Mitochondrial 2,4-dienoyl-CoA Reductase Deficiency in Mice Results in Severe Hypoglycemia with Stress Intolerance and Unimpaired Ketogenesis 英文参考文献.docVIP

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Mitochondrial 2,4-dienoyl-CoA Reductase Deficiency in Mice Results in Severe Hypoglycemia with Stress Intolerance and Unimpaired Ketogenesis 英文参考文献.doc

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Mitochondrial 2,4-dienoyl-CoA Reductase Deficiency in Mice Results in Severe Hypoglycemia with Stress Intolerance and Unimpaired Ketogenesis 英文参考文献

Mitochondrial2,4-dienoyl-CoAReductaseDeficiencyin MiceResultsinSevereHypoglycemiawithStress IntoleranceandUnimpairedKetogenesis IlkkaJ.Miinalainen1,WernerSchmitz2,AnneHuotari3,KaijaJ.Autio1,RaijaSoininen4,EmielVerLoren vanThemaat5,MyriamBaes6,Karl-HeinzHerzig3,7,ErnstConzelmann2,J.KalervoHiltunen1* 1Department of Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland, 2Theodor-Boveri-Institut fu¨r Biowissenschaften (Biozentrum) der Universita¨t Wu¨rzburg,Wu¨rzburg,Germany,3DepartmentofBiotechnologyandMolecularMedicine,A.I.VirtanenInstituteforMolecularSciences,Kuopio,Finland,4Departmentof Medical Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland, 5Bioinformatics Laboratory, Department of Clinical Epidemiology, Biostatistics and Bioinformatics,AcademicMedicalCenter,Amsterdam,TheNetherlands,6LaboratoryofCellMetabolism,DepartmentofPharmaceuticalSciences,KatholiekeUniversiteit Leuven,Leuven,Belgium,7DepartmentofInternalMedicine,KuopioandInstituteofBiomedicine,DivisionofPhysiologyandBiocenterofOulu,OuluUniversityMedical School,Oulu,Finland Abstract Themitochondrialb-oxidationsystemisoneofthecentralmetabolicpathwaysofenergymetabolisminmammals.Enzyme defectsinthispathwaycausefattyacidoxidationdisorders.Toelucidatetheroleof2,4-dienoyl-CoAreductase(DECR)asan auxiliaryenzymeinthemitochondrialb-oxidationofunsaturatedfattyacids,wecreatedaDECR–deficientmouseline.In Decr2/2mice,themitochondrialb-oxidationofunsaturatedfattyacidswithdoublebondsisexpectedtohaltatthelevelof trans-2,cis/trans-4-dienoyl-CoAintermediates.Inlinewiththisexpectation,fastedDecr2/2micedisplayedincreasedserum acylcarnitines, especially decadienoylcarnitine, a product of the incomplete oxidation of linoleic acid (C18:2), urinary excretion of unsaturated dicarboxylic acids, and hepatic steatosis, wherein unsaturated fatty acids accumulate in liver triacylglycerols.MetabolicallychallengedDecr2/2miceturnedonketogenesis,butunexpectedlydevelopedhypoglycemia. Induced expression of perox