脂质代谢课件课件.ppt

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2025-03-04 发布于广东
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脂质代谢课件课件.ppt

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*Afterfattyacyl–carnitineisformedattheoutermembraneorintheintermembranespace,itmovesintothematrixbyfacilitateddiffusionthroughthetransporterintheinnermembrane.Inthematrix,theacylgroupistransferredtomitochondrialcoenzymeA,freeingcarnitinetoreturntotheintermembranespacethroughthesametransporter.AcyltransferaseIisinhibitedbymalonyl-CoA,thefirstintermediateinfattyacidsynthesis(seeFig.21–1).Thisinhibitionpreventsthesimultaneoussynthesisanddegradationoffattyacids.**Inhumansandmostothermammals,acetyl-CoAformedintheliverduringoxidationoffattyacidscaneitherenterthecitricacidcycleorundergoconversiontothe“ketonebodies,”acetone,acetoacetate,andD-β-hydroxybutyrate,forexporttoothertissues.Acetone,producedinsmallerquantitiesthantheotherketonebodies,isexhaled.AcetoacetateandD-β-hydroxybutyratearetransportedbythebloodtotissuesotherthantheliver(extrahepatictissues),wheretheyareconvertedtoacetyl-CoAandoxidizedinthecitricacidcycle,providingmuchoftheenergyrequiredbytissuessuchasskeletalandheartmuscleandtherenalcortex.Thebrain,whichpreferentiallyusesglucoseasfuel,canadapttotheuseofacetoacetateorD-β-hydroxybutyrateunderstarvationconditions,whenglucoseisunavailable.Theproductionandexportofketonebodiesfromthelivertoextrahepatictissuesallowscontinuedoxidationoffattyacidsintheliverwhenacetyl-CoAisnotbeingoxidizedinthecitricacidcycle.**Healthy,well-nourishedindividualsproduceketonebodiesatarelativelylowrate.Whenacetyl-CoAaccumulates(asinstarvationoruntreateddiabetes,forexample),thiolasecatalyzesthecondensationoftwoacetylCoAmoleculestoacetoacetyl-CoA,theparentcompoundofthethreeketonebodies.Thereactionsofketonebodyformationoccurinthematrixoflivermitochondria.Thesix-carboncompound-hydroxy--methylglutary