Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Gene-Targeted Mice 英文参考文献.docVIP

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Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Gene-Targeted Mice 英文参考文献.doc

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Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Gene-Targeted Mice 英文参考文献

Medium-ChainAcyl-CoADehydrogenase DeficiencyinGene-TargetedMice Ravi J.Tolwani1,2,Doug A.Hamm1,Liqun Tian1,J.Daniel Sharer1,Jerry Vockley3,4,Piero Rinaldo5,Dietrich Matern5, Trenton R.Schoeb1,Philip A.Wood1* 1DepartmentofGenetics,UniversityofAlabama,Birmingham,Alabama,UnitedStatesofAmerica,2DepartmentofComparativeMedicine,StanfordUniversity,Stanford, California, United States of America, 3 Department of Medical Genetics, Mayo Clinic College of Medicine, Rochester, Minnesota, United States of America, 4 Division of MedicalGenetics,Children’sHospital,UniversityofPittsburgh,Pittsburgh,Pennsylvania,UnitedStatesofAmerica,5LaboratoryMedicineandPathology,MayoClinicCollege ofMedicine,Rochester,Minnesota,UnitedStatesofAmerica Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inherited disorder of mitochondrial fattyacidb-oxidationinhumans.Tobetterunderstandthepathogenesisofthisdisease,wedevelopedamousemodel for MCAD deficiency (MCAD ) by gene targeting in embryonic stem (ES) cells. The MCADà/à mice developed an organicaciduriaandfattyliver,andshowedprofoundcoldintoleranceat48Cwithpriorfasting.Thesporadiccardiac lesions seen in MCADà/à mice have not been reported in human MCAD patients. There was significant neonatal mortalityofMCADà/àpupsdemonstratingsimilaritiestopatternsofclinicalepisodesandmortalityinMCAD-deficient patients.TheMCAD-deficientmousereproducedimportantaspectsofhumanMCADdeficiencyandisavaluablemodel for further analysis of the roles of fatty acid oxidation and pathogenesis of human diseases involving fatty acid oxidation. à/à Citation:TolwaniRJ,HammDA,TianL,SharerJD,VockleyJ,etal.(2005)Medium-chainacyl-CoAdehydrogenasedeficiencyingene-targetedmice.PLoSGenet1(2):e23. developed a mouse model of MCAD de?ciency by gene targeting in embryonic stem (ES) cells (for reviews [13,14]). Introduction Mitochondrial b-oxidation of fatty acids provides energy, especially during fasting conditions. Fatty acid oxidation occursinmitochondriaandcons