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Heng Savoeun/2009 1
KImI KImICIv³ CIv³KImI KImICIv³ CIv³Heng Savoeun/2009 2
Heng Savoeun/2009 3
KImICIv³ IIKImICIv³emtabUlIs
Heng Savoeun/2009 4
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
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
Heng Savoeun/2009 6
famBl nigemtabUlIsrUbseg¡bénemtabUlIs;cMNIGaharEdlcUleTARtUv:nRsUbyk nigbMElgeTACazamBl tamry;dMeNIrkarmYyEdlry;dMeNIrkarmYyEdleKehAza GsmaNkmµ.zamBlenaHRtUv:neRbIedIm|ICMrujRbtikmµepSg>Edlbeg,ItmUelKulCIv;sMrab´karlUtlasénekasika
Heng Savoeun/2009 7
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.
famBl nigemtabUlIs
Heng Savoeun/2009 9
famBl nigemtabUlIs
Heng Savoeun/2009 10
kareRbIfamBl
Heng Savoeun/2009 11
BnøWCIvH(bioluminescence)rbs;stVGMBilGMEBk
vdþ BnøI CI vH
Heng Savoeun/2009 12
karkRnþak;rbs;sac;duM
Heng Savoeun/2009 13
kareRbobeFobrvagGñkbenÞrfamBl nigGñkbenÞreGLicRtUKImI
enAkñúges[KVITaMgBIrzamBlénlMhUrzamBlénlMhUr
eeGLiRtugRtUv:nykmkeRbIedIm|IeZVIkmµnþ
Heng Savoeun/2009 14
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
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
KøúykUsHeng Savoeun/2009 17
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
KøIkUlIs
Heng Savoeun/2009 19
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
CMhanénRbtikmµrbs;KøIkUlIs 1- hexokinase:
• CMhanTImYyKWCakarepÞrbg<úMpUsVatBI ATP eTAelIKøúykUs ehIybeg,It:nglucose 6-phosphate
Heng Savoeun/2009 21
CMhanénRbtikmµrbs;KøIkUlIs
Heng Savoeun/2009 22
2- glucose phosphate isomerase;• RbtikmµbþÚrBI glucose 6-phosphateeTACa fructose 6-phosphate
• enHKWCaRbtikmµGuIsUEmrkmµBI aldose eTACa ketose
CMhanénRbtikmµrbs;KøIkUlIs
Heng Savoeun/2009 23
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
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
CMhanénRbtikmµrbs;KøIkUlIs
4- aldolase ;Ùpþac´ fructose 1, 6-
bisphosphate eGayeTACaeGayeTACatriose(glyceraldehyde3-
phosphate nig dihydro-
xyacetonephosphate)
Heng Savoeun/2009 26
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
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
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
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’
Heng Savoeun/2009 30
glyceraldehyde 3-phosphate dehydrogenase
Heng Savoeun/2009 31
CMhanénRbtikmµrbs;KøIkUlIs
7- phosphoglycerate kinase ;Ù enHKWCakarplit ATP elIkdMbUg Ù beg,It:n 3-phosphoglycerate
nig Ù beg,It:n
nig ATP
Heng Savoeun/2009 32
CMhanénRbtikmµrbs;KøIkUlIs
8- phosphoglycerate mutase ;Ù CMrujRbtikmµepÞrbg<MúsVat BITItaMg3eTATItaMg2Ù mutase katalIskarepÞrbg<MnaTI BITItaMgmYyeTA
TItaMgmYyepSgeT[tenAelImUelKulmYy
Heng Savoeun/2009 33
9- enolase;Ù CMrujRbtikmµbeg,ItlTÆplJk´kNþalTITI2 EdlsMbUrzamBlÙ 2-phosphoglycerate RtUvedGuIRdatkmµeTACa
phosphoenolpyruvate
CMhanénRbtikmµrbs;KøIkUlIs
phosphoenolpyruvate
enolase, Mg2+
H2O
Heng Savoeun/2009 34
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
Heng Savoeun/2009 35
ÙkñúgRbtikmµCaesrIenH GAP
rgRbtikmµpUsVrIlkmµ nigGuksuItkmµ
rUbsegçbénCMhanTI2rbs;KøIkUlIs
GuksuItkmµÙ RkumpUsVatTaMgBIr manzamBlesrIRKb´RKansMrab´epÞredIm|Ibeg,It ATP
Ù 2mUelKulén GAPRtUv:nbMElgeTACa pyruvate
sMrab´mUelKulKøúykUsnimYy>
Heng Savoeun/2009 36
Rbtikmµrbs;KøIkUlIs
RbtikmµTaMgmUlrbs´KøIkUlIs
Glu + 2NAD+ + 2ADP + 2Pi 2pyruvate +
2NADH +
2ATP +
2H2O +
2H+
Heng Savoeun/2009 37
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
Heng Savoeun/2009 38
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
Heng Savoeun/2009 39
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
Heng Savoeun/2009 40
vasna pyruvate ?
ÙenAeRkamlk¡x&NÐKµanxül´kñúgsacduM ‘kNþal’ pyruvate RtUv:nbMElgeTAmkkøay lactate cMENkkñúg yeast vaRtUv:nbMElgeTACa CO2 nig ethanol‘sþaM’Heng Savoeun/2009 41
el,IgGal;kul (alcoholic fermentation)
electron micrograph of yeast cells
Heng Savoeun/2009 42
karbMElg pyruvate eTAeGtaNuleRkamlkçxNÐKµanxül;kñúg yeast
1- pyruvateRtUvrgRbtikmµdkkabUneRkamGMeBIénGgsuImpyruvate decarboxylasepyruvate decarboxylase
edIm|Ibeg,It acetaldehyde
2- erdukmµ acetaldehyde eTACaethanol eday NADH
Glucose → 2Ethanol + 2ATP + 2CO2
Heng Savoeun/2009 43
elI,gLak;tatkñúgsac;duM(homo lactate fermentation)
smIkarsrub;
Glu + 2ADP + 2Pi → 2 Lactate
+ 2ATP
+ 2H+
Heng Savoeun/2009 44
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
Heng Savoeun/2009 45
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
Heng Savoeun/2009 46
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
Heng Savoeun/2009 47
emtabUlIsGicsUs
pøÚvcUlrbsBYk hexose
eTAkñúgKøIkUlIs Ù fructose‘kñúgsac´duM’ nig mannoseRtUvbM nig mannoseRtUvbM ElgeTACa F6P
Ù fructose‘kñúgezøIm’ RtUvbMElgeTACa GAP
Ù galactose RtUvbMElgeTACa G6P
Heng Savoeun/2009 48
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
Heng Savoeun/2009 49
emtabUlIsénRPuctUs (fructose)
Ù enA~gsrIrag<epSg>eT[teRkABIezøIm k¾ekItmannUvkarbMEbkRPuctUs EdrEtRbRBwtþeTAyWtxøaMgNas
Heng Savoeun/2009 50
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
Heng Savoeun/2009 51
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
Heng Savoeun/2009 52
fg;BUC‘seminal vesicle’
Ù enA~gzg´BUC‘seminal vesicle’RbtikmµenHRbRBwtþeTAedayel|}nx¬s´nigplitnUvRPuctUs~gbrimaNd¾eRcInKYrsm . CIv;sMeyaKénGgsuImTaMgBIrrbs´ polyol pathway zitenAeRkamkarRtYtBinitüGgsuImTaMgBIrrbs´ polyol pathway zitenAeRkamkarRtYtBinitürbs´ testosterone
Heng Savoeun/2009 53
emtabUlIsénkaLak;tUs (galactose)
Heng Savoeun/2009 54
galactosemia
Ù galactosemia(kMritkaLak´tUskñúgQamekIneLIg’KWCakarrMxanénemtabUlIsgalactose BIkMeNIt
Ù kkþaenHGacbNþalmkBIBIrkrNI KW
1-Õ GgsuIm1-Õ karmindMeNIrkarénGgsuIm galactokinase
2-Õ karmindMeNIrkarénGgsuIm galactose 1-phosphate
uridylyltransferase
Heng Savoeun/2009 55
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)
Heng Savoeun/2009 56
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)
Heng Savoeun/2009 57
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
Heng Savoeun/2009 58
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
Heng Savoeun/2009 59
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
Heng Savoeun/2009 60
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
Heng Savoeun/2009 61
dMeNIrpUsVatb:g;tUs
ÙdMeNIrRbRBwtþenHmanEpñkGuksuItkmµnigEpñkKµanGuksuItkmµ
ÙkñúgEpñkGuksuItkmµbeg,It:nNADPH nig ribulose 5-NADPH nig ribulose 5-
phosphate
ÙkñúgEpñkKµanGuksuItkmµplit:n fructose 6-phosphate nigglyceraldehyde 3-phosphate
EdlCaGnþrsmasZatuénKøIkUlIs
Heng Savoeun/2009 62
EpñkGuksuItkmµ
3Glu 6-P + 6NADP+ + 3H2O → 6NADPH +
6H+ +
3CO2 +
3R5P
6-phosphogluconate
dehydrogenase
NADP+ NADPH + H+ CO2
3R5P
Heng Savoeun/2009 63
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
Heng Savoeun/2009 64
karRtYtBinitüdMeNIrpUsVatb:g;tUs
Heng Savoeun/2009 65
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+
Heng Savoeun/2009 66
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
Heng Savoeun/2009 67
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´
Heng Savoeun/2009 68
karxVHGg;suIm glucose 6-phosphate dehydrogenase
glutathioneglutathione
Heng Savoeun/2009 69
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