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Page 1: Heng Savoeun/2009 1 · 2019. 1. 24. · @Chemical elements essential for life are recycled, but energy is not. famBl nigemtabUlIs Heng Savoeun/2009 9. ... ÙenAEpñkxagsItu Usus

Heng Savoeun/2009 1

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KImI KImICIv³ CIv³KImI KImICIv³ CIv³Heng Savoeun/2009 2

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Heng Savoeun/2009 3

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KImICIv³ IIKImICIv³emtabUlIs

Heng Savoeun/2009 4

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emtabUlIs• 1- esckþIefþIm :famBl nigemtabUlIs : • 2- KøIkUlIs• 3- KøIkUEsnemtabUlIsnigkMNk Kúøy kU s• 4- vdþsuIRTikGa suIt• 5- kardwknaMeGLicRtugnigpUsVrIlkmµGu k suItk mµ• 6- lIBItemtabUlIs• 6- lIBItemtabUlIs• 7- eGtaNulemtabUlIs• 8- GamINUGasuItemtabUlIs• 9- nuyekøGUTItemtabUlIs• 10- karrMlay nigkarRsUbsmasFatuGahar• 11- rsµIsMeyaK

Heng Savoeun/2009 5

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emtabUlIs• 1- esckþIefþIm :famBl nigemtabUlIs : • 2- KøIkUlIs• 3- KøIkUEsnemtabUlIsnigkMNk Kúøy kU s• 4- vdðsuIRTikGa suIt• 5- kardwknaMeGLicRtugnigpUsVrIlkmµGu k suItk mµ• 5- kardwknaMeGLicRtugnigpUsVrIlkmµGu k suItk mµ• 6- lIBItemtabUlIs• 7- emtaNulemtabUlIs• 8- GamINUGasuItemtabUlIs• 9- nuyekøGUTItemtabUlIs• 10- karrMlay nigkarRsUbsmasFatuGahar• 11- rsµIsMeyaK

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famBl nigemtabUlIsrUbseg¡bénemtabUlIs;cMNIGaharEdlcUleTARtUv:nRsUbyk nigbMElgeTACazamBl tamry;dMeNIrkarmYyEdlry;dMeNIrkarmYyEdleKehAza GsmaNkmµ.zamBlenaHRtUv:neRbIedIm|ICMrujRbtikmµepSg>Edlbeg,ItmUelKulCIv;sMrab´karlUtlasénekasika

Heng Savoeun/2009 7

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Principles of Energy

Conservation

@ As open systems, cells require outside

energy sources to perform cellular work

(e.g., chemical, transport, and mechanical).@ Energy flows into most ecosystems as

sunlight. --> Photosynthetic organisms trap

a portion of the light energy and transform it

into chemical bond energy of organic

Heng Savoeun/2009 8

into chemical bond energy of organic

molecules. O2 is released as a byproduct.--@ Cells use some of the chemical bond

energy in organic molecules to make ATP-@ the energy source for cellular work. -->

Energy leaves living organisms as it

dissipates as heat.

@ The products of respiration (CO2 and H2O) are the raw materials for

photosynthesis. Photosynthesis produces glucose and oxygen, the raw

materials for respiration.

@ Chemical elements essential for life are recycled, but energy is not.

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famBl nigemtabUlIs

Heng Savoeun/2009 9

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famBl nigemtabUlIs

Heng Savoeun/2009 10

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kareRbIfamBl

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BnøWCIvH(bioluminescence)rbs;stVGMBilGMEBk

vdþ BnøI CI vH

Heng Savoeun/2009 12

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karkRnþak;rbs;sac;duM

Heng Savoeun/2009 13

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kareRbobeFobrvagGñkbenÞrfamBl nigGñkbenÞreGLicRtUKImI

enAkñúges[KVITaMgBIrzamBlénlMhUrzamBlénlMhUr

eeGLiRtugRtUv:nykmkeRbIedIm|IeZVIkmµnþ

Heng Savoeun/2009 14

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ÙkabUGuIRdat lIBIt nigRbUetGuInRtUv:nbMEbkeTACasarZatubnSMedImrbs´va rYcbMElgeTACa acetyl-coenzyme A

Ù acetyl-CoA RtUvrgRbtikmµGuksuItkmµkñúgvdþGasuItsuIRTic kñúgeBl

lMnaMemtabUlIs(Metabolic Pathway)

suItkmµkñúgvdþGasuItsuIRTic kñúgeBlmanvtþmanGuksuIEsn

Ù kñúgeBlCamYyKñaenaH NAD+ nigFAD RtUvrgRbtikmµerdukmµ

Ù kUGg´suImTaMgGs´enHRtUvrgRbtikmµenAkñúgRcvak´dwknaMeGLicRtug nigpUsVrIlkmµGuksuItkmµ rYcbeg,It:nCaTwk nigATP

Heng Savoeun/2009 15

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emtabUlIs• 1- esckþIefþIm :famBl nigemtabUlIs : • 2- KøIkUlIs• 3- KøIkUEsnemtabUlIsnigkMNk Kúøy kU s• 4- vdþsuIRTikGa suIt• 5- kardwknaMeGLicRtugnigpUsVrIlkmµGu k suItk mµ• 5- kardwknaMeGLicRtugnigpUsVrIlkmµGu k suItk mµ• 6- lIBItemtabUlIs• 7- eGtaNulemtabUlIs• 8- GamINUGasuItemtabUlIs• 9- nuyekøGUTItemtabUlIs• 10- karrMlay nigkarRsUbsmasFatuGahar• 11- rsµIsMeyaKHeng Savoeun/2009 16

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KøúykUsHeng Savoeun/2009 17

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ÙKøIkUlIsKWCapøÚvmYyEdlKøúyKUsRtUvbMElgeTACa pyruvate edayqñgkat fructose 1, 6-bisphosphate

ÙcUlrYmedayRbtikmµEdlCMrujedayGg´suImcMnYn10Ùmansar;sMxanNaskñúgemtabUlIszamBl‘energy-metabolism)

Ù~gekasikaGWkarIyUt KWRsed[gKña nigekasikaRbUkarIyUtPaKeRcIn

KøIkUlIs (glycolysis)

Ù~gekasikaGWkarIyUt KWRsed[gKña nigekasikaRbUkarIyUtPaKeRcInÙGg´suIménKøIkUlIs~gGWkarIyUtzitenA~gsIutUsul‘cytosol’

ÙÙÙ ehAza Embden-Meyerhof-Parnas-Way pgEdr

Heng Savoeun/2009 18

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KøIkUlIs

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dMeNIrRbRBwtþénKøIkUlIsEckCa2dMNak;

ÙÙ- dMNak´TI1;‘Rbtikmµ 1-5’• KøúykUsRtUvrgRbtikmµP¢ab´CamYypUsVat bnÞab´mkRtUv:nbMElgeTACa2 triose

• RtUvkar2mUelKul ATP• RtUvkar2mUelKul ATP

ÙÙ- dMNak´TI2; ‘Rbtikmµ 6-10’• glyceraldehyde 3-phosphate TaMgBIrRtUvbþÚrRTgRTayeTACa pyruvate

• beg,It:n4mUelKul ATP

Heng Savoeun/2009 20

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CMhanénRbtikmµrbs;KøIkUlIs 1- hexokinase:

• CMhanTImYyKWCakarepÞrbg<úMpUsVatBI ATP eTAelIKøúykUs ehIybeg,It:nglucose 6-phosphate

Heng Savoeun/2009 21

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CMhanénRbtikmµrbs;KøIkUlIs

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2- glucose phosphate isomerase;• RbtikmµbþÚrBI glucose 6-phosphateeTACa fructose 6-phosphate

• enHKWCaRbtikmµGuIsUEmrkmµBI aldose eTACa ketose

CMhanénRbtikmµrbs;KøIkUlIs

Heng Savoeun/2009 23

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CMhanTI1;karcgP¢ab´su)bRsþaCMhanTI2; GasuIténGgsuIm‘ε-amino group énsMNl´

CMhanTI3; :s (carboxylate group énsMNl´ glutamate) dkykecjnUvRbUtugBIC2 edIm|Ibeg,It cis-enediolate intermediate

CMhanTI4; RbUtugRtUv:nbþÚrkEnøgeTAelI C1. RbUtugEdlRtUv:ndkykecjeday:s bþÚry¨agel]nCamYyRbUtugénZaturMlay.

CMhanTI5; rgVg:nbiTedIm|Ibeg,ItplitplEdlRtUvrMedaHecj edIm|Ipþl´CaGgsuImesrI

‘ε-amino group énsMNl´lysine)katalIskarebIkrgVg

Heng Savoeun/2009 24

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CMhanénRbtikmµrbs;KøIkUlIs3- phosphofructokinase ;Ù eRbI ATPCaelIkTIBIrÙ P¢ab´bg<úMpUsVateTAnwg fructose 6-phosphate

Ù beg,It:n fructose 1, 6-bisphosphate

ÕkatalIs nucleophilic

attack eday C1-OH group

rbs fructose 6-phosphate

eTAelI electrophilic γ-phosphorusatom rbs Mg2+- ATP-complex

Heng Savoeun/2009 25

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CMhanénRbtikmµrbs;KøIkUlIs

4- aldolase ;Ùpþac´ fructose 1, 6-

bisphosphate eGayeTACaeGayeTACatriose(glyceraldehyde3-

phosphate nig dihydro-

xyacetonephosphate)

Heng Savoeun/2009 26

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1’ karP¢ab´su)bRsþa2’ karbeg,It schiff base

rvagLys kñúgmCÄmNÐlskmµrbsGg´suImnigTMrg´ExSebIkrbs FBP

3’ karkat´ aldoledIm|Ibeg,It enamine

intermediateénGg´suImnigDHAPCamYynigkarbeJ©j GAPbeJ©j GAP

4’ tautomerizationnigRbUtugkmµedIm|Ibeg,ItTMrg´iminiuménschiff base

5’ hydrolysisén schiff

baseCamYynigkarbeJ©jDHAP

Heng Savoeun/2009 27

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CMhanénRbtikmµrbs;KøIkUlIs

5-triose phosphate isomerase;Ù glyceraldehyde 3-phosphate

nigdihydroxyacetonephosphate

Ca ketose nig aldose-isomere

Ù vaTaMgBIrenHGacdUrRTgRTayeTAmkÙ vaTaMgBIrenHGacdUrRTgRTayeTAmkÙ edaysar glyceraldehyde 3-

phosphateRtUv:neRbIR:sCanic©enAkñúgRbtikmµénKøIkUlIs k*eZVIeGay dihy-

droxyacetonephosphate køayCaglyceraldehyde 3-phosphate CarhUtEdr

Heng Savoeun/2009 28

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Ù hexose rgpUsVrIlkmµ GuIsUEmrkmµ pUsVrIlkmµmþg

karsegçbénCMhanTI1rbs;KøIkUlIsenAkñúgRbtikmµCaesrI

GuIsUEmrkmµ pUsVrIlkmµmþg eT[t nigbnÞab´mk katpþac´eTACa 2 triose phosphate

Ù 2 ATPRtUv:neRbI

Heng Savoeun/2009 29

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CMhanénRbtikmµrbs;KøIkUlIs

6- glyceraldehyde 3-phosphate dehydrogenase ;Ù RbRBwtþeTAtamry;RbtikmµGuksuItkmµ nigpUsVrIlkmµrbs glyceraldehyde

Ù RtUvkar NAD+ CaPñak´garGuksuItkmµÙ mankarsMeyaK acyl phosphate 1,3-bisphosphoglycerate zamBlx¬sÙ mankarsMeyaK acyl phosphate 1,3-bisphosphoglycerate zamBlx¬s

☻ KYrrMlwkza acyl phosphate KWCasmasZatuEdlmanbUtg´EsülbenÞrpUsVatx¬s‘high phosphoryl group-transfer potential’

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glyceraldehyde 3-phosphate dehydrogenase

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CMhanénRbtikmµrbs;KøIkUlIs

7- phosphoglycerate kinase ;Ù enHKWCakarplit ATP elIkdMbUg Ù beg,It:n 3-phosphoglycerate

nig Ù beg,It:n

nig ATP

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CMhanénRbtikmµrbs;KøIkUlIs

8- phosphoglycerate mutase ;Ù CMrujRbtikmµepÞrbg<MúsVat BITItaMg3eTATItaMg2Ù mutase katalIskarepÞrbg<MnaTI BITItaMgmYyeTA

TItaMgmYyepSgeT[tenAelImUelKulmYy

Heng Savoeun/2009 33

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9- enolase;Ù CMrujRbtikmµbeg,ItlTÆplJk´kNþalTITI2 EdlsMbUrzamBlÙ 2-phosphoglycerate RtUvedGuIRdatkmµeTACa

phosphoenolpyruvate

CMhanénRbtikmµrbs;KøIkUlIs

phosphoenolpyruvate

enolase, Mg2+

H2O

Heng Savoeun/2009 34

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CMhanénRbtikmµrbs;KøIkUlIs10- pyruvate kinase;Ù Rbtikmµpþl´zamBlx¬s p<tp<g´RKb´RKansMrab´bg¡MeGaymankarsMeyaK ATP

Ù CaRbtikmµminRtLb´

1’ nucleophilic attackénGatUmrbs ADP β-phosphoryl oxygen

enAelIGatUmpUsVatén PEPedIm|Ibeg,It:n ATPnig enolpyruvate2’ tautomerizationén enolpyruvateeTACa pyruvate

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ÙkñúgRbtikmµCaesrIenH GAP

rgRbtikmµpUsVrIlkmµ nigGuksuItkmµ

rUbsegçbénCMhanTI2rbs;KøIkUlIs

GuksuItkmµÙ RkumpUsVatTaMgBIr manzamBlesrIRKb´RKansMrab´epÞredIm|Ibeg,It ATP

Ù 2mUelKulén GAPRtUv:nbMElgeTACa pyruvate

sMrab´mUelKulKøúykUsnimYy>

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Rbtikmµrbs;KøIkUlIs

RbtikmµTaMgmUlrbs´KøIkUlIs

Glu + 2NAD+ + 2ADP + 2Pi 2pyruvate +

2NADH +

2ATP +

2H2O +

2H+

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malate cycle nig glycerophosphate cycle

Ù enAeRkamlk¡xNÐmanxül´ KWtulüPaBzamBllÁbMput .NADH/H+EdlekIteLIgedaysar glyceraldehydes 3-

phosphate dehydrogenase RtUvrgGuksuItkmµ~g respiratory chain

Ù ~gkrNIenH TamTarnUvkardwknaMén NADH/H+ BIsuItUsulcUleTA~gmItUkugRDI

Ù ~gkrNIenH TamTarnUvkardwknaMén BIsuItUsulcUleTA~gmItUkugRDI

Ù edaysar NADH/H+ minGacsayPayqøgkatPñas~grbsmItUkugRDI:n enaHman malate cycle k¾dUc glycerophosphate cycle

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α-glycerophosphate cycleÙenAEpñkxagsuItUsus‘intermembraneroom’ KW dihydroaceto-

nephosphate rgerdukmµeday NADH/H+eTACa α-glyceropho-

sphateeRkamGMeBI glycerophosphate Dehydrogenase (GPDH)

cytosol

Ù edaysarglycerophosphate

oxidase(GPOX)eZVIeGay cytosol

inner

membrane

oxidase(GPOX)eZVIeGayα-glycerophosphatekøayCadihydroxyacetonephosphate

ÙeRkamCMnYyén ubichinoneZVIeGay FADH2 én flavoprotein

dehydrogenasekøayCa FADvij

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malate cycleNADH/H+ énsuItUsuseZVIerdukmµoxaloacetate eTACa malateeRkamGMeBIén malate dehydro-

genase(MDHc)rbssuItUsul .kardwknaMmalate cUleTA~gmatrix room sMerceTA:ntamry; malate/ketoglutarate

carrier (M-K-carrier)EdlenAkEnøgenaH vargGuksuItkmµeTACaoxaloacetate eRkamGMeBIénoxaloacetate eRkamGMeBIénmalate dehydrogenase

(MDHm)rbsmItUkugRDIedaybeg,It:n NADH/H+ . oxaloacetate rgRbtikmµepÞrGamInkmµCamYy glutamateeTACa aspartate eRkam«TÆiBlén aspartate aminotransferase

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vasna pyruvate ?

ÙenAeRkamlk¡x&NÐKµanxül´kñúgsacduM ‘kNþal’ pyruvate RtUv:nbMElgeTAmkkøay lactate cMENkkñúg yeast vaRtUv:nbMElgeTACa CO2 nig ethanol‘sþaM’Heng Savoeun/2009 41

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el,IgGal;kul (alcoholic fermentation)

electron micrograph of yeast cells

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karbMElg pyruvate eTAeGtaNuleRkamlkçxNÐKµanxül;kñúg yeast

1- pyruvateRtUvrgRbtikmµdkkabUneRkamGMeBIénGgsuImpyruvate decarboxylasepyruvate decarboxylase

edIm|Ibeg,It acetaldehyde

2- erdukmµ acetaldehyde eTACaethanol eday NADH

Glucose → 2Ethanol + 2ATP + 2CO2

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elI,gLak;tatkñúgsac;duM(homo lactate fermentation)

smIkarsrub;

Glu + 2ADP + 2Pi → 2 Lactate

+ 2ATP

+ 2H+

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ÙeRkamlk¡x&NÐénkarxMRbwgGtibrma dUcCa karRbNaMrtxøIel}n enaHmankarbeg,It ATP tamlk¡x&NÐGt´xüleTACa lactate

ÙkarekIneLIgén lactate kñúgsac´duM minbNþaleGaysacduMQWeT eRBaHsacduMGacrkSakic©karrbsva:nkñúgvtþmanénkMhablactatex¬s´ ebIsintMélpH enAezr

Lak;tat nigkarQWsac;duM

GacrkSakic©karrbsva:nkñúgvtþmanénkMhablactatex¬s´ ebIsintMélpH enAezrÙ ebIsin lactate minRtUv:ndwknaMeTAezøImeT enaH pH k¾minzycuHEdr eRBaH

lactic acid CaGasuItexSay. Eteday lactate RtUv:ndwknaMezøIm dUcenHenAslEt H+enaHk¾eZVIeGaytMél pH cuH rYck¾bNþaleGaysacduMQW

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sac;stVEdlRtUvedj ehIysMlab;

manrs;Catiq¶aj; ?ÙBYkGñkRbm¨aj:ncMNaMdwgzaÙBYkGñkRbm¨aj:ncMNaMdwgza

sac´stVEdlRtUv:nedj ehIysMlab´ vamanrsCatiGasuIt

ÙviTüasaRsþeRkamlk¡x&NÐénkarxMRbwgGtibrma sYtminGacp<t'p<g'GuksuIEsnRKb'RKan'

dUcenH sac´duMRtUvEtpøas´bþÚreTArkkarbeg,It ATP tamry;KøIkUlIsGtxüleTACa lactate

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karRtYtBinitüÙ PavrsKWCaRbB&nÆcMhrÙ Gg´suImxøHmanlk¡N;RtYtBinitü allosteric‘allosteric

regulation’ EdlRtUv:nRtYtBinitütamry;eGpicT&r‘effector)

Ù eGpicT&rTaMgenHKWCasu)bRsþa plitpl rWk*CakUGgsuIm

enzyme inhibitor activator

Hexokinase G6P ATP

Phosphofructokinase ATP, citrate ADP, AMP, F6P

Pyruvate kinase ATP

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emtabUlIsGicsUs

pøÚvcUlrbsBYk hexose

eTAkñúgKøIkUlIs Ù fructose‘kñúgsac´duM’ nig mannoseRtUvbM nig mannoseRtUvbM ElgeTACa F6P

Ù fructose‘kñúgezøIm’ RtUvbMElgeTACa GAP

Ù galactose RtUvbMElgeTACa G6P

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emtabUlIsGicsUs

ÙenAmanGicsUs3eT[tEdlCalTÆplénkarkinrMlayEdrKW;- RPuctUs‘fructose); ecjmkBIGuIRdUlIssakarUs‘ sucrose)

- kaLak´tUs‘galactose); ecjmkBIGuIRdUlIss,rTwkedaHeKa- m¨aNUs‘mannose); ecjBIGuIRdUlIsbUlIsakarIt nigKøIkURbetGuIn- m¨aNUs‘mannose); ecjBIGuIRdUlIsbUlIsakarIt nigKøIkURbetGuIn

ÙbnÞab´BIGuIRdUlIsmkmUNUsakarItTaMgenH k*RCabcUleTAkñúgQam nigRtUv:nbJ¢ÚneTAdl´srIrag<epSg>

ÙvaRtUv:nbMElgkñúgdMeNIremtabUlIseTACaplitplJkkNþalTIénKøIkUlIs ehIyvak*RtUv:nrMlaybnþeTAeT[t dUcKøúykUsEdr

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emtabUlIsénRPuctUs (fructose)

Ù enA~gsrIrag<epSg>eT[teRkABIezøIm k¾ekItmannUvkarbMEbkRPuctUs EdrEtRbRBwtþeTAyWtxøaMgNas

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emtabUlIsénRPuctUs (fructose)

Ù RPuctUsGacRtUv:nsMeyaKecjBIKøúykUs eRkamGMeBIénGgsuImrbs polyol pathway

Ù CadMbUg Gg´suIm aldosereductase(polyoldehydrogenase) CMrujRbtikmµerdukmµEdlGaRs&ynwg NADPH / H+ BIKøúykUs eTACa sorbitol

Ù bnÞab´mk sorbitol rgGuksuItkmµenA~gRbtikmµmYyGaRs&y NAD+ EdlCMrujedayGgsuIm ketose reductase eTACaRPuctUs

CIv;sMeyaKRPuctUsecjBIKøúykUskñúgekasikaeRkAezøIm

aldose reductase ketose

reductase

GgsuIm ketose reductase eTACaRPuctUs

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diabetic cataract ?

Ù enAkñúgzg´BUC polyol pathway RbRBwteTAy¨agskmµ

Ù polyol pathway k¾manvtþmanenA~gCalikaCaeRcInEdlminGaRs&y insulin .ehtuenH kMhab´RPuctUsekIneLIgedaysar polyol pathway cMeBaHkMhab´KøúykUsx¬s~gQamx¬s~gQam

ÙRPuctUsminGaccakecjBICalikaEkvEPñk:neT enaHkMhab´RPuctUsekIneLIgrhUtdl´kMhab´mYyEdlman«TÆiBlGUsµÚs

Ù karenHnaMeTArkkMeNInénkarhUrcUlrbsTwkeTA~gCalikaEkvEPñkEdleZVIeGayrIke:¨g nigxUclk¡N;GubTik EdlcugbJ©b´naMeTAdl´ diabetic cataract

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fg;BUC‘seminal vesicle’

Ù enA~gzg´BUC‘seminal vesicle’RbtikmµenHRbRBwtþeTAedayel|}nx¬s´nigplitnUvRPuctUs~gbrimaNd¾eRcInKYrsm . CIv;sMeyaKénGgsuImTaMgBIrrbs´ polyol pathway zitenAeRkamkarRtYtBinitüGgsuImTaMgBIrrbs´ polyol pathway zitenAeRkamkarRtYtBinitürbs´ testosterone

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emtabUlIsénkaLak;tUs (galactose)

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galactosemia

Ù galactosemia(kMritkaLak´tUskñúgQamekIneLIg’KWCakarrMxanénemtabUlIsgalactose BIkMeNIt

Ù kkþaenHGacbNþalmkBIBIrkrNI KW

1-Õ GgsuIm1-Õ karmindMeNIrkarénGgsuIm galactokinase

2-Õ karmindMeNIrkarénGgsuIm galactose 1-phosphate

uridylyltransferase

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galactosemia

Ù1- ebIskmµPaBgalactokinasemindMeNIrkar enaHnaMeGayekIneLIgénplitplbnÞab´Bul‘toxic metabolic by-product’

ó - karekIneLIgénkMhab´ galactosekñúgQam pþl´lTÆpleGaykMhab´galactose x¬skñúgEkvEPñkEdlenATIenaH galactose rgerdukmµeTACa galactitol

- vtþmanén sugar alcohol enAkúñgEkvEPñk bg,eGayEPñkeLIg:y cataract- vtþmanén sugar alcohol enAkúñgEkvEPñk bg,eGayEPñkeLIg:y cataract

(EkvEPñklÁk´ clouding of the lens)

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galactosemia

Ù2 ebI galactose 1-phosphate uridylyltransferase mindMeNIrkar ehIyskmµPaBrbs galactokinase enAdMeNIrkarZmµta enaHnwgbNþaleGaymankarekIneLIgénkMhab´ galactose 1-phosphate

Ù galactose 1-phosphate eTAbgÁak´skmµPaBGg´suIm;- phosphoglucomutase,

- glucose 6-phosphatase- glucose 6-phosphatase

- glucose 6-phosphate dehydrogenase

☻dUcenH vanaMmknUvkarrMxany¨agxøaMgdlemtabUlIsKøúykUs. GñkCYbRbTHnwgbJØaenH maneraKsJ¦a dUcCa ;

- ezøIm:t´bg´tYnaTI- karlUtlasyWténR:C\a- karcuHzykMhab´KøúykUs(hypoglycemia)

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emtabUlIsm:aNUs (mannose)

- CaplitplénkarrMlaypolysaccharidenig glycoprotein

- KWCa C2-epimer én glucose

1- hexokinase bMElgm¨aNUseGayeTACa mannose 6-phosphate1- hexokinase bMElgm¨aNUseGayeTACa mannose 6-phosphate

2- phosphomannose isomerasebMElg aldoseeTACa ketosedUcCafructose 6-phosphateCaedIm

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dMeNIrpUsVatb:g;tUs

ÙtYnaTIrbs´ pentose phosphate pathway rW hexosemonophosphate

pathwaymanBIrKW;- plit NADPH

- beg,It ribose 5-phosphate- beg,It ribose 5-phosphate

smIkarsrubKW;

3Glu 6-P + 6NADP+ + 3H2O → 6NADPH + 6H+ +

3CO2 + 2Fructose 6-P +

Glyceraldehyde 3-P

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dMeNIrpUsVatb:g;tUsÙ NADPH ;

- bMerICaerdukr kñúgkarRbqaMgnwgCatiBul H2O2 kñúgekasikaQamRkhmEdlbeg,ItedayRbtikmµminCMrujedayGgsuIm

- CaerdukrkñúgkarsMeyaKGasuItxøaj nig cholesterol- CaerdukrkñúgkarsMeyaKGasuItxøaj nig

Ùribose 5-phosphate; ‘’;

- eRbICaZatupSMd¾sMxan´sMrab´CIv;sMeyaK nucleotide

- RtUv:nbMElgeTACa fructose 6-phosphate nig 3-phospho

glycerinaldehyde

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dMeNIrpUsVatb:g;tUs

dMeNIrRbRBwtþeTAenH eKEckCaBIr;Ù EpñkGuksuItkmµÙ EpñkGuksuItkmµkarbeg,It:n NADPH nig ribulose 5-P ecjBI glucose 6-P

ÙEpñkKµanGuksuItkmµCakarbMElg ribulose 5-P eGayeTACa fructose 6-P nigglyceraldehyde 3-P

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dMeNIrpUsVatb:g;tUs

ÙdMeNIrRbRBwtþenHmanEpñkGuksuItkmµnigEpñkKµanGuksuItkmµ

ÙkñúgEpñkGuksuItkmµbeg,It:nNADPH nig ribulose 5-NADPH nig ribulose 5-

phosphate

ÙkñúgEpñkKµanGuksuItkmµplit:n fructose 6-phosphate nigglyceraldehyde 3-phosphate

EdlCaGnþrsmasZatuénKøIkUlIs

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EpñkGuksuItkmµ

3Glu 6-P + 6NADP+ + 3H2O → 6NADPH +

6H+ +

3CO2 +

3R5P

6-phosphogluconate

dehydrogenase

NADP+ NADPH + H+ CO2

3R5P

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EpñkKµanGuksuItkmµ(non oxidative stage)

ÙenA~gEpñkKµanGuksuItkmµéndMeNIrpUsVatb¨g´tUsmanGg´suImBIr transketolase nig trans-

aldolase EdledIrtYnaTIsMxan´Ùribulose 5-phosphate epimerase naMmknUvbMErbMrYlénRTgRTayenARtg´ én

Ribulose-5-P

Epimerase Isomerase

Xylulose-5-PRibose-5-P

Transketolase

Sedoheptu-

lose-7-P3-P-glycerin-

aldehyde

Ù naMmknUvbMErbMrYlénRTgRTayenARtg´ C-atom 3 énribulose Edl~gkrNIenH xylulose 3-phos-

phate manrUbrageLIgÙeRkam«TÆiBlénribulose 5-phosphate

isomerase beg,It:n ribose 5-P

Ùribose5-PbMerICaZatupSMd¾sMxan´sMrab´CIv;sMeyaK nucleoside

Transaldolase

Fructose-6-P

Xylulose-5-P

3-P-Glycerin-

aldehydeFructose-6-P

Transketolase

Erythro-4-P

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karRtYtBinitüdMeNIrpUsVatb:g;tUs

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karxVHGg;suIm glucose 6-phosphate dehydrogenase

Ù PñasrbsekasikaQamRkhmRtUvkar glutathion(GSH) CaeRcInedIm|IrkSavtþmanrbsvaÙ tYnaTId*sMxan´rbsglutathion enAkñúgekasikaQamRkhm KWkarrMlay

H2O2 nig GuIRdUEBGuksuItsrIrag<‘organic hydroperoxide)

Ù H2O2 BulxøaMgNas eRJHvaeZVIRbtikmµCamYysm¬&nÆBIrCan´én fatty acid-rest

rbsPñasekasikaRkhm nigbeg,ItCaGuIRdUEBGuksuItsrIrag< rYcvak*pþacsm¬&nÆGatUmkabUnrbsPñasekasikaRkhm nigbeg,ItCaGuIRdUEBGuksuItsrIrag< rYcvak*pþacsm¬&nÆGatUmkabUnnigGatUmkabUneGayeTACaGasuItxøaj´ . edaysarGMeBIenHEdleZVIeGayPñasRtUvxUc .

Ù EBGuksuIt‘peroxide)RtUv:nbM:t´eday glutathion-peroxidase

2GSH + R-O-O-H glutathione-peroxidase GSSG + ROH + H2O

bnÞab´mkmanRbtikmµerdukmµén GSSG eday NADPH:

GSSG + NADPH + H+ glutathione reductase 2GSH + NADP+

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karxVHGg;suIm glucose 6-phosphate dehydrogenase

Ù ebIGñkCMgWCYbRbTHnUvkarxVHénskmµPaBrbs glucose 6-phosphate

dehydrogenase(genetic defect) enaHCamUlehtubNþaleGayekItmanCMgWxVHQamRkhmy¨agxøaMg eBlEdleKRtUvkarBüa:lGñkCMgWedayzñaMRbqaMgnwgCMgWRKuncaj´ primaquin (antimalaria)

Ù CaGñkbeg,Inkarbeg,ItEBGuksuIt ehIytMrUvkareTAelIRKuncaj´

Ù primaquine CaGñkbeg,Inkarbeg,ItEBGuksuIt ehIytMrUvkareTAelI NADPH

k*ekIneLIgEdr . buEnþ ebIekasikaxUc enaHminGacsMeyaK NADPH eGay:neRcIneT

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karxVHGg;suIm glucose 6-phosphate dehydrogenase

ÙenAtMbn´manCMgWRkuncaj´‘malaria’ ekItmankarxUcGgsuIm glucose 6-

phosphate dehydrogenase CaeRcIn eRJHza ebIGgsuIm glucose 6-

phosphate dehydrogenase xUceZVIeGayeKZn´RTaMGactTlRbqaMgnwgBYkemeraKEdlbg,CMgWRKuncaj‘malaria parasite)eQµaH plasmodium :nemeraKEdlbg,CMgWRKuncaj‘malaria parasite)eQµaH plasmodium :nÙedayehtuza ekasikaQamRkhmEdlman glucose 6-phosphate

dehydrogenase tictYc eZVIeGay plasmodium Bi:krsenACagekasikaQamRkhmZmµta eRJH parasite RtUvkarcaM:c´nUvplitpléndMeNIrpUsVatb¨gtUs(NADPH) rWm¨üageT[tzaedayekasikaQamRkhm RtUvrlaymunnwg parasite manlTÆPaBrIklUtlas´

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karxVHGg;suIm glucose 6-phosphate dehydrogenase

glutathioneglutathione

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