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DOI: 10.3791/65426-v
Please note that some of the translations on this page are AI generated. Click here for the English version.
这项工作提出了一种内皮到间充质转化诱导的纤维化的动物模型,如先天性心脏缺陷,如严重主动脉瓣狭窄或左心发育不良综合征,可以进行详细的组织学组织评估、调节信号通路的鉴定和治疗方案的测试。
心内膜纤维弹性变性阻碍了左心室生长,并阻碍了先天性危重主动脉瓣狭窄和左心发育不良综合征患者的双心室手术修复。虽然手术切除是目前唯一可用的治疗选择,但 EFE 经常复发。因此,我们旨在研究 EFE 背后的机制,并使用动物模型开发新的治疗策略。
EndMT研究领域的主要发展仅限于具有固有局限性的细胞培养模型,并且由于缺乏体内模型,研究受到阻碍。我们小组先前确定,EFE形成的潜在机制涉及心内膜内皮细胞向成纤维细胞的表型变化,这一过程称为内皮到间充质转化。该动物模型允许对EndMT诱导的心脏纤维化进行更全面的检查,而其他模型则旨在通过基因修饰,高血压或饮食限制来诱导EndMT。
由于 EFE 经常在初次切除后复发,有时甚至具有更浸润的生长模式,因此我们对与潜在心肌的分子相互作用特别感兴趣。
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