Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension.
Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension.
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DOI：
10.3390/ijms19092499
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发表时间：
2018-08-24
影响因子：
5.6
通讯作者：
Spiekerkoetter E
中科院分区：
文献类型：
--
作者：
Andruska A;Spiekerkoetter E
自2000年与家族性肺动脉高压（PAH）相关以来，骨形态发生蛋白受体II（BMPR2）及其相关信号通路已被公认为肺血管稳态的关键调节因子。在此，我们将BMPR2缺陷定义为受体失活、受体表达降低或受体下游信号通路受损。尽管传统上认为PAH中BMPR2缺乏的表型后果仅限于肺血管系统，但有证据表明BMPR2信号传导异常可能对许多其他器官系统和细胞区室产生影响。重新审视BMPR2在整个健康和疾病过程中如何在肺血管系统以外的细胞和器官中发挥作用，可以深入了解这些器官系统对PAH发病机制的贡献以及PAH的潜在全身表现。在这里，我们回顾了我们对BMPR2缺乏在多个器官系统中的后果的了解。
Since its association with familial pulmonary arterial hypertension (PAH) in 2000, Bone Morphogenetic Protein Receptor II (BMPR2) and its related signaling pathway have become recognized as a key regulator of pulmonary vascular homeostasis. Herein, we define BMPR2 deficiency as either an inactivation of the receptor, decreased receptor expression, or an impairment of the receptor’s downstream signaling pathway. Although traditionally the phenotypic consequences of BMPR2 deficiency in PAH have been thought to be limited to the pulmonary vasculature, there is evidence that abnormalities in BMPR2 signaling may have consequences in many other organ systems and cellular compartments. Revisiting how BMPR2 functions throughout health and disease in cells and organs beyond the lung vasculature may provide insight into the contribution of these organ systems to PAH pathogenesis as well as the potential systemic manifestation of PAH. Here we review our knowledge of the consequences of BMPR2 deficiency across multiple organ systems.
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影响因子：
7.9
作者：
Austin ED;Hamid R;Hemnes AR;Loyd JE;Blackwell T;Yu C;Phillips Iii JA;Gaddipati R;Gladson S;Gu E;West J;Lane KB
通讯作者：
Lane KB
影响因子：
2.6
作者：
Brittain, Evan L.;Pugh, Meredith E.;Hemnes, Anna R.
通讯作者：
Hemnes, Anna R.
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8.9
作者：
Claessen, Guido;La Gerche, Andre;Heidbuchel, Hein
通讯作者：
Heidbuchel, Hein
BMPR2在氧化过程中保留线粒体功能和DNA，以促进内皮细胞的存活和反向肺动脉高压。
影响因子：
29
作者：
Diebold I;Hennigs JK;Miyagawa K;Li CG;Nickel NP;Kaschwich M;Cao A;Wang L;Reddy S;Chen PI;Nakahira K;Alcazar MA;Hopper RK;Ji L;Feldman BJ;Rabinovitch M
通讯作者：
Rabinovitch M
影响因子：
37.8
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Caruso P;Dunmore BJ;Schlosser K;Schoors S;Dos Santos C;Perez-Iratxeta C;Lavoie JR;Zhang H;Long L;Flockton AR;Frid MG;Upton PD;D'Alessandro A;Hadinnapola C;Kiskin FN;Taha M;Hurst LA;Ormiston ML;Hata A;Stenmark KR;Carmeliet P;Stewart DJ;Morrell NW
通讯作者：
Morrell NW