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This study evaluates a clinically applicable lentiviral vector for treatment of Gaucher disease type 1. Hematopoietic stem cells transduced with the vector and transplanted into a mouse model successfully halted or reversed pathology. These data were used as proof-of-concept for regulatory filing enabling the commencement of an international phase 1/2 clinical trial.
Co-optimization of urban morphology and distributed energy systems is key to curb energy consumption and optimally exploit renewable energy in cities. Currently available optimization techniques focus on either buildings or energy systems, mostly neglecting the impact of their interactions, which limits the renewable energy integration and robustness of the energy infrastructure; particularly in e
Objective: To explore diagnoses of postpartum haemorrhage following vaginal birth, in relation to socio-demographic and obstetrical data from women who gave birth at term, in Sweden, during the years 2005–2015. Study design: A register-based cohort study was carried out, describing and comparing socio-demographic variables, obstetric variables and infant variables in 52 367 cases of diagnosed post
A novel sliding vane rotary compressor with a rotating cylinder is proposed in this paper. The sliding vane dynamics analysis reveals that, compared with the traditional compressor, the friction loss at the sliding vane tips and between rotor and cylinder can be reduced to 0.25% and 10.23%. To numerically investigate the internal flow mechanism, a reliable three-dimensional simulation method is de
Alterations in gut endocrine cells and hormone levels have been measured in patients with irritable bowel syndrome (IBS). The hypothesis of the present study was that hormone levels would change after 4 weeks of a starch- and sucrose-reduced diet (SSRD) intervention corresponding to decreased carbohydrate intake and symptoms. Among 105 IBS patients from primary and tertiary healthcare, 80 were ran
Malfunction and amyloid formation of the Islet Amyloid Polypeptide (IAPP) are factors contributing to Type 2 diabetes. Unravelling the mechanism of IAPP aggregate formation may forward our understanding of this process and its effect on pancreatic β-islet cell. Such mechanistic studies require access to sequence homogeneous and highly pure IAPP. Here we present a new facile protocol for the produc
Packed beds with methane steam reforming reaction (MSR) can be used as thermochemical storage devices. The packing configuration can have a significant effect on the flow distribution which will further influence the heat/mass transfer, dispersion and reaction characteristics. In the present paper, the hydraulic, dispersion, heat/mass transfer and the MSR reaction properties are investigated in pa
We investigate the origin of close-in planets and related phenomena orbiting white dwarfs (WDs), which are thought to originate from orbits more distant from the star. We use the planetary architectures of the 75 multiple-planet systems (four, five, and six planets) detected orbiting main-sequence stars to build 750 dynamically analogous templates that we evolve to the WD phase. Our exploration of
Maintaining soil health is integral to agricultural production, and the archaeological record contains multiple lines of palaeoclimatic and palaeoenvironmental proxy evidence that can contribute to the understanding and analysis of long-term trajectories of change that are key for contextualizing 21st century global environmental challenges. Soil is a capital resource and its nutrient balance is m
Background: Second primary cancers (SPCs) are increasing due to improving survival in first primary cancers. Previous studies on SPCs in renal cell carcinoma (RCC) have focused on treatment and other risk factors, but data of RCC as an SPC are scarce. Objective: In this study, we want to elucidate the risk for any SPC after RCC, and in reverse order, for RCC as an SPC after any cancer. We addition
The matched window reassigned spectrogram relocates all signal energy of an oscillating transient to the time- and frequency locations, resulting in a sharp peak in the time-frequency plane. However, previous research has shown that the method may result in split energy peaks for close components and in high noise levels, and the peak energy is then erroneously estimated. With use of novel knowled
In this work, we study under which circumstances it is appropriate to use simplified models for range determination using radar. Typically, pulsed radar systems result in the backscattered, demodulated, and matched signal having a chirp signal structure, with the frequency rate being related to the range to the reflecting target and the relative velocity of the transmitter and reflector. Far from
This work is concerned with determining optimal microphone placements that allow for an accurate location estimate of the sound sources, taking into account the expected signal structure of voiced speech, as well as the expected location areas and the typical range of the fundamental frequencies of the speakers. To determine preferable microphone placements, we propose a scheme that minimizes a th
Begonia putaoensis is reduced to a synonym under Begonia dicressine based on morphological characters and geographical location. Characters of the two species are compared and no significant difference can be established. In addition, both species are collected along the same narrow path in Kachin, northern Myanmar.
Introduction People with anterior cruciate ligament (ACL) reconstruction (ACLR) are at high risk of sustaining a graft rupture and/or contra-lateral ACL injury. The main factors that may predispose individuals for subsequent ACL injuries are, however, not established. To reduce the risk of reinjuries, it is of particular interest to identify modifiable risk-factors, for instance, those related to
Distant and predictable features in the environment make ideal compass cues to allow movement along a straight path. Ball-rolling dung beetles use a wide range of different signals in the day or night sky to steer themselves along a fixed bearing. These include the sun, the Milky Way, and the polarization pattern generated by the moon. Almost two decades of research into these remarkable creatures
Endohedral fullerenes are perfect nanolaboratories for the study of magnetism. The substitution of a diamagnetic scandium atom in Dy2ScN@C80 with gadolinium decreases the stability of a given magnetization and demonstrates Gd to act as a single atom catalyst that accelerates the reaching of thermal equilibrium. X-ray magnetic circular dichroism at the M4,5 edges of Gd and Dy shows that Gd affects
High-spin states in Xe123 were populated in the Se80(Ca48, 5n)Xe123 reaction at a beam energy of 207 MeV. γ-ray coincidence events were recorded with the Gammasphere spectrometer. Four new high-spin bands have been discovered in this nucleus. The bands are compared with those calculated within the framework of cranked Nilsson-Strutinsky and cranked Nilsson-Strutinsky-Bogoliubov models. It is concl
COVID-19 restrictions may prevent people from reaching recommended levels of physical activity (PA). This study examines self-perceived changes in the extent and intensity of PA during the COVID-19 pandemic, and the relation between perceived changes in PA and general life satisfaction and perceived physical capacity. A total of 1318 participants (mean age 47.8 SD12.6; 82.1% women) were recruited
BACKGROUND & AIMS: Liver tight junctions (TJs) establish tissue barriers that isolate bile from the blood circulation. TJP2/ZO-2-inactivating mutations cause progressive cholestatic liver disease in humans. Since the underlying mechanisms remain elusive, we characterized mice with liver-specific inactivation of Tjp2.METHODS: Tjp2 was deleted in hepatocytes, cholangiocytes, or both. Effects on
