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首页> 外文期刊>Circulation research: a journal of the American Heart Association >Hungry for more: Autophagy in the pathogenesis of pulmonary arterial hypertension
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Hungry for more: Autophagy in the pathogenesis of pulmonary arterial hypertension

机译:渴望更多:自噬在肺动脉高压的发病机理中

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摘要

Genetic alterations in pulmonary arterial hypertension (PAH) have become increasingly recognized in both known familial or heritable and sporadic or idiopathic PAH (IPAH). Unrecognized genetic alterations have now been found in up to 40% of the IPAH in which no familial predisposition is apparent. Bone morphogenetic protein (BMP) receptor II (BMPR-H) is the most common gene implicated in this hereditary form of PAH; furthermore, it is implicated in the pathogenesis of nonhereditary forms of PAH with a significant reduction in the expression of BMPR-II in both IPAH and experimental animal models of pulmonary hypertension (PH) BMPs represent the largest group of cytokines in the transforming growth factor-beta superfamily and regulate growth, differentiation, and apoptosis in multiple cell types, whereas BMPR-II has been shown to have unique roles in differing cells.
机译:在已知的家族性或遗传性和偶发性或特发性PAH(IPAH)中,肺动脉高压(PAH)的遗传改变已得到越来越多的认可。现在,在多达40%的IPAH中发现了无法识别的遗传变异,其中没有明显的家族性倾向。骨形态发生蛋白(BMP)受体II(BMPR-H)是这种PAH遗传形式中最常见的基因。此外,它也与非遗传性PAH的发病机理有关,IPAH和肺动脉高压(PH)实验动物模型中BMPR-II的表达均显着降低。BMP代表转化生长因子-中最大的细胞因子组。 β超家族并调节多种细胞类型的生长,分化和凋亡,而BMPR-II已显示在不同细胞中具有独特作用。

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