
Overview:
PDHA1pyruvatedehydrogenase(lipoamide)alpha1isamemberofthepyruvatedehydrogenase(PDH)complex.PDHA1isanuclear-encodedmitochondrialmultienzymecomplexwhichcatalyzestheoverallconversionofpyruvatetoacetyl-CoAandCO(2),andprovidestheprimarylinkbetweenglycolysisandthetricarboxylicacid(TCA)cycle.PDHA1playsacriticalroleinthebrainthatusuallyobtainsallofitsenergyfromtheaerobicoxidationofglucose(1).MutationsinthePDHA1areassociatedwithpyruvatedehydrogenaseE1-alphadeficiencyandX-linkedLeighsyndrome(2).
GeneAliases:
PDHA;PDHCE1A;PHE1A
GenbankNumber:
NM_000284
References:
1.Brown,G.K.et.al:Pyruvatedehydrogenasedeficiency.J.Med.Genet.31:875-879,1994.2.Chun,K.et.al:MutationsintheX-linkedE1-alphasubunitofpyruvatedehydrogenaseleADIngtodeficiencyofthepyruvatedehydrogenasecomplex.Hum.Molec.Genet.2:449-454,1993.
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算上其他生物24种
大概是记不太清了
NaturalProteinStopsDeadlyHumanBrainCancerInMice
ScientistsfromJohnsHopkinsandfromtheUniversityofMilanhaveeffectivelyproventhattheycaninhibitlethalhumanbraincancersinmiceusingaproteinthatselectivelyinducespositivechangesintheactivityofcellsthatbehavelikecancerstemcells.ThereportispublishedinNature.
Themostcommontypeofbraincancer-glioblastoma-ismarkedbythepresenceofthesestem-cell-likebraincells,which,insteadoftriggeringthereplacementofdamagedcells,formcancertissue.Stemcells,unlikeallothercellsinthebody,arecapableofformingalmostanykindofcellwhentheright"signals"triggertheirdevelopment.
Fortheirtreatmentexperiment,theresearchersreliedonaclassofproteins,bonemorphogenicproteins,thatcauseneuralstem-cell-likeclusterstolosetheirstemcellproperties,whichinturnstopstheirABIlitytodivide.
Firsttheypretreatedhumanglioblastomacellswithbonemorphogenicprotein4(BMP4),theninjectedthesetreatedcellsintomousebrains.Inmiceinjectedwithcellsthatwerenotpretreated,large,invasivecancersgrew.InthemicewithBMP4-treatedcells,nocancersgrewatall.Threetofourmonthsafterinjection,allmicethatgotuntreatedcellsdied,andnearlyallmicewithBMP4-treatedcellswerealive.
Next,thescientistsdeliveredslow-releaseBMP4-containing"beads"directlyintomousebrainswithimplantedglioblastomacells.Micethatgotemptybeadsdevelopedlargemalignanttumorsanddied.MicewithBMP4beadssurvivedmuchlonger,and80percentsurvivedfourmonthsaftercancercellimplants.
"OurideaistotreatpatientswithBMP4orsomethinglikeitrightaftersurgerytoremoveglioblastomainhopesofpreventingtheregrowthofthecancerandimprovingsurvivaltime,"saysAlessandroOlivi,M.D.,directoroftheDivisionofNeurosurgicalOncologyatHopkinsandacontributortothestudy.
OlivisaysclinicalstudiesusingBMP4couldbeginwithinayearand,ifsuccessful,drugtherapiescouldbeavailabletothepublicwithinthreetofouryears.
"ThiswasproofoftheideathatBMPscouldstopglioblastomabydepletingthestem-cell-likepopulationthatfeedsit,"saysHenryBrem,M.D.,chairmanoftheDepartmentofNeurosurgeryatHopkinsandacollaboratorinthestudy."Thisopensexcitingdoorstofutureresearchintotreatmentsandtherapiesforsuchadevastatingdisease."

