骨髓增生异常综合征患者线粒体铁蛋白表达分析

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1、摘要照组相比差异均具有统计学意义(p<0．05)，总铁结合力(TIBC)在正常范围内，与对照组相比差异无统计学意义(p>0．05)；2、19例MDS患者中17例检测到MtFt基因的表达，11例对照组患者中均未检测到目的基因的表达，实验者的阳性表达率高于对照组，且差异具有统计学意义(P<0．05)；3、MtFt基因在MDS4个亚型间的表达不完全相同(p<0．05)，RARS亚型MtFt基因的相对表达量高于RA、RAEB一1及RAEB．2亚型，差异均具有统计学意义(p<0．0083)，RA、RAEB．1及RAEB．2亚型间M

2、tFt基因表达量的差异无统计学意义(P>0．0083)．结论l、MtFt基因在实验组骨髓细胞中的阳性表达率显著高于对照组；2、MtFt基因在MDS各亚型中的相对表达量不完全相同，RARS患者的MtFt基因相对表达量高于RA、RAEB．1及RAEB．2患者；3、MtFt基因在MDS患者骨髓细胞中的表达可能与MDS患者(尤其是RARS)线粒体铁超载的形成有关。关键词：骨髓增生异常综合征；铁超载；线粒体铁蛋白AbstractIronisavitalelementforalmostalllivingorganisms，asiti

3、sacofactorforenzymesinvolvedinmanymetabolicprocessessuchaSoxygentransport,DNAsynthesis，nitrogenfixation，electrontransportandphotosynthesis．ButitCanalsobeharmful，sinceitsexcessisthoughttoenhancetheproductionofreactiveoxygenspeciesandinduceoxidativedamagewhichthenc

4、ausedamagetomembranes，proteinsandnucleicacids．Mitochondriahaveakeyroleinironmetabolism，becausehemeandvariousiron—sulfur(Fe—S)cluster-containingproteinsaresynthesizedinthem．Asthemostactiveorganellesofredoxreactionelectrontransfer,mitochondrialironlevelsmustbewellr

5、egulatedbecauseaninadequatesupplyofironwouldimpairthemetabolicandrespiratoryactivitiesoftheorganelle．InthesametimedefectsinmitochondrialirontransportandutilizationCanresultinmitochondrialironoverload．Recently,mitochondrialferritin(MtFt)hasbeenidentifiedasanovelfe

6、rritintypespecificallytargetedtomitochondria，namedbyLeviin2001．ThesequenceofthematureproteinisidenticaltothatofthecytosolicH-ferritinandhasferroxidaseactivity．Inhumanitisencodedbyanintronlessgenelocatedonchromosome5q23．1，Thecorrespondingfull-lengthmRNAhasasizesim

7、ilartothatofcytosolicferritinbutlackstheIREconsensussequenceforiron-dependenttranslationalcontr01．MtFthasathree-dimensionalstructureandbiochemicalpropertiesanalogoustothoseofhumanH-ferritin．RelatedstudiesimplicatedthatFtMtmayplayaimportantroleintheprocessofironme

8、tabolism，oxidativestressdamageandcellapoptosis．Myelodysplasticsyndromes(MDS)areaheterogeneousgroupofclonalstemcelldisorderscharacterizedbydysplasticcytopeniaso