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ABSTRACT

Electric heat pumps feature prominently in projected energy transitions in the UK and elsewhere. Owing to their high electricity consumption, heat pumps are viewed as important targets for demand-side response (DSR). Findings are presented from a field trial of a new control system that aims to optimize heat pump performance, including under time-varying tariff conditions. The trial involved monitoring 76 properties with heat pumps, but without dedicated heat storage; 31 of these received the control system. Interviews were conducted with a subsample of 12 participants. The controller successfully evened out electricity demand over the day (reducing the evening peak), but this was associated with increased late night and daytime temperatures. Interview participants reported some disturbance owing to overnight heating and noise, as well as usability issues with the controller interface and hardware. These issues present risks to the future acceptability of such systems. While the system delivered short-term demand reductions successfully, longer-term demand shifting risked causing unacceptable disturbance to occupants. Future control systems could overcome some of the issues identified in this pioneering trial through more effective zoning, using temperature caps or installing dedicated heat storage, but these may either limit the available flexibility or be challenging to achieve.  相似文献   
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Transmission electron microscopy‐based grain orientation mapping method was employed to investigate the microtexture of the alumina scale formed in commercial thermal barrier coatings (TBCs) with two standard types of Pt‐enriched bond coats. Reliable orientation/phase maps with a spatial resolution down to 2 nm were acquired on the alumina grains. It was observed that the alumina scale on the Pt‐aluminide β‐phase bond coat has a stronger c‐axis texture normal to the bond coat surface, in comparison with that on the Pt‐diffused γ/γ′‐phase bond coat. The microtexture of the alumina scale could affect its effective coefficient of thermal expansion, which is a contributor to the severity of the bond coat rumpling mechanism of TBCs failure.  相似文献   
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Biallelic pathogenic variants in the SEC23B gene cause congenital dyserythropoietic anemia type II (CDA II), a rare hereditary disorder hallmarked by ineffective erythropoiesis, hemolysis, erythroblast morphological abnormalities, and hypo-glycosylation of some red blood cell membrane proteins. Abnormalities in SEC23B, which encodes the homonymous cytoplasmic COPII (coat protein complex II) component, disturb the endoplasmic reticulum to Golgi trafficking and affect different glycosylation pathways. The most harmful complication of CDA II is the severe iron overload. Within our case series (28 CDA II patients), approximately 36% of them exhibit severe iron overload despite mild degree of anemia and slightly increased levels of ERFE (the only erythroid regulator of hepcidin suppression). Thus, we hypothesized a direct role of SEC23B loss-of-function in the pathomechanism of hepatic iron overload. We established a hepatic cell line, HuH7, stably silenced for SEC23B. In silenced cells, we observed significant alterations of the iron status, due to both the alteration in BMP/SMADs pathway effectors and a reduced capability to sense BMP6 stimulus. We demonstrated that the loss-of-function of SEC23B is responsible of the impairment in glycosylation of the membrane proteins involved in the activation of the BMP/SMADs pathway with subsequent hepcidin suppression. Most of these data were confirmed in another hepatic cell line, HepG2, stably silenced for SEC23B. Our findings suggested that the pathogenic mechanism of iron overload in CDA II is associated to both ineffective erythropoiesis and to a specific involvement of SEC23B pathogenic variants at hepatic level. Finally, we demonstrated the ability of SEC23B paralog, i.e., SEC23A, to rescue the hepcidin suppression, highlighting the functional overlap between the two SEC23 paralogs in human hepatic cells.  相似文献   
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Preeclampsia (PE) constitutes one of the principal reasons for maternal and perinatal morbidity and mortality worldwide. The circumstance typically implicates formerly healthful normotensive women, after 20 weeks of gestation, typically withinside the third trimester, without regarded threat elements or past deliveries. PE can be further complicated with hemolysis and thrombocytopenia, leading to the emergence of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low platelets). Both conditions are classified as hypertensive diseases of pregnancy (HDP), and their pathogenesis has been linked to an excessive maternal inflammatory response, accompanied by enhanced endothelial activation. Several studies have found that in pregnancies affected by PE/HELLP, von Willebrand factor (vWF) antigen levels (vWF:Ag) are significantly elevated, while its cleaving protease (ADAMTS-13, A Disintegrin-like and Metalloprotease with Thrombospondin type 1 motif, member 13) activity is normal to decreased. Furthermore, the higher urine excretion of the terminal complement complex C5b-9, as well as its greater deposition in the placental surface in preeclamptic women, imply that the utero-placental unit’s distinctive deficits are intimately tied to disproportionate complement activation. The goal of this updated evaluation is to provide the most up-to-date molecular advances in the pathophysiology of PE/HELLP syndromes. Recent medical data on vWF:Ag levels in patients with PE, ADAMTS-13, and dysregulation of the complement system, are highlighted and evaluated. Furthermore, we discuss the relationship between those entities and the progression of the disease, as well as their significance in the diagnostic process. Finally, considering the difficulties in analyzing and controlling those symptoms in pregnant women, we can provide a current diagnostic and therapeutic algorithm.  相似文献   
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This work investigates the effect of potato presence on the degradation of extra virgin olive oil (EVOO) during repeated frying. For this reason the performance of EVOO during frying was compared with its performance during heating at frying temperatures. In order to make meaningful comparisons the temperature profiles obtained during the frying experiments were replicated during the heating experiments by means of a high energy exchange rate cooling/heating system. The effects of potato‐to‐oil ratio (1/7 and 1/35 kgpotatoes/Loil) and number (N) of batches (N = 0, 1, 0, 20, 30, 40) were examined. EVOO was analysed using high performance size exclusion chromatography (HPSEC). It was found that polymerisation products increased linearly during frying and heating and that they were not affected by potatoes presence at low ratio. The effect of potato presence became statistically significant (yet not remarkable) at the high ratio. Decomposition products increased during frying whereas they decreased during heating.  相似文献   
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