poBaHbl l1X aMHHOKHCJIOTHbie ITOCJie)lOrnlTeJibHOCTH, H3y•-meTC51 HX pOJib B MeTa60JIHqeCKMX rrpeBpameHHHX.

B poJIM 6MoKaTaJIM3aTOpos MOryT BbICTyrraTb M He6eJIKOBhie coe)lHHeHHH. HarrpHMep, HeKOTOpb1e Tl111bI PHK Bhl3hIBaIOT fl1Jlpomn ¢oc¢oJlM3cPMPHbIX CBH-

Jett ttyKJieMHOBbIX KHCJIOT. TaKHe MOJieKYJihI PHK c KaTaJIMTJfqeCKOtt aICTMBHOCTblO Ha3blBaJOT pM603MMaMM, O)lHaKO l1X 3HaqeHMe B XHMMqecKOM rrpespa- w:eHMM COe)lMHeHMtt HaMHOro MeHhWe, lJ:eM y ¢epMeHTOB.

CTocKOJibKY ¢epMeHTbl - 6eJIKOBbie MOJieKYJibl, CJie)lOBaTeJibHO, OHM o6Jia)laIOT BCeMM CBOttCTBaMH, xapaKTepHbIMH llJUI 6eJIKOB. B TO )Ke speM51 OHM HMeIOT oco6eHHOCTH CTpOeHH51, xapaKTepH3YIOW:He HX KaK KarnmnaTOpbr. PaccMoTpHM ocHOBHhie CBOttCTBa ¢epMeHTOB KaK 6MOJIOfHqecKHX KarnmnaTopoB.

A. CnELJ,H<1>wrnocTh

EttonornqecKa51 cPYHKUHH ¢epMeHrn, KaK H n106oro 6enKa, o6ycnoBJieHa HanwrneM Bero cTpyKzype aKTMBHOro ueHTpa. JlMraH)l, B3aHMO)le:ficrnyIOW:HH c aKTHBHbIM ueHTpOM ¢epMeHTa, Ha3bIBaIOT cy6cTpaTOM. B aKTHBHOM ueHTpe ¢epMeHrn ecTb aMHHOKHCJIOTHbie OCTaTKH' cPYHKUHOHaJibHbie rpyrrrrhI KOTOphrx 06ecrreqttsa10T CB513brnam1e cy6cTparn, H aMHHOKHCJIOTHbie OCTaTKH, cPYHKUHOHaJibHbie rpyrrnhI KOTOpbIX ocyrn,eCTBJI51IOT XHMMqec Koe npeBpaw:eHHe cy6cTparn. YcnoBHO 3TH rpyrrnhI o603HaqaIOT KaK yqacTOK CB513bIBaHH51 cy6CTparn H KaTaJIHTHqeCKHtt yqacTOK, O)lHaKO CJie- )lyeT TIOMHHTb, l.J:TO He scer)la 3TH yqaCTKH HMeIOT qfaKoe npoCTpaHCTBeHHOe pa3)leJieHHe H }fHOf)la MOryT «IIepeKpbIBaTbCH» (pMC. 2-1 ).

B yqacTKe CB513hIBaHH51 cy6cTpaT np11 rroMOIIlH HeKOBaJieHTHbIX CB513ett B3aHMO)leticrnyeT (CB513blBaeTC51) c ¢epMeHTOM, ¢opMHPYH ¢epMeHT-cy6- CTpaTHbI:fi KOMITJieKc. B KaTaJIMTMlJ:ecKoM yqacTKe cy6cTpaT npeTeprreBaeT XHM11qecKoe rrpesparn,eHMe B rrpO)l)'KT'KOTOpbltt 3aTeM BbICB060)l()laeTC51 H3 aKTHBHoro ueHTpa ¢epMeHrn. CxeMaTMqHo rrpouecc KaTaJIH3a MO)l(HO npe)lCTaBHTb CJie)lyIOlUHM ypaBHeHHeM:

E + s H ES H EP H E + P,

r)le E - ¢epMeHT (3H3HM), S - cy6CTpaT, P -

rrpOJlYKT. )1,aHHhie o6o3HaqeHMH o6w:enpMH51TbI 11 npOHCXO)lHT OT aHrJIHMCKHX CJIOB enzyme, substrat,

product.

Cneu11¢WIHocTb - Hatt6onee Ba)l(HOe csoi1cTBo ¢epMeHTOB, onpe)leAAIOIIlee 611onomqecKyio 3HalJ:H- MOCTb 3THX MOJieKYJI. PaJJimaIOT cy6cTpaTHYIO M KaTaJIHTmeCKYJO crreuM¢mHocTM ¢epMeHTa, onpe)leAAeMhie CTpOeHHeM aKTHBHOro ueHTpa (pttc. 2-2).

1. Cy6cmpamullR cneu,U<pu'luocmb

Ilo)l cy6cTpaTHOH cneu11¢w-rnocTbIO noHMMaIOT cnoco6HOCTb Kax)J,Oro ¢epMeHTa B3aHMO)letiCTBOBaTb JIMWb c O)lHHM MJIM HeCKOJibKHMH onpe- )leJieHHbIMH cy6cTparnM11. Pa3n11qa10T:

•a6COJIIOTH)'IOcy6cTpaTHYIO crrew1¢11qHOCTb;

•rpyrrnoByIO cy6cTpaTHYIO cneuMcPMlJ:HOCTb;

•cTepeocrreu11¢11qHocTb.

A6coJIIOTHrui. cy6cTpaTHrui. cneuml>H11noCTb

AKTHBHbIH uemp ¢epMeHTOB, o6na)laI01IIHX a6conIOTHol1: cy6cTpaTHOH cneu11¢HlJ:HOCTbIO, KOMITJieMeHTapeH TOJihKO OJlHOMY cy6cTpazy. Cne)lyeT OTMeTHTb, l.J:TO TaKMX ¢epMeHTOB B )JCHBbIX opraHH3Max MaJIO.

Ilp11Mep ¢epMeHTa c a6COJIIOTHOH cy6cTpaTHOH crreu11¢HlJ:HOCTblO - aprnHa3a, KaTaJIH3HPYJOW:aH peaKUHIO paCIIlenJieHHH apmHHHa )lO MoqeBHHbl H opHMTHHa:

NH2

I

C=NH

J],pyroti rrpttMep cpepMeHTa c a6COJIIOTHOH cy6CTpaTHOM crreuttcp.wIHOCTblO - ypeaJa, KaTa.JIM3MPYIOrn:aSI rw1pomn MOlfeBHHbI JJ:O JJ:HOKCHJJ:a yrnepoJJ,a M aMMttaKa.

~Hz Ypea3a

C=O + H20 ______. C02 + 2NH3.

I

NH2

fpynnoBaH cy6cTpaTHaH cne~clHl'IHOCTb

EoJiblllMHCTBO cpepMeHTOB KaTaJIHJttpyeT OJJ,HOTMITHbie peaKUMM c He60JibllIHM KOJIHlJeCTBOM (rpyrrrroH) CTPYKTYPHO rroxmrmx cy6cTpaToB.

TaK, cpepMeHT rrattKpeaTttlJecKaSI nttna3a KarnJIM3ttpyeT rn,IJ,pOJIM3 )((l1P0B B JJ,BeHaJJ,UaTMrrepCTHOH KHllIKe qeJIOBeKa, KaTaJIH3HPYSI rrpeBparn;eHMe JllO-

6otf MOJieKYJibl )l(Hpa (TPHaUHJirJIMUepona) )l;O MOJieKYJlbl MOHOaUHJirJIMUepona M ,11,Byx MOJieKYJI BblCllIHX )l(ttpHbTX KHCJIOT. CTattKpeaTttlfecKaSI JittrraJa fH.[(POJIHJyeT 3cpMpffY10 CBSl3b y a-aTOMOB yrnepoJJ,a rnnuepona, HeJaBHCHMO OT Toro, KaKHe )l(HpHbie KHCJIOThl BXOJJ:SIT B COCTaB MOJTeKYJTbI )l(Mpa (CM. cxe-

MY Ha C. 4).

EoJTbllIHHCTBO rrpoTeOJIHTMlJecKHX cpepMeHTOB, ocyrn:eCTBJISIIOrn;MX rH,!1,pOJIM3 6eJIKOB, HMeeT rpyrrIIOBYIO cy6cTpaTHYIO crreuncpwrnocTb, rH.[(pOJIM3Y51 rrerrTM.[(Hbie CB513M, o6pa30BaHHbie pa3HbIMM aMHHOKHCJIOTaMM.

CTepeocnenuclJH11uocTb

IlpM HaJI.wIHH y cy6cTPaTa HeCKOJibKHX CTepeoH30MepoB ¢epMeHT npoHBJISieT a6co.JI10Ttty10 crre-

Y'lacTOKces:13b1BaH111s:1

l

06ecne4111eaer cy6crpaTHYIO cne4111cp111'1HOCTb

(eb16op cy6crpara)

1

-a6co1110THasi cy6crparHas:i cne4111cplll'IHOCTb

-rpynnoeasi cy6crparHaff cne4111cplll'IHOCTb

-crepeocne4111cp1114HOCTb

KaTaI1lllT111'1ecK111MyYacTOK

l

06ecne'1111eaerBb16op nyrn XlllMlll'leCKOfOnpeepall.10Hlllff ,QaHHOro cy6crpara

l

Cne4111cp111'1HOCTbnyrn

npeepall.leHll!ff

u:mpH'IHOCThK O.UHOMY H3 HIDC. B opraHH3Me qeJIOBeKa Ha6JIIO,llaIOT cneumpH'IHOCTb<t>epMeHTOB K CJie,llyK>IUHM CTepeOH30MepaM.

CrepeocneD,H<J>ll'IHocTbK D-caxapaM. BoJiblllHHcTBO MOHOCaxapH,llOB H npo.uyKTOB ill( o6MeHa B opraHH3Me qeJIOBeKa H .UPYfIDC MJieKOTIHTaIOIUHX OTHOCHT K D-crepeomoMepaM. <I>epMeHThI, ocymeCTBIDIIOIUHe HX MeTa60JIH3M, HMeIOT cneutt<f>H'lHOCThK D-, a He K L-caxapaM.

~00•AT• """"'"'M~~,H, + A,Q<P

CTepeocneD,H<J>ll'IHoCTbK L-aMHHOKHCJIOTllM. EeJI-

KH qeJIOBeKa COCTOITT H3 aMHHOKHCJIOT L-pMa.

EoJihlllHHCTBO <f>epMeHTOB, o6ecrretIHBaIOIUHX

npespameHHe aMHHOKHCJIOT, HMeeT cTepeocne-

UH<f>HtIHOCTh K L-aMHHOKHCJIOTat>f.

CTepeocneIU14>1111uocTh K IU1C-Tpauc-a10MepaM.

<I>epMeHT <l>YMapa3a OKa3bIBaeT .ueliCTBHe TOJibKO Ha <l>YMapaT. ManeirnaT (UHC-H30Mep <f>y- Mapam) He .sIBJI.sieTcH cy6cTpaTOM <l>YMapa3bI.

H H

I I

C=C

I I

·ooc coo-

Mane11HaT

J1CKJIIOl.fem1e COCTaBJUllOT TOJibKO <f>epMeHTbI 3TIHMepa3bl (paueMa3hI), KaTaJil1311PYJOIUHe rrpeBpameHHe OIITII'leCKHX1130MepoB.

CrepeocneD,H4>H'IHOCTbKa- H P-rJIHK031l!lllbIM ces- lJIM. <l>epMeHT aM11J1a3a .n:eM:crnyer TOJibKO Ha a- rJIHK03HJJ:Hbie CB513ll, lfTO II03BOJI5IeT rH)J,poJIH30BaTh KpaxMaJI 11 rJIHKoreH (rroJIHMepbI rmoK03hI), ocraTKH rmoK03hI B KOTOPhIX coe}l.MHeHhI a-r.Jili- K03HJJ:HhIMH CBH3HMH. UeJIJI10JI03a - TIUOKe rroJIHMep rJIIOK03bI, OJJ:HaKO OCTaTKH rJIIOK03bl B HeM CBH3aHbI P-rJIHK0311)J,HbIMH CB513HMH. B pe3yJibTare OTCyrcTBHH y qeJIOBeKa <t>epMeHTOB, crreUH- <PMl.fHhIX K P-rJIMK03HJJ:HOM CBH311, ueJIJIIOJI03a He rn:Iij)OJIMJYeTCH B KIDIIelfHHKe qeJIOBeKa 11 He MO- )f(eT CJI}')KHTh 11CTO'IHHKOMfJIIOK03bl.

2. Kamll.llumu11ecKaa cneu,utf>u11nocmb

<l>epMeHT KaTaJI11311pyeT rrpeBpameH11e rrp11coe-

.n:11HeHHoro cy6crpaTa ITO OllHOMY 113 B03MO)f(HbIX rryTeM: ero rrpeBpameHHH. 3ro cBottcTBO 06ecrreq11BaeTcH CTpoeHl1eM KaTaJil1Tl1lfeCKOf0 yqaCTKa aKTl1BHOfO ueHTpa <PepMeHTa 11 Ha3bIBaeTCH KaTaJll1Tl11.feCKOM crreu11$MlfHOCTblO, 11Jil1 crreu11$11qHOCTblO rryT11 rrpeBpamemrn cy6cTpaTa. TaK, MOJieKYJia rn10Ko3o-6-<Poc<Parn B KJieTKax rrel.fett11 qeJIOBeKa - cy6cTpaT 4 pa3JIHl.fHblX <PepMeHTOB: <Poc- <PorJIIOKOMYTa3bl, rn10Ko3o-6-<Poc<PaT<Poc<PaTa3hI, <Poc<Porn10Kom0Mepa3b1 11 rn10Ko3o-6-<Poc<PaT.n:e- rH,UporeHa3bI. 0.n:HaKo 113-3a oco6eHHOCTeM: cTpoeH11H KaTaJil1TMlfeCKMX yqacTKOB 3TMX <PepMeHTOB rrpOMCXOJJ:MT pa3Jil11.fHOe rrpeBpameH11e 3TOf0 co- e,IJ,I1HeHM51 c o6pa3oBamieM 4 pa3JI11qHhIX rrpo,IJ,yKTOB (CM. cxeMy HIDKe).

.6. KATA.JUtTJlqECKASI 3<1><1>EKTHBHOCTh

b0Jlbllll1HCTBO KaTaJI11311pyeMblX <PepMeHTaMl1

peaKUMM BblCOK03<P<PeKT11BHhl, OHl1 rrpoTeKalOT B

108-10 14 pa3 6hICTpee, l.feM HeKaTaJIM3MpyeMbie peaKU1111. Krut<,u,ag MOJieKYJia <PepMeHTa crroco6Ha 3a

CeKYHJJ:Y TpaHc<PopM11poBaTb OT 100 llO 1000 MOJieKYJI cy6cTparn B rrpOJJ:YKT.

KoJittl.fecTBo MOJieKYJI cy6cTparn, rrpeBpameHHhIX B ITPOllYKT c ITOMOIUblO OJJ:HOM MOJieKYJibl <PepMeHTa 3a 1 c, Ha3bIBalOT lfMCJIOM o6opOTOB <PepMeHTa, MJil1 MOJrnpHOM aKTl1BHOCTblO.

B. JIABHJihHOCTh <l>EPMEHTOB

KaTaJI11T111.fecKaH 3<P<PeKTl1BHOCTh <PepMeHTa, KaK 11 JII06ow 6eJIKOBOW MOJieKYJihl, 3aBMCMT OT ero KOH$OpMau1111, 11 B l.faCTHOCTM OT KOH$OpMau1111 aKTMBHOfO ueHTpa.

,[(JUI <PepMeHTOB xapaKTepHa KOH$OpMaUMOHHaH Jia6MJibHOCTb - crroco6HOCTb K He60JibllIMM 113MeHeHl15IM HaTl1BHOW KOH$OpMau1111 BCJie.D:CTBl1e pa3pbrna cJia6bIX CBH3etl. Tio3TOMY B03.D:ei1cTB11e

.n:eHarypwpyiom11x areHTOB, crroco6HblX 113MeH51Tb KOH$OpMau1110 MOJieKYJibl <PepMeHTa, rrp11BOllHT K 113MeHeHl110 KOH$OpMau1111 aKTMBHOfO ueHTpa 11 CHIDKeHl110 crroco6HOCTl1 rrpwcoe.D:MH51Tb cy6cTpaT.

B pe3yJihTaTe 3Toro YMeHhlllaeTcH KaTaJIMTMl.fecKaH 3<P<PeKTMBHOCTb <PepMeHTa.

r. CnoCOBHOCTh <l>EPMEHTOB K PErYJUIU,HH

AKTl1BHOCTb <PepMeHTOB B KJieTKe 3aBl1Cl1T OT KOJIHlfeCTBa MOJieKYJI cy6cTparn, IIPOllYKTa, HaJil1lfl151 Ko<PaKTOpOB 11 Ko<PepMeHTOB. ,UettCTBMe <PepMeHTOB B KJieTKe, KaK rrpaBMJIO, crporo yrropMo- 1.feHo: rrpo.n:YKT o.n:How <PepMeHTaTl1BHoi1 peaKU1111 5IB.IDieTC51 cy6cTpaTOM .n:pyroM:, 06pa3y51 Ta.KMM o6pa30M «MeTa6om1qecKtte nyrtt>>. Cpenn: MHO)f(ecnm <PepMeHTOB npaKTl1lfeCKM K~OfO MeTa60Jil11.feCKOf0 rryTl1 pa3JIHl.falOT KJIIOl.feBbie, MJil1 peryJIHTOpHhie, <PepMeHTbI, aKTMBHOCTb KOTOpbIX MO)l(eT ll3MeHHTbC51 B 3aBllC11MOCTl1 OT rrorpe6HOCTl1 KJieTKM B KOHelfHOM IIPOllYKTe MeTa60JIMl.feCKOf0 IIYTH. PeryAATOpHb1e <PepMeHThI pacrroJIO)l(eHhI, KaK rrpaBMJIO, B HalfaJie H/tt:JIM B MeCTe pa3BeTBJieHH51 MeTa60JIMlfeCKOfO nyrtt. OHM KaTaJIM3Mpy10T JIM6o

caMbie Me,UJieHHbie (CKOPOCTb-JIHMHTHPYJOIUHe peaKUHH), n1160 Heo6paTHMbie peaKUHH. Ilo;::i:po6Ho 0 TOM, KaK ocymecTBJUieTCSI KOHTIJOJib MeTa60Jil13Ma nyTeM perymIUHH aKTHBHOCTH <l>epMeHTOB, on11caHo B nonpa3;::i:ene VI I.

II. KJlACClf1C!>lf1KAUlf1SI

lf1 HOMEHKJlATYPA C!>EPMEHTOB

KillK.llhIH ¢epMeHT HMeeT 2 Ha3Battl1H. Ilepooe - KOPOTKoe, TaK HaJbrnaeMoe pa6oqee, y;::i:o6ttoe ,UJIH noBcenHeBHoro ttcnonb30BaHHS1. BTopoe (6onee nonHoe) - CHCTeMaTHqeCKOe, np11MeHS1eMOe MSI O)Uf03HaqHOH ll)leHTHcPHKau1111 <t>epMeHTa.

A. pABQqEE HA3BAHJIE

B Ha3BaH1111 6oJibllll1HCTBa ¢epMeHTOB co;::i:ep)KJITCSI cy¢¢HKc «aJa», npttcoe;::i:11tteHHhlli K HaJBaHHIO cy6CTIJaTa peaKUHH, ttanp11Mep ypea3a, caxapaJa, n11na3a, ttyKJiea3a llJIH K Ha3BaHMIO XHMW:leCKOro npeBpamettmr onpe;::i:enettttoro cy6cTPaTa, ttanp11Mep naKTaT)lerHL1:poretta3a, anettHJiaTUHKJia3a, <t>oc<t>o- rnIOKOMYTa3a, nttpyBaTKap6oKCHJia3a. Cornactto pOCCMHCKOH KJiaCCHcPMKau1111 <t>epMeHTOB (K<l>), Ha3BaHHSI ¢epMeHTOB IlllIIYTCSI CJIHTHO. 0)lHaKO B ynorpe6neH11M coxpaHHJICH PH.ll: TPHBHaJihHhIX, 11cropttqeCKH 3aKpeIUieHHbIX Ha3BaHHH ¢epMeHTOB, KOTOpble He )laIOT npe;::i:CTaRJieHHSI HM 0 cy6CTPaTe, HH o nme XHMlfllecKoro rrpeBpamemrn, ttarrpHMep TPHTICMH, nerrc11tt, pettMH, TPOM611H.

B. K.!IACChl <llEPMEHTOB

Me)l()J,yttapo;::i:HbIH coI03 6ttoXHMttll H MOJieKYJrnpHOH 6HOJIOrHH B 1961 r. pa3pa60Tarr CHCTeMaTHqeC- KYID HOMeHKJiarypy, cornacHo KOTopoit ace ¢epMeHTbI pa36llTbI Ha 6 OCHOBHbIX KJiaCCOB B 3aBMCHMOCTH OT Tttna KaTaJil13ttpyeMOH XHMMqecKOll peaKUHM. KillKJlbIH KJiacc cocToHT 113 MHoroqttcnettHhIX rro;::i:- KJiaccoB M no;::i:rro;::i:KJiaccoB c yqeroM rrpeo6paJyeMoti

XHMmecKoH: rpyrrrrbI cy6cTPaTa, noHopa M aKUemopa rrpeo6pa3yeMbIX rpyrrrmpoBOK, HaJIHqMSI ,ll;OITOJIHHTeJibHbIX MOJieKYJI ll T.)l. KillK)lbIH M3 6 KJiaCCOB HMeeT CBOM TIOPMKOBbIB HOMep, CTPOfO 3aKpeITJieHHbIB 3a HHM.

1. OiccuiJopeiJyKma:Jbt

KaTarrl13ttpyIOT pa3n11qHbie OKHCJIHTeJihHO-Boc- CTaHOB11TeJibHhie peaKUHH c yqaCTHeM 2 cy6cTpaTOB (rrepeHOC e- JUIH aTOMOB BO,ll;Opo;:i:a c O)lHOro cy6cTparn Ha ;:i:pyroti).

C11cTeMaT11qecKoe Ha11Mett0Batt11e ¢epMeHTOB COCTaBJISIIOT no cPOPMYJie «,ll;OHOp: aKUerrTop-oK- Cll.ll:OpeeyKTa3a», pa6oqee - cy6cTpaT-no;:i:KJiacc OKCH)lOpe)lyKTa3.

.IJ:envqioreHa3bI. B 3TOT rrollKJlacc BXOMT cl>epMeHTbI, KaTaJIB3l1PYJOIIDie peaKUHl1 ;:i:ern.upttpoBattMSI (oTIIIermeHAA Bo;:i:opo;:i:a). B KaqecTBe aKuenropos 3JielCTpOHOB HCilOJlb3YIOTCSI Kocl>epMeHTbl NAO+, NADP+, FAD, FMN (cM. HIDKe). Bee <t>epMeHTbI 3TOH rpyrrIIbI 06m1,1::i;aIOT BbICOKOH cy6CTPaTHOH cneu11<l>mHocThIO. flpHMep peaKUHH:

0Kclf,ll,a3bI. AKuerrTopoM 3JieKTJJOHa CJIY)KHT MO-

JieKYllilPHhiit KHCJIOpo;::i:. IlpttMep peaKUHH, Ka-

TaJil13ttpyeMOH Ul1TOXPOMOKCH.ll:a3oit:

l..llATOXpOMOKCIAA838

;:i:a M3 MOJieKYJibI KHCJiopo;::i:a rrp11coe;::i:11IDieTcS1 K cy6cTpary. Ilp11Mep peaKu1111:

2. Tpancf/>epU3bt

KaTaJill3llPYJOT nepeHOC cPYlil<I..I.MOHaJibHblX rpyrrrr OT o)J,Horo coemrnemrn K llPYfOMY· Ilo.upaJ.D:eJISDOT B 3aBMCMMOCTI1 OT nepeHOCMMOH rpyrrIIbI.

HaJBaHMe 3THX cPePMeHTOB COCTa.BJUIJOT no cl:>oPMYJie <<.uoHop: aKI..J,eIITOp--'fPa.HCnopTMpyeMaHrpyrrrra- TpaHcct>epa3a». K KJiaccy TpaHcct>epa3 OTHOCHT aMMHOTpa.HCcPePaJhl, a.iwmpaHCcPePaJbI, MernJITPa.HCcPePaJbI, fJIMK03MJITPaHCcPePa3bI, KMHa3bl (ct>occt>o- Tpa.HCcPePa3bI). IlpMMepbI peaKJ.Oill (cM. cxeM)'A).

3. I'uiJpo.!lU3bl

KaTanmnpYJOT peaKUnn rn.uponn3a (pacmeIIJieHHH KOBaJieHTHOH CBH3ll c npncoe.unHeHneM MOJieK)'JiblBO.Ubl no Meczy pa3pbrna). Ilo.upa3,UeJIHIOT B 3aBHCHMOCTM OT pacmeIIJIHeMOH CBH3H.

Ha11MeHOBattne ct>epMeHToB coCTa.BIDIJOT no ct>opMyne «cy6cTpaT-m.upona3a» HJIH npHMbIM npnco-

e.unHeHHeM K Ha3BaHHIO cy6cTpaTa cyct>ct>MKca «a3a», HanpMMep npoTea3a, JIHIIaJa, ct>occt>onnnaJa, p1160JIYKJ!ea3a. IlpMMep peaKUHH (cM. cxeM)'E).

,UJrn OT,UeJibHbIX KJiaCCOB fHllPOJia3 npnMeHMMbl cneU:HaJibHbJe TepMMHbl, xapaKTepM3yJOI.JJ;Me fll,U- POJIM3 onpe.ueneHHo.H XMMWiecKo.H CBH3H: 3CTepa3bI, ct>occt>arn3bI n .up.

4. Jlua3bt

K n11a3aM OTHOCHT ct>epMeHTbI, OTIIIeIIJIHIOIIIMe OT cy6cTpaTOB Hern.upoJIMTHqecKMM n)'TeMonpe-

.ueneHHYIO rpynny (npH 3TOM MOf)'TOTIIIenJIHTbCH co2, Hp, NH2, SH2 H .up.) HJIH np11coe,UHHHrom11e qame Bcero MoneKyny BO.Ubl no .UBOHHOtt CBH3ll.

Ha11Mett0Batt11e ct>epMeHTOB cocTaBJIHIOT no ct>opMyne «cy6cTpaT-oTI.JJ;eIIJIHeMaH HJIM npttcoe-

.UHHHeMaH rpyrrnHpOBKa». IlpHMepbI peaKU:HH (CM. cxeMy B).

CH3

I

HC-NH2

I

COOH

AnaHl.1H

I

+(CH2)2

I

COOH

a-KeromYTapoeaf! Klilcnora

npoTel.1HK"1Ha3a

5. H30Mepa3b1

KaTaJIH3MPYIOT pa3JmqHhie BHYfPMMone1<ym1pHhie npeBpa:r.u;emra. Ilo.u.pa3,l1.eJUllOT B 3aBMCMMOCTM OT TMna peaKUMM M30MepM3al.l,MM.

Kai<: o6:r.u;ee Ha3BaHMe ¢>epMeHTOB 3Toro KJiacca npMMeIDUOT TepMMH «M30Mepa3bl», HanpMMep (CM. cxeMY A).

1130Mepa3bl MOiyr KaTaJIM3MpoBaTh BHYTPMMOJieKYIDIPHbie OKMCJllITe.llhHO-BOCCTfilfOBMTeJihHhie peaKUMM, ocy:r.u;ecTBJIIDI BJaMMonpeBpa:r.u;emrn aJih)J.03 M KeT03, KeTOHHhIX M eHOJihHhlX rpyrrn, nepeMeIIJ;eHMH )J.BOllHhlx cmne:R BHYfPM MOJieK)'JlhI(cM. cxeMY B).

Kor.u.a M30MepM3aUMH COCTOMT BO BHYfPMMOJieKYJISIPHOM nepeHoce rpynnhI, ¢>epMeHT Ha3hIBaIOT «M)'Ta30H», HanpwMep (cM. cxeMY B).

6. Jluza:Jbt (cunmema:Jbt)

KaTaJIM3MpYJOT peaKUMM npwcoe)J.MHeHMH .u.pyr K .u.pyry )J.Byx MOJieI<yJI c o6pa30BaHMeM KOBaJieHTHOH CBH3M. 3TOT npouecc conpIDKeH c pa3phIBOM ¢>oc¢>o3¢>MpHo:R CBH3M B Mone1<yne AT<I> (mm .u.pyrnx HyKJie03M)J.TpM¢>oc¢>aTOB) MJIM c pa3pblBOM MaKp03prwqecKMX CBSl3e:R .u.pymx COe)J.MHeHMH. B nepBOM cnyqae (npw wcnoJih30BaHMM 3HeprMM rM,l1.pOJIM3a AT<I>) TaKMe ¢>epMeHThI Ha3hIBaIOT JIMra3aMM, MJIM CMHTeTa3aMM (CM. cxeMy f).

B cnyqae, Kor.u.a MCToqHMKOM 3Heprww CJIY)KMT mo6oe i:i:pyroe MaKpo3pmqecKoe coei:i:wHeHMe (He AT<I>), ¢>epMeHThI Ha3hIBaIOT CMHTa3aMM (cM. cxe- MY Ha C. 83).

0

II

C-0- Pol- l

HC-OH

I

H2C-0- Pol-

coo·

I

HC-0- P032-

1

H2C-0- P032-

B. CHCTEMATIIqECKOE HA3BAHIIE

B coorneTcrn1111 c KJiaccmp11Kal(11eii KIDK.UhIH <l>epMeHT IIOJiyq:HJI CHCTeMaT11qecKoe Ha3BaHHe, O,UH03HaqHo xapaKTep113yrorn;ee KaTMH3MpyeMyIO HM XHM11qecKYIO peaKl(HIO. HanpHMep, D-rm1l(e- PMh.Uem.u-3-<Poc<l>aT: NAD-oKcM,Uope.uyKTaJa (pa6oqee Ha3BaHHe - rnHl(epaJib.UerH,ll,<Poc<l>aT .uerH,ll,- poreHa3a). lfa Ha3Bamrn <l>epMeHTa cne.uyeT, qTQ cy6cTpaTOM 3Toro <PepMeHTa cny)KHT D-rn11l(e- paJih.UerM,U-3-<l>oc<l>aT, nm KaTaJIH311pyeMoif peaK- l(HH - OKHCJIHTeJibHO-BOCCTaHOBHTeJibHall B np11CYTCTBHH Ko<l>epMeHTa NAD+.

B 1972 r. KOMHCCHeH no HOMeHKJiazype 6110XHMHqecKHX coe.u11HeHHH Me)K)lyHapo,UHoro co103a reopeT11qecKoif 11 npMKJia,I(HOH XHMHH 6hmH npe.uno- )KeHbl «IIpaBHJia HOMem0IaTypbI <l>epMeHTOB»' HMeIOil(He KO.UOBOe qeTbipeX3HaqHoe l(H<l>posoe o6o3HaqeH11e, r.ue nepBas:I l(M<l>pa o6o3HaqaeT KJiacc <l>epMeHTa, BTOPall l(M<l>pa (nO,UKJiacc) YTOqmJeT npeo6pa3yeMyIO rpynmi:pOBKY, TpeTbH (no.urro.u- KJiacc) - yTOqIDJeT ,UOIIOJIHIITeJibHbIX yqaCTHHKOB peaKl(HH (Hanp11Mep, .uottopa 11 aKl(emopa) H qeTsepTaH - IIOpH,UKOBblH HOMep <l>epMeHTa B ,UaHHOH no.urpyrme. TaK, cpepMeHT MaJiaT.uer11.uporeHa3a MMeeT cwcTeMaTJ1qecKoe Ha3BaHHe L-MaJiaT: NADOKCM,Uope.uyKTaJa H KO,UOBbIB llIH<l>P 1.1.1.38. IllH<l>P 03HaqaeT, ~o 3TOT <PepMeHT OTHOCHT K nepBOMY KJiaccy <l>epMeHTOB - OKCM,Uope,n:yKTa3, OKHCJIHeMall rpynna - m.upoKCHJibHaH rpynm1posKa (1) B rrpMCYTCTBMH Ko<l>epMeHTa NAD+ ( 1) M rropH,UKOBbIH HOMep <l>epMeHTa B 3TOH rro.urpyrrrre - 38. Ko.uosyro HOMeHKJiazypy <l>epMeHTOB B OCHOBHOM HCITOJ1b3YJOT B HayqHOH JIHTepazype.

Ill. KOCl>AKTOPbl

IA KOCl>EPMEHTbl

EoJibllIHHCTBO <l>epMeHTOB ,UJIH rrpoHBJieHHH <l>epMeHTaTHBHOH aKTHBHOCTM H}')K,l(aeTCHB HH3KOMOJieKYJIHPHbIX opraHwqecmx coe.UMHemrnx He6enKOBOH rrpMpO,Ubl (Ko<l>epMeHTax) M/IDIH B MOHax MeTaJIJIOB (KocpaKTopax).

TepMMH «Ko<l>epMeHT» 6brn sse.ueH B HaqaJie xx BeKa 11 o603HaqaJI qacTb HeKOTOpbIX <l>epMeHTOB, 'KOTopaHnerKO OT,UeJIHJiaCb OT 6eJIKOBOH MOJieKyJibl <l>epMeHTa 11 y.uanAAach qepe3 nonynpoHMl(aeMyIO MeM6paHy npH .u11an113e. HecKOJibKO II03)Ke 6bmO Bbl.SICHeHO, qTo 60JibllIHHCTBO <l>epMeHTOB COCTOHT H3 TepMOJia6:HJibHOH 6eJIKOBOH qacTH 11 TepMOCTa6HJibHOro He6eJIKOBOf0 <l>aKTopa - Ko<l>epMeHTa. EenKOBall qacTh nonyqHJia Ha3BaHHe «ano<l>epMeHT»' KOTOPblH B OTCYTCTBHe Ko<l>epMeHTa He o6na.uaeT KaTaJIHTHqecKOH aKTHBHOCTbIO. Ko<l>epMeHT c 6eJIKOBOH MOJieKyJIOH (arro<l>epMeHTOM) <l>opMHPYIOT MOJieKyny XOJIO- <l>epMeHTa, o6na,ual0Il(YIO KaTaJIHTHqecKOH aKTHBHOCTblO.

A. KO<l>AKTOPbl

Eonee 25% scex <l>epMeHTOB .uJIH npoHBJieHHH IIOJIHOH KaTaJIHTHqecKOH aKTHBHOCTM H}')K,l(aeTCH B HOHax MeTaJIJIOB. PaccMOTpHM ponb Ko<l>aKTOpoB B <l>epMeHTaTHBHOM KaTaJIH3e.

1. PoJ1b MemaM06 B npucoeounenuu cy6cmpama

B aKmUBHOM u,enmpe t}JepMenma

lfoHbI MeTaJIJia BbIITOJIIDIIOT <l>YHKI.(HIO CTa6HJIH3aTOPOB MOJieKYJibl cy6cTPaTa, aKTHBHOrO l(eHTPa <l>epMeHTa H KOH<l>OpMal(HH 6eJIKOBOH MOJieKYJibI <l>epMeHTa, a HMeHHO TPeTJ1qHOH H qeTBeJJ'IWIHOH CTPYKTYP·

110Hbl MeTaJIJIOB - CTaOHJIH3aTOpbl MOJieKYJibl

cy6cTPaTa

~ HeKOTOpbIX <l>epMeHTOB CyOCrpaTOM CJIY)KHT KOMilJieKC npespamaeMOro Bell(eCTBa c HOHOM MeTaJIJia. Hanp11Mep, ,UJIH 6orrbIIIBHCTBa KHHa3 B KaqeCTBe O,UHOro H3 Cy6cTPaTOB BbICTYITaeT He MOJieKYrra AT<I>, a KOMnJieKc Mg2+-AT<I>. B 3TOM cnyqae HOH Mg2+ He BJaHMO)J,eHCTBYeT Henocpe,UCTBeHHO c <l>ePMeHTOM, a yqacmyeT Bcra6mrn3allHH MOJieKYJibI AT<I> 11 HeifTPaJIH3al(HH OTplil(aTeJibHOfO JaPHM cy6crpa-

Ta, ~o o6nerqaeT ero rrpwcoe,mrneHHe K aKTHBHOMy l(eJITpy <l>epMema (CM. cxeMy Ha c. 84).