1、University of ManitobaAdam BurgenerUniversity of ManitobaAdam BurgenerProteinAmino acidsketo acidsAmmoniaUreaexcretionPyruvateTCA intermediatesetc.EnergyCHCNH3+ROO-CCOROO-University of ManitobaAdam BurgenerCOCHNH3+CH3O-CCOCH2OCH2CO-O-OGlutamateAlaninePyruvate-KGCCOCH2OCH2CHO-O-NH3+COCOCH3O-Alanine t
2、ransaminaseTransamination and deaminations involved in removal of amino group from an amino acidPLPUniversity of ManitobaAdam BurgenerAttaches to a lysine residue on the transaminase enzymeUniversity of ManitobaAdam BurgenerEnzymeNH+CHPLPLysCHCNH3+ROO-Amino acidEnzymeCHCNH+ROO-CHPLPNH3+LysOH2CCOROO-
3、Keto acidEnzymeNH3+LysNH3+CH2PLPCCOROO-Keto acidEnzymeCCNH+ROO-CH2PLPNH3+LysOH2EnzymeCHCNH+ROO-CHPLPNH3+LysCHCNH3+ROO-New Amino acidSTARTUniversity of ManitobaAdam BurgenerAmino acidKeto acid-KGGlutamateBuild-upGlutamateCCOCH2OCH2CHO-O-NH3+Glutamine synthetaseNH3ATPADP+PiCCOCH2OCH2CHO-NH2NH3+Glutami
4、neGlutamateCCOCH2OCH2CHO-O-NH3+GlutaminaseNH4+CCOCH2OCH2CHO-NH2NH3+GlutamineOH2Glutamate dehydrogenaseNAD(P)+NAD(P)H+H+CCOCH2OCH2CO-O-O-KGNH4+Urea cycleUrea cycle(blood)LiverUniversity of ManitobaAdam BurgenerUniversity of ManitobaAdam BurgenerAmino acidsketo acids-KGGluGlnNH4+transaminaseGln synthe
5、taseBloodGlnLIVERGlu-KGNH4+NH4+GlutaminaseGlu DehydrogenaseTCAUniversity of ManitobaAdam BurgenerCHCH2NH3+COO-OHSerineCCH3OCOO-PyruvateOH2OH2Serine dehydrataseNH4+PLPCHCHCOO-COO-FumarateCHCH2COO-COO-NH3+NH4+Aspartate deaminaseAspartateUniversity of ManitobaAdam BurgenerNH3CNH3+NH3+OUreaUniversity of
6、 ManitobaAdam BurgenerAmino acids(excess)keto acids-KGGluNH4+Carbamoyl PhosphateCO22 ATP2 ADP+2 PiOrnithineCitrullineAspartateArginosuccinateArginineFumarateOAAMalateCitrullineOrnithineUREAAmino acids(excess)keto acidsGlu-KGMitochondriaCytosol2 ATP2 ADP+2 PiUniversity of ManitobaAdam BurgenerNH4+HCO
7、3-(1)+2 ATPCONH2(P)+2 ADP+2 Pi+2 H+Carbamoyl-P synthetase(2)O-O-NH3+OOCHCCCH2CHCCH2CH2CH2NH2NH3+OO-Ornithine+CONH2(P)Carbamoyl PhosphateCarbamoyl PhosphateOrnithine transcarbamoylaseCONH2CHCCH2CH2CH2NHNH3+OO-CitrullinePi(3)CONH2CHCCH2CH2CH2NHNH3+OO-Citrulline+AspartateArginosuccinate synthetaseATPAM
8、P+PPi2 PiO-O-NHOOCHCCCH2CNH2+CHCCH2CH2CH2NHNH3+OO-Arginosuccinate(4)O-O-NHOOCHCCCH2CNH2+CHCCH2CH2CH2NHNH3+OO-ArginosuccinateArginosuccinate lyaseNH2CNH2+CHCCH2CH2CH2NHNH3+OO-O-O-OOCHCCCHFumarateArginine(5)NH2CNH2+CHCCH2CH2CH2NHNH3+OO-Arginine+OH2ArginaseCHCCH2CH2CH2NH2NH3+OO-Ornithine+NH2CNH2OUreafr
9、om carbamoyl-Pfrom aspartateUniversity of ManitobaAdam BurgenerUniversity of ManitobaAdam BurgenerCitrate-KGSucc CoAFumarateOAAgluglnhisproargIlethrmetvalphetyraspasnleulysphetyrtrpileleutrpcystrpglyseralathrAcetoacetyl CoAAcCoAPyruvateABBCUniversity of ManitobaAdam BurgenerCHCOCHNH3+OHCH3O-CONH3+CH
10、2O-AlanineGlycineThreonineCONH3+CHO-CH2OHSerine hydroxymethyl transferaseCH3CHOAcetaldehydePLPSerineFH4-CH2 Methylene-tetrahydrofolateFH4TetrahydrofolateOH2Serine dehydrataseSerine hydroxy-methyl transferasePLPCONH3+CHO-CH3COOCO-CH3PyruvateOH2OH2NH4+COOCO-SCoAAcetyl CoAPyruvate dehydrogenaseCO2NAD+N
11、ADH+H+CoASH-KGGluAlanine aminotransferaseUniversity of ManitobaAdam BurgenerUniversity of ManitobaAdam BurgenerNNHNNNHglutamateOONH2NHCH2H510NNHNNNHglutamateOONH2NHCH2HHHNNHNNNHglutamateOONH2NHCH2HHHHHNADPH+H+NADP+NADPH+H+NADP+Folate=FDihydrofolate=FH2Tetrahydrofolate=FH4551010Dihydrofolate reductas
12、eDihydrofolate reductaseUniversity of ManitobaAdam BurgenerNNHNNNHglutamateOONH2NHCH2HHHHHSerineGlycineSerine hydroxymethyl transferaseFH4510510CH2NNHNNNHglutamateOONH2NCH2HHHHN5,N10-methylene FH4OH2NADP+NADPH+H+510CHNNHN+NNHglutamateOONH2NCH2HHHHN5,N10-methenyl FH4Donates formyl groups in purine bi
13、osynthesis pathwayMethenyl FH4 reductase510OH2CHNNHNHNNHglutamateOONH2NCH2HHHHON10-Formyl FH4CycohydrolaseInvolved in pyrimidine biosynthesis pathway,amino acid synthesisUniversity of ManitobaAdam BurgenerPhenylalanineNAD+NADH+H+TetrahydrobiopterinDihidrobiopterinOH2O2Phenylalanine hydroxylaseTyrosi
14、neONH3+O-ONH3+OHO-COCCH2OOHO-p-Hydroxyphenyl pyruvateTyrosine aminotransferasePLP-KGGluO2CO2P-hydroxyphenylpyruvate dioxygenaseCCH2OOHOHO-HomogentisateHomogentisate 1,2-dioxygenaseO2H+CCH2CCCCH2OO-OCCOO-OHHMaleyl acetoacetateCCH2CCCCH2OO-OCCOO-OHHMaleyl acetoacetate isomeraseFumaryl acetoacetateFuma
15、ryl acetoacetaseOH2CCH3CCH2OO-OCCCCOO-OHHO-FumarateAcetoacetateSuccSucc CoAKetoacyl CoA transferaseCCH3CCH2OSCoAOAcetoacetyl CoAa C4 fatty acid-keto acyl CoA thiolase(-oxidation pathway)CCH3OSCoA2 xAcetyl CoATCA CYCLEUniversity of ManitobaAdam BurgenerCCHONH3+CHCH3CH3O-ValineBranched chain AA-transa
16、minase-KGGluCCOOCHCH3CH3OH-keto isovalerate-ketoacyl dehydrogenaseTPP,FADCoASHNAD+NADH+H+CO2SCoACOCHCH3CH3Isobutyryl CoAAcyl CoA dehydrogenaseFADFADH2SCoACOCCH3CH2Methyl acrylyl CoASCoACOCHCH3CH2OHOH2Enoyl CoA hydratase-hydroxy isobutyryl CoAhydroxy isobutyryl CoA hydrolaseCoASHO-COCHCH3CH2OH-hydrox
17、yl-isobutyrate-hydroxy isobutyryl dehydrogenaseNAD+NADH+H+O-COCHCH3CHOMethyl malonyl semi-aldehydeMethyl malonyl semi-aldehyde dehydrogenaseNAD+NADH+H+CO2SCoACOCH2CH3CoASHPropionyl CoA(1)Propionyl CoA carboxylase(2)Methyl malonyl CoA epimerase(3)Methyl Malonyl CoA mutaseSCoACOCH2CH2CO-OSuccinyl CoAOH2PLPUniversity of ManitobaAdam Burgener谢谢！
