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підручники. біохімія / Uchebnik_po_Biokhimii_E_S_Severin

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4-50).
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KHX 3K30HOB. B .upyr11x cnyqaJIX B 3pe11oti MPHK coxpaH51eTC51 liaCTb HHTpOHa H BKJIIO'IaeTC51 B coCTaB 3K30Ha c 5'HJIH 3'-Kottua. Crurntl:c11.Hr MO)l(eT BJTJ151Tb Ha BbI6op rrpoMOTOpa HJTH yqacTKa IIOJIH- a.n:eHtt1111poBaH 1151..

c IIOMOW,hIO aJihTepHaTHBHOfO CIIJiaHCHHra B rrpouecce CHHTe3a aHTHTe11 o6pa3yIOTCH MeM6paHOCBH3aHHbie J1 ceKpeTOpHbie cpopMbl aHTHTeJI (p11c. 4-54). TaK, rrepBoHa'IaJihHO B-n11Mcj:io- UHThI npo.n:yuwpyIOT TpaHCKpHIITbI, IIOJIHaJJ:eHHJIHPOBaHHbie IIOCJTe BTOporo CTOn-KO)],OHa, a HHTpott, B KOTOPOM HMeeTC51 nepBblH CTOil-KO,ll,OH, YD:aJIHeTC51. B pe3yJibTaTe CHHTe311pyIOTCH lgM,

CB513aHHbie c KJieTO'IHOH MeM6paHOH, TaK KaK MPHK TaKHX KJieToK co)lep)l(J1T Ha 3'-KoHue3K- 30H, KO!lMPYIOlllHH yqacTOK IIOJIHIIeIITHD:HOH uentt, COCT051W,11H 113 rH)lpocj:io6HbIX aMHHOKl1.CJIOT. c IIOMOW,bIO 3TOfO yY.aCTKa rrpOHCXO)lHT «3a51KOpHBaHHe>) lgM B MeM6patte. Kor.n:a B-JT11Mcj:iou11.ThI npeBpaw.aIOTCH B IIJia3MaTl1.Y.eCKJ1e KJieTKH, TO B

197

112 ,

a)

1 /~, 2 /~, 3 /~, 4

/', 5

 

6)

, •·~·-

3'

5'

 

 

 

'-?/ -................................

,.,,/

 

a)-/~,.~~,./~,./~,,

II

5'

 

 

3'

 

' .. ,/ ......

,/

'v'/

-.....,.,,;·

 

6)

-/~,./~,./~,./~,,

3'

 

5'

 

 

 

 

 

 

 

.......,,,/

\,if/

.......

,/

,,,,/

 

Ill

5'

 

 

 

 

 

3'

 

 

 

'/

 

 

 

 

 

 

TATA

 

 

 

 

 

IV

5'

em=-----

1

-----

.,~,,

3'

 

 

 

L ,___,./

 

'/

 

 

 

 

TATA

 

 

AATAAA

 

 

 

,~,

 

--------

i

 

v

5'

l .--

I

 

--II

3'

 

 

.......,,,/

.............................

 

f

 

 

AATAAA

Pitc. 4-53. l.lacTo ecTpe'la10~1tecsi eapMaHTbl cnnaiiicitHra nepBlt'IHblXTpaHcKpMnToe PHK. I. Bb1peaaHHe OAHOro H3 3K30Hos: a) CHHTea 6enKa, coi:iep>Ka1.11ero nonHblili Ha6op 3K30HOB (1-5); 6) CHHT63 6enKa, nHweHHOro OAHOro 3K30Ha (1, 2, 4, 5); II. CoxpaH6HHe y4acrKa HHTpoHa: a) c 5'-KOHUa;6) c 3'-KOHUa.Ill. CoxpaHeHllle uenoro HHrpoHa. IV. VlcnonbaosaHHe anbrepHarnBHblX npoMoropos (n11160 nepei:i 3K30HOM 1, nL-160 nepei:i 3K30HOM 2). V. V1cnonb30BaHH6 anbrepHaTlllBHblX y4aCTKOB nonH8/:16HHnHposaHHSI (HanpHMep, npH nocnei:ioearenbHOM CWHBaHHH 3K30HOB nocne 3K30Ha 3, a ecnH 3K30H 3 He npo4HTb1saercS1, ro nocne 3K30Ha 4).

pe3yJihTaTe aJibTepHanrnHoro crrnartc1rnra 06pa3yeTcH MPHK, B KOToport coxpaHHeTcH HHTPOH, conep)[<alll,HH nepBbIH CTOil-KOJJ:OH. Il03TOMY npoHCXOJJ:HT 6oJiee paHHee IlOJIHa)J;eHHJIHpOBaHHe H HCIJe3aeT 3K30H, KOlUIPYJOW.HH nmpoq>06HbIH yqacToK MoJieKyJihI. CHHTe311py10TcH yKopoIIeHHhie MOJieKYJibl aHTHTeJI, ceKpeTHpyeMhie B KPOBb.

«Pe;::uucruposauue» PHK. On11caH PHJJ: CJiyqaeB, Korna nepBMIJHaH cTpyKTypa MPHK H3MeHHeTCH ( «peJJ:aKTHpyeTCH») IlOCJie TpaHCKpHilll,HH. nOCJieJJ:OBaTeJihHOCTb HyKJieOTHJJ:OB B TaKHX reHaX OJJ:H- HaKOBa, a TpaHcKpH6HpyeMaH B pa3Hb!X TKaHHX MPHK pa3JIHIJaeTcH B pe3yJihTaTe noHBJieHHH B MOJieKYJie 3aMeH, BCTaBOK HJIH Bblila)J;eHHH HyKJieOTHJJ:OB. Ilp11Mep «peJJ:aKTHpoBamu1» PHK - 06-

pa30BaH11e anonpoTeHHa B (ano-B) B KJieTKax nelleHH M TOHKOfO KHWel!HMKa (p11c. 4-55). Ano-B - OCHOBHOH KOMilOHeHT JIHilOilpOTeMHOB, yqacTByJOIll,HX B TpaHcnopTe TpHaUHJifJIHll,epHHOB M3 3THX TKattetl: B KpOBh. Xorn anonpoTeMH B KOJJ:HpyeTcH OJJ:HHM H TeM )[(e reHOM, BapHaHT 6eJIKa, o6pa3yJOIll,HHCH B neIJeHH, Ha3bIBaIOT ano- B-100, H OH conep)[(HT 4563 aMHHOKHCJIOTHblX OCTaTKa, TO[JJ:a KaK 6eJIOK, CHHTe3HpOBaHHblH B KJieTKax KMWel!HHKa, COCTOHT 113 2152 aMMHOKMCJIOT. B reHe, KOJJ:HPYIOmeM 3TOT 6eJIOK, IlOCJieJJ:OBaTeJihHOCTb HyKJieOTHJJ:OB B TPHnJieTe 2153 - CAA H wmppyeT BKJIIOIJeHMe B IlOJIHileilTHJJ:HyJO uenb OCTaTKa rnyTaMHHa.

B KJieTKax KMrnellHHKa B nepBMl!HOM TpaHcKpHn-

Te reHa a30T11crne octtoBaHHe - UMT03MH ( C) KO-

JJ:OHa 2153 )J;e3aMHHMpyeTCH H npeBpamaeTCH B ypa11,HJI (U). Bo3HHKaeT cTon-KOJJ:OH - UM, npe-

Kpaw,aIOw,11i1 TpaHCJIHUMIO MPH K B cepeJJ:HHe MoJieKYJihI H npHBOMill,HH K CHHTe3y YKOPOIJeHHOfO 6eJIKa. B pe3yJihTaTe 06pa3yeTcH 6eJioK (B-48), MHHa KOToporo COCTaBJIHeT 48% OT MHHbl 6eJIKa CMHTe3HpyeMOfO neIJeHhIO.

lIJMeueuue CTa6HJibHOCTH MPHK. ,lJ,.Jrn Toro, l!T06bI yqacTBoBaTh B cHHTe3e 6eJIKa, MPHK JJ:OJDKHa BbIHTM H3 HJJ:pa B UHTOilJia3MY l!epe3 H)J;epHbie nopbI. YcrnHoBJieHo, 'ITOB Hnpe KJieToK o6bIIJHO CHHTe311pyeTcH 6oJibWMH Ha6op reTeporeHHhIX PHK, t.IeM TOT, 'ITO BbIXOJJ:HT B UHTOilJia3My. MHOrHe npoJJ:YKTbl TpaHCKPMilll,HM IlOJJ:BepraIOTCH pacmellJieHMIO HyKJiea3aMH, a Te MPHK, 'ITO,TpaHcnoPTHPYlOTCH 113 HJJ:pa B 11,11Torrna3MY, 3am11ma10TcH oT rHJJ:POJIHTHIJeCKOfO pa3pyrneHM51, o6pa3yH KOMil-

JieKCbl c 6eJIKaMH.

BpeMH )[(H3HM 3yKapMOTHIJecKMx MPHK 3HaIJHTeJibHO 60JibIIIe (tl/2 COCTaBJIHeT OT HeCKOJibKHX qacoB no HecKOJibKHX JJ:Hert), IJeM t 112 MPHK npoKapMOTOB, paBHoe HeCKOJihKMM MMHYTaM. OIIeBHJJ:-

HO, IJTO cTa6HJihHOCTh MOJieKYJI MPHK - cpaKTop,

H3MeHeHMe KOTOporo BJIHHeT Ha ypoBeHb TpaHCJIHUHH. Crn6MJI113a11,11H MPHK npH cpHKcttpoBaHHOtl: CKOPOCTH TPaHCKPHilll,HH npHBOJJ:HT K HaKOilJieHHIO 11 YBeJIHIJeHMIO KOJIHt.IecTBa o6pa3yJOmerocH 6eJIKoBoro nponyKTa.

npo)J;OJI)[(HTeJibHOCTb )[(H3HH pa3HbIX MPHK BapbHpyeT B nocrnTOl!HO urnpoKMx npeneJiax. HeKoTOphie reHbl KOJJ:HPYIOT npOJJ:YKT c 60JibllIOH npoJJ:OJI)[(HTeJihHOCThJO )[(H3HH. TaK, B xone TpaHcKp11n11,1111 retta P-rno611Ha 06pa3yeTcH MPHK c t paBHOM npHMepHo I 0 IJ. )],pyrne reHhI o6pa3yJOT MPHK c KopoTKOM npoJJ:OJI)[(HTeJibHOCThJO )[(H3HH: MPHK, Ha KOTOphIX cHHTe3HpyJOTCH cpaKTOPhI po-

198

4-54.

 

 

 

 

Y'lacroKpacu..1enneHvm np11

 

YCJ8CTOK paci.J..lenneH1t1H npvi

 

 

 

GV!H re3e KOpOTKOro TpaHCKpvmni

 

CV!HTe3e .Qm1HHOro TpaHCKpV!ma

 

5'y•-mcrnK cn11ai1c1-1Hrc:

 

3'yyacroK cnnai1c0Hra

;

 

 

 

 

 

..•':~?~i~2~~~~~:.~t~::~,H~-.i"

~-~-==:.---3K30,~ -~;:.~t==--~-

5'

 

 

 

 

 

CTOn ·KO/~OH 1

 

i

CTOfJ-.KOAOH 2

 

 

 

 

 

 

 

 

 

I

 

 

 

 

 

 

 

 

 

 

 

 

lpaHCKpMn1~'1R

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

"'-"'··----~- ---~......,

 

 

 

 

 

 

 

 

 

 

 

 

 

 

....

 

 

 

AnviHHblM PHK rpaHc1<pV1rn

 

 

 

 

 

KopOTK'10PHK rpaHcKp0m

 

 

 

 

 

 

 

 

 

 

 

Crnn-KOAOH 1

Crnn-KOAOH 2

 

 

 

 

 

 

 

 

 

5'-yYaCTOKcnnai1CV1Hf8

i'

3'-Y'l§!CTOK

I

 

 

 

5'-y4acTOKcnnaiklllHra

Crnn-KOAOH 1

 

 

cnna'1e>1Hra

1

 

 

 

 

..I

_/_·./?7-L2I==t==-tl:·'z'F·x::Ifi:==--

 

 

 

 

 

 

 

 

i 3'

1illHrpoHcnnai1rnHrey.aan;:ierc;:i np!A

 

- llom1-A

 

 

 

 

=.£}- llon1-1-A

 

3'

 

 

 

 

 

 

 

 

 

 

 

 

MPHK

nocnegoaarenbHOCTb

MPHK

 

 

 

 

 

 

 

 

MHrpoHa He y.Qamiercs:i

l

 

Crnn-Ko,noH 2

 

 

 

 

 

 

..

 

Crnn-KOAOH 1

 

 

 

 

 

non1.1-A.

 

 

 

 

 

(~(2:.~~7- ----~-j-J-nonw-A

 

 

 

 

 

 

 

 

 

 

 

1Tpm~cnm.\l.1H

3'

 

 

 

 

 

 

 

 

 

 

 

 

MeM6paHHo-csR38HHoe aHnneno

(fgM)

 

 

 

 

 

CeKpernpyeMble aHrnTena (lgM)

-2~(~7,ZZ'.Z--cooH

 

 

 

 

 

Z?ZZZ;2I:·.:_---:-.·-_--_-=_-_----.·__-_}-cooH

 

 

 

 

 

 

 

 

 

 

 

 

L __ __..___J

r1-1Apo¢0nbHb111t C-KoHel\

l>1.apocjJ061-1b1i-'1C-K0He1..1

P111c. 111cnonb30BaM111e Mexafm3MOB anbrepHanrnMoro cnnaliic1rnra 111 non111aAeH111n111posaH111S1 B xoAe ClllHTe3a MeM6paHHOCBsi3aHHblX 111 ceKpe1opHblX lg. Ecm1 rpaHcKplilm no11BepraercH nonV1aAeHV1nvipoBaHV110 nocne sroporo cron-KO/.IOHa, npV1cyrcrny10wero B 3K30H8 reHa lgM, 10 CV1HT831-1PYIOTC5l 6enK[ll, y KOTOi)blX Ha C-KOHL\8 nplilcyrcrnyeT rn11pocpo6Hbliil AOM8H, 06ecneLJ'1Ba10lJ.lV!i.iCB5l3blBaHMe c nna3MaHl'-18CKOi.iM8M6paHoiil. flpVI CTV!Myns:i1,1t1V1 B-n1>1McpOLjVITOB B Kn8TK3X ocywecrnm1eTC5l anbTepHaH1BHbli.i cnnafic1t1Hr nepBVILJHoro rpaHcKpV1ma, npvi KornpoM lilHTpoH, co1-1ep>(all.(V1iil nepr;b1V1 cron-KO/.IOH, coxpaH5l8TC5l. 06pa3y10rcs:i 6onee KOPOTKV!e MPHK, nom1a11eH1~nV!pOBaH1,1e KOTOpl.IX npOV!CXOAV!T nocne nep130rQ CTO<l-KO/.IOHa.

r-eH anonporn11Ha B--- ------------ CAJJ..-----------------

1

fpaHCKpMfll\\llR

l

 

 

- ----------!C :AA---

-------- - ------ TpaHcKp11nr

 

 

 

 

',,

PegaKrnposaH11e PHK

 

 

 

 

'~ KJleTKax KMW8L\Hl1Ka

MPH!{

-

- CAP.

 

---

:-LJ-!AA ----- MPHK

 

 

I

 

Crori=l<oAOH

neYeHb

 

TpaHc:n>1L11t1>i

I

Ano-B

 

l

 

 

.. Ano-B KfleroK Kl-1LUe'lHl-1Ka

 

 

 

 

 

~-----4563 ------1

~- 2152--1

Korn~Yecrso aMMHOK\llcno• B 6enKOBOM npop,yKTe

P111c. 4-55. «PeAaKn1posaH111e» MPHK anonp0Te111Ha B. B xo11e rpaHcKplilnqVIV! reHa anonpornV!Ha B B neYeHlil 06pa3yercs:i MPHK, cny)l(alJ.la5l MaTplilqei.i An5l CV!HT83a 6enKa, COCT05llJ.l8r0 V3 4563 aMVlflOKVICnOTHblX OCTaTKOB. B Kn8TKax TOHKOro KV!W8'-IHV!Ka3KCnpecClil5l rnro )1(8 reHa Bbl3b\Bae1 o6pa30BaHV1e 6enKa, cocrn5llJ.l3ro lil3 2152 aMV!HOKV!cnor. B PHK rpaHcKp1.-rnre q1.1T031.1H KOAOHa 2153 - CAA npespawaerrn B ypat.11.1n (U), H B03Hl.1Kaer CTOn-KO/.IOH B cepe1-11.1He MoneKynbl MPHK. 3TO npV!BDAVIT K CV!HT83Y yKop0'-18HHOrD6enKa.

199

MeT-TPHKM••

reMKlllH838 (HeaKT.)

 

MPHK

{

}

l

Cy6beA11tH11tl..lbl l!teM]

 

[teM]

p"160COM

 

 

 

reMK"1H838 (aKT.)

 

j@?"\'®®

 

aKT. AT<l>

 

A,Q<l> HeaKT.

 

lt1H"11..11t81..1"1s:! TP8HCJ1s:ll..1"1"1

1

C11tHre3 6enKa

P14CYHOK 4-56. 3aBl4Cl4MOCTb CKOpOCTl4 Cl4HTe3a rno614H8 OT KOHl..leHTpal.11414 reMa. Kori:ia sHyrpviKneT04Hbl"1 yposeHb reMa BblCOK, cjJaKTOP VIHllll..IVl81..1VIVI elF2 He cjJoccpopvinviposaH VI 8KTlllB6H, npovicxoi:ivir c111Hrea rno611tHa. Ecnvi coi:ieplKaHvie reMa e KneTKe CHVllK86TCs:I, cjJaKTop VIHVll..IVl81..1VIVI cpoccpop11tn1<1pyercs:1, VIH8KTlllBV1pyerci:1 111 ClltHTe3 6enKa npeKpaL1.1aerci:1.

cm, 11MeJOT t 112 Mettee I 'LI1oKa3aHo, 'ITOno;rn(A)- cpparMeHT Ha 3' -KoHue MPHK yBe1111'111BaeTrrponom1<11TeJihHOCTh )1(113Hl1 MOJieKYJI. qeM MMHHee ITOJI11(A)-cpparMeHT' TeM 60Jibllle BpeMH )!(113Hl1 MPHK.

Om1catto Mttoro np11MepoB peryAAUinf KOJI11LieCTBa CHHTe311PYJOll111XC51 6eJIKOB 3a CLieT 113MeHeHl151 npo.IIOJI)!(l1TeJihHOCTl1 cpyHKUl10H11pOBaHH51 MPHK. TaK, crn611J1hHOCTh MPHK-MaTp11u MH Cl1HTe3a MOJieKyJI rl1CTOHOB CHJihHO 3aBl1CHT OT cpa3hI KJieTOLIHoro u11KJia. B S-cpa3e mcTOHhI noCTOHHHO Cl1HTe3HpyJOTC51 11 11CI10Jlb3YJOTC51 MH YK-

Jia,UKl1 BHOBb o6pa30BaHHOH ,UHK B HYKJieOCOMhI. f11cTOHOBaH MPHK B 3TOT neptton crn611JihHa B TeLieH11e HeCKOJihKl1x qacos. IlocJie S-nep11ona, Korn.a ,UHK y)!(e He Cl1HTe3ttpyeTC51, B KJieTKax 06pa3yeTC5! He60JihUJOe KOJil1LieCTBO rl1CTOHOB, TaK KaK OHH He Tpe6yJOTC51 nAA cpopM11poBaHl15I HYKJieOCOM. B 3TOT nep11on t, 12 MH rHCTOHOBoti MPHK COCTaBJIHeT 10- J5 MHH.

6. Pezy.!lRu,ua mpanC/l.Ru,uu u nocmmpanC/l.Ru,uon-

nbtX MOdU<}JUKQU,UU

II3MCHCHHe cKopocTu TpaHCJIHl.UfH

Xorn 113MeHett11e cKopocTtt o6pa3oBaHAA 6eJIKOB Ha ypOBHe TpaHCJIHU1111 He OTHOCITT K LIMCJIY OCHOBHhlX cnoco6oB peryJ1Hu1111 KOJIM'lecTBaH pa3Hoo6pa3HH 6eJIKOB, HeKOTOpbie CJIYLiaH TaKOH peryJIH- U,1111 113BeCTHhI. Ha11:60Jiee H3y'!eHHhIHnp11Mep - Cl1HTe3 6eJIKOB B peTl1KYJIOUl1Tax. lfaBeCTHO, 'ITO Ha 3TOM ypOBHe .Ill1<P<PepeHUHPOBKH KPOBeTBOpHbie KJieTKM Jil1UJeHbl HD.pa, a CJI.enosaTeJibHO, If ,UHK.

PeryAAU1151 CHHTe3a 6eJIKa-rno611Ha OCYllleCTBJrneTCH TOJlbKO Ha ypOBHe TpaHCfillUl111 11 3aBl1C11T OT conep)l(aHHH reMa B KJieTKe (p11c. 4-56). EcJ111 BHYTPHKJieTO'IHaH KOHUeHTpaU1151 reMa Bh!COKa, TO rno6HH CHHTe3HpyeTcH; Korna conep)!(aH11e reMa CHlf)!(aeTCH, TO HHm611pyeTC5! 11 o6pa30BaH11e rno611Ha. OcrnHOBKa Cl1HTe3a 6eJIKa ocymecTBJIHeTCH 3a cqfa cpoccpopHJIMpOBaHHH cpaKTOpa l1Hl1UHau1111 elF2, KOTOpbIH B <Pocct>op11J111poBaHHOH cpopMe HeaKTHBeH. feM npe110TBpa1UaeT ct>occt>op11JI11:posaH11e elF2, CB513hIBaHCh co cneu11ct>11LieCKOH npoTel1HKl1Ha30H, KOTOpaH IlOJIYL!l1Jia Ha3BaHl1e reMKl1Ha3hl.

HeKoTOphie MPHK conep)!(aT 311eMeHThI srnp11LIHOH CTPYKTYPhI Ha 5'- HJI11 3'-KoHuax Herpattc1111pyeMoro yqacTKa MPHK, K KOTOphIM Moryr np11coe1111HHThCH 6eJIKH 11 l1Hm611poBaTb TpaHCJIHUl1!0. Hanp11Mep, CHHTe3 ct>eppHT11Ha - 6e11Ka, o6ecne- 'IHBaJOmeroxpatteirne 110HOB )l(eJie3a B KJieTKe, yc111111BaeTcH npH flOBhIUJeHHH BttyTp11KJieTOLIHOH KOHueHTpaumi: )l(e11e3a (cM. pa3neJI 14). 06Hapy)l(eHo, 'ITOMPHK cpeppHTHHa Ha 5'-Kom1e 11MeeT nern11, K KOTOpbIM npH HH3KOH KOHUeHTpaU,1111 )!(eJie3a npHcoen11HHeTC5! pery11HTOpHb1ti 6eJIOK. Korn.a 3TOT 6e-

110K CBH3aH c MPHK, To rpattcJIHUl1H He 11neT. Ec1111 KOHUeHTpaUl151 110HOB )!(eJie3a B KJieTKe ITOBb!WaeTCH, TO Fe3+ B3aHMOJJ.eHCTByeT c 6e11KoM, 113MeHHeT ero KOHcpopMau11JO 11 cponcrno K MPHK. MPHK OCB060)l()1,aeTC51 OT peryfillTOpHoro 6e11Ka, 11 Ha Hei1 HalfHHaeTCH CHHTe3 cpepp11THHa.

Pa3JIU11HH B nponoJDKHTeJihHOCTH *113HH MOJie-

KYJI 0CJ1Ka

I1oc11e Toro KaK 6eJIKH Cl1HTe3HpOBaHhl, BpeMH Hx )1(113Hl1 perymi:pyeTCH npoTea3aMH. Pa3Hhie 6e11KH 11MeIOT pa3Hbie tl/2: OT HeCKOJlbKl1X LiaCOB no HeCKOJlbKHX MeCHUeB, a irnorna l1 JieT (Ta6JI. 4-6).

B Ka)i().l,OH KJieTKe cKopocTh pacmen11eHHH 6e11KOB BapbMpyeT B UJMPOKHX npene11ax. <l>epMeHThl, KaTaJ1113HPYJOIUHe peryJIHTOpHbie peaKLIJ1l1 MeTa60Jil1LieCKl1X nyreM., KaK npaBHJIO, nonBepraJOTCH 6hICTPOMY pacmeilJleHHIO, TT03TOMY CKOpOCTb o6HOBJieH1151 3Tl1X MOJieKYJI JJ.OCTaTOLIHO BbICOKa. <l>H3HOJ10fl1LieCKOe coCTOHHHe opraHH3Ma TaK)!(e BJil151eT Ha npOJI0Jl)!(J1TeJibHOCTb )l(l13HH 6e11KoB. KpoMe Toro, CYlllecrnyeT MOIUHaH CHCTeMa 3aIUHThl, 06ecneLI11BaJOIUaH 6h1crpoe pacme1IJ1eH11e ae<PeKTHhIX 6e11KoB.

HeKoTopbre 6e11KH pacme1IJ1HIOTCH 11113ocoMHhIM11 cl>epMeHTaM11. B rrp01.1,ecce ayrocpam11 conep- )l(J1Moe KJieTKH, BKJIIO'-!a5lopraHeJIJibl'OKpy)l(aeTCH MeM6paHOH, cmrnaeTCH c Jll130COMOH npyrott KJieTKll If I10.IJBepraeTC51 nei1CTBHIO Jll130COMHbIX cpep-

200

B 90%
Tpaucno1oub1 -

:vieHTOB. B pe3yJibTaTe rH11,pomna o6pa3yIOwttec>1

MOHOMepbl IIOCTYilaIOT B Ul1TOTIJia3MY )J,JISl IIOBTOp-

HOI'O HCTIOJib30B3HH51.

Ta6nM~a 4-6. nep140A nonypacnaAa HeK0Topb1x 6enKoe e KneTKax MneKOnl4T8l011.114X

<l>epMeHT

T112, q

 

 

0,5

0pHHTHH.[(eKap6oKCHJia:m

T11po311naM11HOTPaHccpepa3a

2,0

Kap6oKCHKHHa3a

5,0

cpoccpoenonrr11pysaTa

 

Apnma3a

96

AnI:..n:ona3a

118

flaKTaT.n:er11.n:porena3a

144

U11wxpoM C

150

,U.1ur n:pyrnx 6e.JIKOB noKa3atto pacw,enJiem1e B UttTornrn3Me rrpoTea3aM11. TaK, noJJ,Jie)[{arn,tte pa3pymemno 6e.JIKH nepsottaqaJihHO OTMe4aIOTC>I KJleTKOH nYTeM rrp11coemrnemm 6eJIKa rroa Ha3Batt11eM

YOHKBHTHH. 3TOT tte60JibllIOM 6e.JIOK, COCT051lll,HM 113 76 aMl1HOKI1CJIOTHblX OCTaTKOB, o6ttapy)l(ett y MHOI'11X oprattl13MOB.

VIII. MEXAHl'l3Mbl rEHETl'l'-IECKOill l'l3MEH'-ll'IBOCTl'I. nonl'IMOP<t>l'l3M6EnKOB. HACnE.QCTBEHHblE 60nE3Hl'I

To4Ha51 pa6orn Bcex MaTpl14»hrx 611oc11ttTe30B - peTIJI11KaQHH, TpaHCKpl1IIQl1H 11 TpaHCJUIUHH - o6ecne411BaeT Kom1p0Batt11e rettoMa 11 Bocnpo113seaett11e ¢ettOTl1IIJ1lleCKl1X xapaKTep11CTHK oprattl13Ma B ITOKOJiettl1HX, T.e. HaCJie)J,CTBeHHOCTH. 01J.HaKO 6ttoJIOI'l14eCKaH3BOJIIOQl1H l1 ecTeCTBeHHbIM OT6op B03M0)1(HbI TOJibKO nptt HaJIM4MM reHernlfeCKOH M3MeH411BOCTH. YcTattoBJietto, 'HO rettoM rrocT051HHO npereprresaeT pa3Hoo6pa3ttb1e H3Mettemrn. HecMOTp5:1 Ha 3epepeKTHBHOCTb Mexatttt3MOB KOppeKQHH 11 penapau11H ,UHK, 43CTb IIOBpe)K,lleHMM 11JIH ourn60K B LI.HK OCTaeTC5l. l13MettettttH B nocJie)J,OBaTeJTbHOCTH nypl1HOBbIX HJTH n11p11Ml1).l,MHOBblX OCHOBaHHM B rette, He ttcrrpaBJieHHbre ¢epMeHTaM11 perrapauIDI., IIOJIY4HJIH Ha3Batttte «MYTIUJ,HH». 0Ll.H11 113 HMX OCTaJOTCH

B COMaTl14eCKHX KJleTKaX, B KOTOpblX OHM B03HHKJIM, a npyme o6ttapy.lKHBaIOTCH B IIOJIOBbIX KJieTKax, nepeJJ,aJOTC51 no HaCJTeJJ,CTBY H MOfYT np051BJI51TbC51 B ¢eHOTl1IIe IIOTOMCTBa KaK HaCJieJJ,cTBeHHa51 6oJie3Hb.

Cyw,ecrnetttthIM BKJiaJJ, B retter11qecKyIO H3MeH'm- socTb BHOCITT rrepecTpOMKH xpOMOCOM B npouecce Me:l103a. KaK yA<e YKaJhIBaJIOCh pattee, CJIH51Htte 5:1HQeKJieTKH co crrepMaT030HJJ,OM y 3yKapttOTOB corrpoBO)K,llaeTC51 reHeTH4eCKl1MH peKOM6ttHaUM5:1MM, B XO)le KOTOpbIX npoMCXO)lllT o6MeH yqacTKaMH .UHK Me)!()zy fOMOJIOfMqtthJMM xpOMOCOMaMM. 3To rrpMBOJIMT K rro- 5:1BJieHl1IO IlOTOMCTBa c HOBOM KOM6Httau11ei1 reHOB.

feH MJIH 'lacn1reHOB MOfYT rrepeMern,aTbC5:1 H3 0).1,HOro MeCTa xpoMOCOMbl B npyme. 3Tl1 IIO)J,Bl1)[{- Hhie 3.JieMeHTbl 11JIH Q:>parMeHThI .UHK TIOJIY'-ll1JIM Ha3BaHMe TpaHCI1030HOB l1 peTpOTpaHCIT030HOB.

yqacTKH ,UHK, YL1.aJI>1eMb1e 113 01JHOro JIOKyca xpoMOCOMbl 11 BCTpattBaeMbie B

npyroti JioKyc TOH .lKe MJIM npyro:H xpoMocoMbI. PeTpoTpaucno1ouh1 He rrOKH1IaJOT 11cxo1IHOro rro-

JIO.lKeHH5:1 B MOJieKyJie ,UHK, HO MOfYT KOIIMpoBaTbC5:1, 11 KOmrn BCTpmrnaIOTC5I, Il01I06Ho TpaHCII030HaM, B HOBblll y'-!aCTOK. 8KJIJOlfa5lCb B reHhl II.JIM yqacTKH OKOJIO reHOB, OHH MOryT Bbl3bIBaTb MYTaUMH H l13MeH5lTb 11X 3KCIIpecCHIO.

feHOM 3yKapHOTOB Il01JBepraeTC5:1 113MeHeHH5!M 11 rrp11 3apa.lKeH1111 ,UHK11JIH PHK-conep)[{aIUtt- MH BttpycaMH, KOTOphre BHe1Jp51IOT CBOH reHeT114eCKHH MaTepttaJI B .UHK KJleTOK X03HHHa.

A. MYTArEHE3

H3MeHeHl1H B reHoMe MOryT 6bITh pa3Hoo6pa3HhI H 3aTparttBaTb pa3JTJ11IHbie no rrpOTJI)[{eHHOCTl1 y'-!aCTKH,UHK OT xpoMOCOM l1 reHOB )lO OTlreJibHblX HYKJieOTH1IOB (Ta6JI. 4-7).

Hatt6oJiee 1IPaMant4Hhl rettOMHbie tt xpoMocoMtthre MYTaUHH, qacTo Ha6JTJO)JaeMhie tta ypostte coMaT114ecKHx KJieTOK. ECJIM OHM HMeJOT MeCTO B noJIOBbIX KJieTKax, TO 11,JIH opraHH3Ma 3TO HMeeT qaiue BCero JieTaJibHbie IIOCJie1JCTB1151. llacTOTa MYT3UMH B nOJIOBhIX KJleTKax BhICOKa. Cyw,ecrnyIOT ,JJ,attttbre, YKa3bIBaIOIUMe Ha TO, '!TOB 20% CJiyqaeB npH 6epeMeHHOCTH y 3M6pHOHOB Ha6JIIO)J,aIOT ttapywettl15:1 CTpYKTYPhI xpoMocoM. cnyqaes 3TO np11B01IHT K ttettopMaJihHOMY pa3BITTHJO TTJI01Ia tt 3.JIHMHttMpoBaHHJO 3apo,l1,bllliei1 B pe3yJibTaTe CIIOHTaHHbIX a6opTOB. BhlKilllhIWH, rrpo11cxo)J,Hlll,l1e B TelfeHtte nepBbIX

HeCKOJibKHX He)leJih 6epeMettttOCTII, CBH3aHbl c ce- P~3HbIMl1 HapyUieHH5lMH xpoMocoM. B 50% c.Jiyqa-

es OTMe4aeTC5! TpHCOMH5I no aYTOCOMaM, T.e. BMeCTO

201

Ta611MLU1 4-7. KnaccMcpMKaLIM~ MYT8LIMK

Tun

Xapainep MyTau;uonnhIX

IlpuMephI

MyTa1'UH

U3Meueuuu

DOCJie)J,CTBUH

 

 

 

feHOMHbIH

lhMeHeHIIe tfHCJia xpoMOCOM

bOJie3Hh ,IJ,ayHa (II05IBJieHHe

 

 

LIOIIOJIHHTeJihHOH xpOMOCOMbl 21)

XpoMocOMHbie

06rn,ee tfHCJIO xpOMOCOM RC Mem1eTCH.

Mb111Ie1rnai:i LIHCTPOQlHH ,Il,roIIIeHHa

 

Ha6rrroL1aIOT nepecTpOHKH xpoMOCOM, o6bitfHO

(L1eJ1e[\1111 X-xpoMocoMbl)

 

Bli,[\liMble rrpu MHKpOCKOIIHtfeCKOM 11ccrre.uoBaHHH.

 

 

 

 

feHHhie

IhMeHeHHH 3aTPar11BaIOT OLIHH KOLIOH mm

CeprroBHLIHO-KJieTOtfHai:i aHeMHH,

 

He6orrhIIIOH OTPe30K reHa 11 He o6Hapy)JrnBaIOTCH

Bbl3BaHHaH 3aMeHOH OLIHOro

 

[\HTOreHeTHtfeCKH

HyKJieOTHLla B reHe ~-Qerru rrro6uHa

 

 

 

rrapbl xpoMOCOM Ha6mo)laIOTCH T'pl1. IlpHMep TaKOH rraTOJIOrnH - 60JIC3Hb Ll,ayHa, rrpH KOTOpOH xpoMOCOMa 21 rrp11cyrcrnyeT B 3 3K3eMITJIHpax.

HeKOTOpbre reHHbie MyraIIHl1 JaKpeil.JiffiOTCH B rro- ITY.JUIUID:I, CTIDIOBHTCH ttacJie)lCTBeHHhIMH 11 orrpeJJ,e- JIHIOT 3BOJIIOIIHOHHblC rrpOIICCCbl. c MyraIIHHMH TaKOfO rnrra CBH3aHO IIOHRTICHHe pa3.11WIHblX HaCJIC)l- CTBeHHblX rraTOJIOfHH, corrpoBO)[(,ll,aIOIIIHXCH rrpeKpalll,CHHCM CHHTC3a 6eJIKa, KO)lHpyeMOfO IlOBpe)[(,ll,eHHbIM I'CHOM,JIH6o C11Hfe30M l13MeHeHHOfO 6eJIKa.

feHHhie, HJIH TO'IC'IHhie,MyraIIHH 6hrnaIOT B ocHOBHOM 3 BH)lOB:

3aMeHbl, rrpH KOTOpbIX O,ll,HO a30THCTOe OCHOBa-. HHe B LI.HK 3aMelll,aeTC51 Ha ,ll,pyroe;

BCTaBKll, 06ecrrel£11BaIOlll,He BHe)lpeHHe B MOJieKYJIY LI.HK O,IJ,Horo HJIH HeCKOJihKHx ,u:onoJIHHTeJihHhlX HyKJieOTH,ll,OB;

JJ;eJielUIH (HJIH BbIIla,IJ,CHHH) O,ll,HOro HJIH HeCKOJibKHX HyKJieOTH)lOB, nptt: KOTOpbIX rrpOHCXO,ll,HT

YKOPO'IeHu:eMOJieKYJihI Ll,H K.

1. Mymau.uu no muny 3aMenb1 B03Htt:KaJOT B pe3YJihTaTe 3aMeHbl O,ll,HOfO a30THCTOfO OCHOBaHHH Ha JJ,pyroe, 'ITOBbl3bIBaeT 113MeHeHHe B O,ll,HOM 113 KO,IJ,OHOB MyraHTHoro reHa. EcJIH KO)l11pyrom11tt TPHIIJICT, B KOTOpOM HaXO,ll,HTC51 l13MCHeHHblH HYKJICOTH,IJ,, l13-3a BblpO)[(,ll,CHHOCTH KO,IJ,a Bbl3bIBaeT BKJIIO'IeHHeB 6eJIOK Toti: .>Ke aMHHOKHCJIOThI, 'ITO HCXO)],HblM KOD:OH (HJIH KO,ll,OH «D:HKOrO» THIIa), TO TaKYIO MY'f~IIHIO Ha3bIBaIOT «MOJI'Iallleti:», l1 6eJIKOBblH npOJJ;YKT octafaCH TeM .>Ke.

Kor,IJ,a JaMeHa o,u:Horo ocHoBaHHH rrp11BOLIHT K 3aMeHe aMHHOKHCJIOTbl B MyraHTHOM 6eJIKe, TO Ta- KYIO Myrau1110 HaJhrnaroT «Mucceuc-MfTil~». BpH,IJ,e CJly'IaeB, HCCMOTp51 Ha rrpOH30lllCLl,lllYJO 3aMeHy' 6eJIOK COXpaH51CT 6HOJIOrH'ICCKYfOaKTHBHOCTb. mo,

KaK npaBHJIO, CB513aHO c TCM, 'ITOl13MCHeHHa51 aMHHOKHCJIOTa HaxO,IJ,HTCH B y'IaCTKC6eJIKa, He HMeIOmeM cPYHKIIHOHaJihHOro 3Ha'IeHHH, 11 K TOMY .>Ke OHa no CTPYKType l1 CBOHCTBaM HaITOMHHaeT HCXO,IJ,HYIO aMHHOKHCJIOTY. TaKaH MyrauHH TO.>Ke 6y- )leT «MOJI'Iamett»'a 3aMeHa - 3KBHBaJICHTHOH.

 

Tpm1JieT

HJMenennMii

 

(()J,HKOrO» THIIa

TpunJieT

MaTpHL(a ,IJ,HK

3'-TAA-5'

3''-GAA-5'

KoL10H MPHK

5'-AUU-3'

5'-CUU-3'

AMHHOKHCJIOTa

-Hrre-

-JieH:-

 

 

 

11HOf,IJ,a aMHHOKHCJIOta, 0Ka3aBlllCUICH 3aMCHeHHOH, pacrroJiaraeTcH B o6JiacTH, Ba.>KHoti: M51 npoHBJTeHH51 cPYHKIIHOHaJThHOH aKTHBHOCTH 6eJIKa, l1 ee JaMemeH11e rrp11Bo,IJ,11T K 06pm0Batt1110 Q:>yttKu110HaJihHO HeaKTHBHoro rrpo)lyKTa. TaK, TO'Ic'IHaHMYtaIIllil B KO,ll,OHe cepHHa (Cep - Ba.>KHeti:wHti: CT'pYKTYPHbIH KOMIIOHCHT aKTHBHOfO uettTpa cepHHOBblX rrp0Tea3: T'pHIICHHa, XHMOTPHIICHHa l1 HCKOTOpbIX ,IJ,pyrHX Q:>epMCHTOB) IIPHBO)lHT K IIOJIHOH IIOTepe aKTHBHOCTM. ECJIH rro)lo6ttbill <t>epMeHT YlfacrnyeT B peaKUH51X rnaBHblX Meta60JTH'ICCKl1Xrryreti:, TO TaKCUI «HC3KBHBaJICHTHaH» 3aMeHa MO)[(CT CTaTb JieTaJThHOH.

 

TpunJieT

H3MenennL1ii

 

«)J,HKOrO» Tuna

TpHnJieT

 

 

 

MaTPnQa ,IJ,HK

3'-GGT-5'

3'-GGA-5'

Ko,L1,0H MPHK

5'-CCA-3'

5'-CCU-3'

AMHHOKHcrrorn

-Ilpo-

-Ilpo-

 

 

TpunJieT

HJMeneunL1u

 

«JJ:UKOrO» Tuna

TpunJieT

 

 

 

 

MaTpHL\a ,IJ,HK

 

3'-AGA-5'

3'-AAA-5'

KOLIOH MPHK

 

5'-UCU-3'

5'-UUU-3'

AMHHOKHCJIOTa

 

-Cep-

-<l>eH-

202

B pHJle cnyqaeB MyraHTHhIH 6e110K, HeCMOTp51 Ha BXOMIIJYIO B Hero l13MeHeHHYJO aMHHOKMCJlO'fY,coxpaH51eT crroco6HOCTh BhlIIOJlH51Th CBOIO <l>YHKUHIO, HO MO)f(eT 6hITh He CTOJlh 3<P<l>eKTl1BHhIM, KaK 6e110K «LIHKOro>> Tttrra. B pe3yJlhTaTe Myrauww y ¢epMeHTa MO)f(eT OKa3aThC51 6011ee BhICOKHM 3Ha'leHweK m l1Jll1 6011ee Hl13KMM 3Ha'!eHl1eVmax' a HHOr)Ja TO l1 .11,pyroe O.JJ,HOBpeMeHHO. TaK11e 1.mcnI'lHO¢YHKUHOH11py1011U1e 6MKH Ha3hIBaIOT MyraHTHhlMH 6e11KaMH c HeIIOJlHOCThIO IIO.JJ,aRlleHHOH cPYHKUHeH.

lfape.JJ,Ka B pe3y11hTaTe Myrau11w 6e11KOBhIH rrpo-

.11,YKT reHa oKa3hrnaeTc51 11yqwe rrpwcrroco611eHHhIM K BhIIIOJlHeHHIO cBoeil cPYHKllttH. Tame Myrauww

.JJ,aIOT IIOTOMCTBY rrpewMylll,eCTBa B 6opb6e 3a cymecTBOBaH11e, a cepl151 COOTBeTCTBYJOIUHX MyraUIIM MO)f(eT rrpttBeCTM K llOHBJleHIIIO HOBOro BH.JJ,a.

Hau6011hWHM rroBpe)[()J,aIOIUHM ).l,ei1:crnIIeM 0611a).l,a10T MyTaUHH, llpl1BO).l,51IUl1e K o6pa30BaHHIO O)lHOro l13 TepMHHHPYIOIUHX KO)lOHOB (HOHCeHc- MYTaumI). B rrpouecce CMHTe3a 6e11Ka pa6orn ptt6ocoMhr 6y)JeT OCTaHOBJleHa Ha MyraHTHOM TµHllJleTe MPHK: UAA, UAG l1Jll1 UGA. Ilpo51BJleHtte HOHCeHC-MYTaUHH 3aBttCHT OT llX BHYTPIIreHHOM JlOKaJll13aUHl1. qeM 6Jll1)Ke Myrau115I K 5'-KOHUY reHa, T.e. K Ha'!MyTpaHCKpl1llUl1l1, TeM KOpoqe ee 6eJlKOBhlM rrpO)J.yKT, a CJle)J.OBaTeJlhHO, TeM MeHhwe OH crroco6eH K ocymecrn11eHII10 6II011omqec-

Koti cPYHKUl1l1.

 

TpmmeT

lhMeHeHHbIH

 

«JJ,HKOro» THIIa

Tpn11JieT

MaTp:iu-1a ,ll,HK

3'-GTC-5'

3'-ATC-5'

KoLlOH MPHK

5'-CAG-3'

5'-UAG-3'

AMHHOKHcJioTa

-fJIH-

CTOil-KO):IOH

2. Mymau,uu no muny ncmanKu ll.llU iJe.!leu,uu uyK.11eomuiJon

Eo11ee MHoroqwc11eHHhI u orracHhI )1.Jl5I KJieTOK MYTauwn rro nmy BCTaBKH HJlH .ueneuttII (yrpaThI) HYKJieOTH)lOB.

Ecn11 Myrau1rn rrpttBO)J.IIT K BCTaBKe lIJIII )1.e11eu1m B reH 0)1.HOM ttyKJieOTl1)1.HOH rrapbl l1Jll1 yqaCTKa )lByxueno'le'lHOHMOJieKyJihl ,!lHK c 'll1CJ10M MOHOMepoB, He KpaTHhIM 3 , TO 3TO Bhl3hIBaeT 113MeHeHtte C'll1ThIBaHl151 BCex IIOCJie)J.yiolll,IIX KO)J.0- HOB, TaK KaK rrpottCXO)J.HT C)lBHr «paMKH C'll1ThIBaHl15l» )lHK l1 HapyweHIIe COOTBeTCTBl151 Me)[(.11,y KO)lOHaMH B )lHK l1 aMHHOKHCJIOTaMII B KOHe'l- HOM rrpo.uyKTe - 6e11Ke (pwc. 4-57).

KaK BII)J.HO H3 p11c. 4-57, Hapyruemrn: B npotITemm 11HcpOpMaUHl1 Ha'!ttHaIOTCHc yqaCTKa, B KOTOPOM npott30lUJia MYTalll15l, TaK KaK ttMeHHO B

3TOM MeCTe rrpOHCXO)J.HT C)J.BHr «paMKII C'll1TbIBaHl1R» HH<t>opMaunw. beJIKOBhIH rrponyKT 3a TO'l- KOM MYTaUIIII 6yneT 11MeTh cnyqai1Hy10 rroc11e.uoBaTeJihHOCTh aMttHOKMCJJOT. Myrnu11w co C)J,BHroM paMKH C'll1TbIBaHH51qacTO npl1BO)l51T K II051BJleHIIIO BHyrpeHttero TepM11Httpy10mero KO)J,OHa, BhI3bIBaIOIUero rrpe)K)JeBpeMeHHoe npeKpameHwe CHHTe3a rroJinrrenTII.UHOH uerru H o6pa3oBaHtte YKOPO'leHHoro npo)lyKTa, JIHWeHHOro 6ttOJIOrtt'!eCKOM aKTl1BHOCTl1 .

MyrauIIw co C)lBIIroM «paMKH C'lIIThIBaHHH»ttH-

)1.yu11pyioT l1Hrtt6HTOPhI MaTplI'!HhIX Cl1HTe30B - «ttHTepKaJJ5ITOpb1». Mx 6011hwIIe IIJIOCKIIe MOJleKYJihI, noxm1rne Ha o6hI'!HhieaJOTHCThre ocHoBaHHH HJIH rraphl OCHOBaHHH, BCTpa11Ba10TC5I Me)K)ly )lByM51 coce)lHHMH rrapaMH OCHOBaHHH, B pe3yJihTaTe B ,!lHK «KaK 6hI» IIOHBJIHeTCR Jll1WHee OCHOBaHHe.

B xone perrJittKaUttH rnKow II3MeHeHHoti uenII ,!lHK B )lO'lepHIOIOHHTh B pe3yJihTaTe ow116o'!ttorocrra-

pnBaHtt51 c «llHTepKaJJllPOBaHHOM» MOJleKyJIOM MO)f(eT BCTpOHThCH )lOJTOJIHIITeJibHhIH HyKJieOTll)l.

11HOr)1.a, xorn l1 Kpai1He pe,!1.KO, TepHeTCH l1Jll1 BKJIIO'laeTC51B ,!lHK OJIHr0)1.e30KCllttyKJieOTH)1., coCTOHIUHH l13 3 l1Jil1 KpaTHOro 3 'llICJiaHyKJJeOTll)lOB. TaKHe Myrau,MII Ha3hrna10T .ueJieUII51MH l1JIH BCTaBKaM11 6e3 C)lBttra «paMKl1 C'll1TbIBaHl151» )lHK.

B 06pa3y10w,eMC5I 6eJIKOBOM rrpOAYKTe B 3TOM yqaCTKe OKa)f(eTCH rrporrylUeHHOM lIJill, Hao6opoT' BKJIIO'leHHOM)lOIIOJIHHTeJlhHO 0).1,Ha lIJIH HeCKOJihKO aMl1HOKl1CJIOT, TOr)la KaK BC5l OCTaJJhHaH aMHHOKIICJIOTHaH TTOCJie)lOBaTeJihHOCTh 6y)leT COOTBeTCTBOBaTb 11cxo.JJ,Hoti MOJieKYne. TaKIIe Myrauu11, KaK rrpaBHJIO, He rrp11HOC5IT 60JihllIOro Bpena.

11tt<PopMaUH51 o paJHhIX rnrrax MyrauIIti u II3MetteHH51X B CTpyKType MyraHTHhIX 6eJIKOB 0606meHbI B Ta6JI. 4-8.

3. qacmoma Mymau,uii

C'lttTaeTcR, 'ITO cpenmrn qacTOTa B03HHKHOBeHH51 MyrauIIi1 B CTPYKTYPHhIX JIOKycax ( o6Jiacrnx JIOKaJ1l13aUl1l1 retta B XpOMOCOMe l1Jll1 B MOJieKyJie )lHK) 'leJIOBeKaKOJie6JieTC51 B rrpe)leJiax OT 1o-s .uo

10-6 Ha O)J.HY raMery Ja Ka)K)loe rroKoJiettIIe. OnttaKo 3Ta BeJIH'IHHaMO)KeT 3Ha'IIITeJibHOBapbttpOBaTh )1.Jl5I pa3HhTX reHOB (OT 10-4 )lJ151 reHOB c BbICOKOM CKOpOCTbIO MyTaUIIM )lO 10- 11 )lJ151 Haw6onee ycTOH'IHBhIX yqacTKOB reHoMa). CToJih cymecrneHHhie KOJie6aHH51 B 'laCTOTeB03Hl1KHOBeHH51 MYTall11tt o6ycJIOB-

203

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