chapter生物最初能量来自阳光

Chapter5线粒体和叶绿chemicalenergy(synthesis,electronicenergy(nervemechanicalenergy(muscularcontraction,cellosmoticenergy(absorption,secretion,Na-Klightenergyheatenergy(body

水氧 Sunlightenergyintochemical

有机 粒体中转换成

“powerhouse”inaMitochondriapresentinnearlyalleucaryoticcells §5.1线粒

FirstdescriptionsofmitochondriaFormulationofureaandTCAcycleRespirationchainandOXPHOSDiscoveryofDNAinmitochondriaFormulationofChemiosmoticHypothesisCompletesequenceofamammalianmtDNAFirstidentificationofmitochondrialdiseasesCompletesequenceofplantmtDNAsMitochondriaandapoptosisCrystalstructuresofcomplexIII,IV,F1-Crystalstructureofcomplex线粒体研究的又一个黄金时Cell’s§5.1.1 线粒 线粒体的可塑

四个独立的成6％ofmitochondtialproteinschannel-formingprotein(porin)permeabletoallof<500021%ofimpermeabletoionsandmostsmallmoleculesfoldedintonumerouscristaethreetypesproteins:respiratorychainATPsynthasetransportproteins6％ofchemicallyequivalenttothe67%ofoxidationofpyruvate,fattyacidsandcitricacidcycleenzymesforexpressionofmitochondrialgenesseveralcopiesofmtDNAgenomemitochondrialribosomesmitochondrialtRNAs生化成不溶性蛋白：integralandstructural脂外膜：halfproteinandhalf内膜：80%proteinand20§5.1.2

β三

净结AcetylCoA+2H2O+3NAD++FAD +3NADH+FADH2 + + 氧化磷酸能量转换过氧化磷酸Real-time 电子传递链的成 ZiheRaoetal.,Cell(2005)121:1043-MitochondrialInhibitorsandAntimycin

F(orComplexNADH-CoQredu e(NADHdehydrogenase)2e-fromNADHtoCoQ2H+transmembraneComplexsuccinate（琥珀酸）-CoQredu e(succinatee-fromsuccinatetoComplexCoQ-CytCredu 2e-fromCoQtoCytCComplexIV2e-fromCytCtoO2E0(V)-0.32

ATP合成膜膜F0—H+F1—light-drivenbacterialprotonpump

ATPsynthaseATPsynthesisinresponsetolight

SynthesizeATPbyharnessingtheproton-motiveforcePumpprotonsagainsttheirThedirectiondependsonthebalancebetweenthesteepnessoftheelectrochemicalprotongradientandthelocalfree-energyforATPhydrolysisRespiratorychainintheinnermembraneisprotontranslocating;itpumpsH+outofthematrixspacewhenelectronsaretransportedalongchain;ATPsynthasealsotranslocatesprotonsacrosstheinnermembrane.pumpH+intointermembranespacebyATPhydrolysisATPsynthesisdrivenbylargeenoughelectrochemicalH+gradientInnermembraneisequippedwithasetofcarrierproteinsthatmediatetheentryandexitofessentialmetabolitesandselectedinorganicions;InnermembraneisimpermeabletoH+,OH-andgenerallytoanionsandcations.线粒体的三大功

Energyproduction（产能Generationofreactiveoxygenspecies（活性Regulationofapoptosis（调控细胞凋亡§5.1.2线粒

mtDNA的结Doubledstrands,circularmoleculewithabout16,569bpEncoding13polypeptideswhichareessentialcomponentsofoxidativephosphorylation22tRNAsand2rRNAsarecomponentsofproteinsynthesisapparatusMajorityofmitochondrialproteinsareencodedbynucleargenesOXPHOS 亚基数核编码亚基mtDNA编码亚I:NADH 7(ND1,2,3,4,4L,5, II: e 1(CytoeIV:Cytochrome 3(CoxI,II,V:ATP 2(ATPase6,

组

细胞器

哺乳动物线粒 的遗传特母系遗多拷高突同质突变和异质Decadeofthe

Invitro

线粒体疾心

肝核 亚

肾核

线粒体

Am.J.Hum.Genet.2006;79:291-302(IFTRMU的突变分ImportintomitochondriaandprocessingofTRMUprecursor 突变2-尿嘧啶修饰的定线粒体tRNA的Northern杂J.Biol.Chem.2005;280:29151-29157(IFmto2葡萄 甘线粒 酵母的Mto2p酵母MTO2与人MTO2§5.2叶绿外 叶绿体的Threeoutermembrane（外膜）innermembrane（内膜）Threeintermembranespace（外膜空间）thylakoidspace（类囊体腔核

solubleproteinsandmetabolicRuBPase(ribulose-1,5-biophosphateplayacentralroleinthe80%ofthylakoidproteinschloroplastDNARNA(rRNA,tRNA,stachgrainlipiddropletStackedthylakoid—Granum（基粒granumlamella（基粒片层）stromalamella（基质片层AinterconnectedandclosedmembraneElectron-transport（电子的转移ATPsynthase（ATP的合成Comparisonofmitochondriaand嵴基 基WaterisoxidizedandoxygenisreleasedinthephotosyntheticCarbondioxideisfixedinthecarbon-fixationreactionLightreaction（光反应）alsoas“photosyntheticelectron-transferreaction”energy chemicalprocessphotodegradationofwater(primaryreaction)electrontransportphotosyntheticphosphorylationfinalproductsATPandPrimaryreaction（初absorbsunlightexcitedbyaunstableandtendtoreturntoitsoriginal,unexcitedstate激活叶绿体II分子回复原状的三

可能 Lightand

SuccessiveelectronPhotosystem（PS光系统catalyzetheconversionofthelightenergycapturedinexcitedchlorophyllmoleculestousefulformstwoclosedlinkedcomponentscapturelightenergyandfeedittoreaction反应中心）--enablelightenergyintochemicalenergy

D-P-

D-P*- D-P+- D+-P-P680primaryelectrondonor—electronaccepter— P700electrondonor— elect