Hypoplastic left heart syndrome (HLHS) is a complex congenital heart disease characterized by abnormalities in the left ventricle, associated valves, and ascending aorta. Studies have shown intrinsic myocardial defects but do not sufficiently explain developmental defects in the endocardial-derived cardiac valve, septum, and vasculature. Here, we identify a developmentally impaired endocardial population in HLHS through single-cell RNA profiling of hiPSC-derived endocardium and human fetal heart tissue with an underdeveloped left ventricle. Intrinsic endocardial defects contribute to abnormal endothelial-to-mesenchymal transition, NOTCH signaling, and extracellular matrix organization, key factors in valve formation. Endocardial abnormalities cause reduced cardiomyocyte proliferation and maturation by disrupting fibronectin-integrin signaling, consistent with recently described de novo HLHS mutations associated with abnormal endocardial gene and fibronectin regulation. Together, these results reveal a critical role for endocardium in HLHS etiology and provide a rationale for considering endocardial function in regenerative strategies.
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Recently, research on the production and transformation of sorbitol has become exciting in chemical industry and in catalysis studies for its broad applications. It opens up a new path for achieving sustainable energy supply and chemicals production. Here we mainly review the catalytic routes for the synthesis of sorbitol and conversion of sorbitol into high value-added compounds such as lower alcohols, paraffins, isosorbide, and other derivatives. Meanwhile, some promising and valuable research directions are suggested based on the major challenges emerged in current research, such as the development of efficient magnetic catalysts, microwave heating, and other hydrogen sources.
The configurational entropy is an emerging descriptor in the functional materials genome. In thermoelectric materials, the configurational entropy helps tune the delicate trade‐off between carrier mobility and lattice thermal conductivity, as well as the structural phase transition, if any. Taking GeTe as an example, low‐entropy GeTe generally have high carrier mobility and distinguished zT > 2, but the rhombohedral‐cubic phase transition restricts the applications. In contrast, despite cubic structure and ultralow lattice thermal conductivity, the degraded carrier mobility leads to a low zT in high‐entropy GeTe. Herein, medium‐entropy alloying is implemented to suppress the phase transition and achieve the cubic GeTe with ultralow lattice thermal conductivity yet decent carrier mobility. In addition, co‐alloying of (Mn, Pb, Sb, Cd) facilitates multivalence bands convergence and band flattening, thereby yielding good Seebeck coefficients and compensating for decreased carrier mobility. For the first time, a state‐of‐the‐art zT of 2.1 at 873 K and average zTave of 1.3 between 300 and 873 K are attained in cubic phased Ge0.63Mn0.15Pb0.1Sb0.06Cd0.06Te. Moreover, a record‐high Vickers hardness of 270 is attained. These results not only promote GeTe materials for practical applications, but also present a breakthrough in the burgeoning field of entropy engineering.
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