Expression data of MCK conditional frataxin knock-out mice

Frataxin deficiency in human is the cause of Friedreich's ataxia (FA), a lethal neuro- and cardio-degenerative disease. Knock-out (KO) mice of this mouse model of FA exhibit classical cardiomyopathy of the patients. The onset of FA phenotypes in the KO mice is approximately 6-7 weeks of age. This gene array analysis was conducted to examine the changes in gene expression in the heart of KO mice relative to their wild-type (WT) littermates at 4- and 10-weeks of age. At 10-weeks of age, the KO mice begin to die from severe cardiomyopathy. RNA from the heart of four female 4-week-old MCK littermates (two WT and two KO) and four female 10-week-old MCK littermates (two WT and two KO) was extracted and hybridised to Affymetrix Mouse Genome 430 2.0 Array.人类体内frataxin（Frataxin）缺陷是弗里德赖希共济失调（Friedreich's ataxia, FA）的致病原因，该病是一种致命的神经与心脏退行性疾病。本FA小鼠模型的敲除（Knock-out, KO）小鼠可呈现患者的典型心肌病表型。KO小鼠的FA表型发病年龄约为6~7周龄。本研究开展基因芯片分析，旨在检测4周龄与10周龄的KO小鼠心脏中，相较于同窝野生型（wild-type, WT）小鼠的基因表达变化情况。10周龄时，KO小鼠会因严重心肌病开始死亡。研究人员提取了4只4周龄MCK同窝雌鼠（2只WT、2只KO）以及4只10周龄MCK同窝雌鼠（2只WT、2只KO）的心脏RNA，并将其与Affymetrix Mouse Genome 430 2.0 基因芯片进行杂交。