Results 61 to 70 of about 154,658 (263)

Epilepsy‐Associated Variants of a Single SCN1A Codon Exhibit Divergent Functional Properties

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Pathogenic variants in SCN1A, which encodes the voltage‐gated sodium channel NaV1.1, are associated with multiple epilepsy syndromes exhibiting a range of clinical severity. SCN1A variants are reported in different syndromes, including Dravet syndrome, which is associated with loss‐of‐function, whereas neonatal/infantile‐onset ...
Lanie N. Liebovitz, Christopher H. Thompson, Linda C. Laux, Alfred L. George Jr.   +3 more
wiley   +1 more source

Flow Characteristics and Loss Mechanism of Tip Leakage Flow in Mining Contra-Rotating Axial Flow Fan

Applied Sciences
Tip leakage flow interacts with the mainstream, impacting the energy transmission process within the impeller of the fan and causing a significant flow loss.
Yongping Chen, Ronghua Liu, Wenqing Peng, Shiqiang Chen   +3 more
doaj   +1 more source

A Depolarizing Leak in Sodium Bicarbonate Cotransporter NBCe1 Causes Brain Edema

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objectives SLC4A4 encodes electrogenic sodium bicarbonate cotransporter NBCe1, prominently expressed in kidney and brain. Recessive loss‐of‐function variants in SLC4A4 cause proximal renal tubular acidosis, no brain edema. In the brain, NBCe1 is expressed by astrocytes, where it regulates pH and mediates astrocyte volume changes.
Quinty Bisseling, Mark D. Parker, Sven Kerst, Richard A. Pasternack, Jacob Tondreau, Marjolein Breur, Gemma M. van Rooijen‐van Leeuwen, Davide Tonduti, Ettore Salsano, Alejandra Darling, Joanna A. E. van Wijk, Susanna Törnroth‐Horsefield, Marianna Bugiani, Petra J. W. Pouwels, Quinten Waisfisz, Marjo S. van der Knaap, Rogier Min   +16 more
wiley   +1 more source

Interaction between Molten Al‐Killed Mn–B Steel and Carbon‐Bonded MgO Refractories Based on Recyclates

Advanced Engineering Materials, EarlyView.
High‐temperature interactions between low‐sulfur Al‐killed Mn–B steel and MgO–C refractories (0 and 50 wt% recyclates) are studied via finger immersion tests (1600 °C). Surface‐active elements influence infiltration. MgO/CaS layer forms, along with spinel and calcium silicate.
Matheus Roberto Bellé, Lukas Neubert, Anton Yehorov, Till M. J. Stadtmüller, Christos G. Aneziris, Olena Volkova   +5 more
wiley   +1 more source

Influence of an Argon/Silane Atmosphere on the Temperature of a Thermal Plasma

Advanced Engineering Materials, EarlyView.
The influence of a silane‐doped argon atmosphere on the chemical composition and temperature of a thermal nontransferring argon plasma is investigated using optical emission spectroscopy. As a result of the high amount of free electrons resulting from the stepwise ionization and dissociation of the silane molecule, even a silane addition of 0.01 vol ...
Lena Kreie, David Kramer, Thomas Scheithauer, Hans Jürgen Maier, Thomas Hassel   +4 more
wiley   +1 more source

Electrical Conductivities of Conductors, Semiconductors, and Their Mixtures at Elevated Temperatures

Advanced Engineering Materials, EarlyView.
This article presents a comprehensive review of temperature‐dependent electrical conductivity data for multiple material classes at elevated temperatures, highlighting a persistent conductivity gap between metals and semiconductors in the range of 102$\left(10\right)^{2}$– 107$\left(10\right)^{7}$ S/m. Metal–ceramic irregular metamaterials are proposed
Valentina Torres Nieto, Marcia A. Cooper
wiley   +1 more source

Bias‐Free Highly Efficient and Stable Dye‐Sensitized Photoelectrochemical Cells via Cascade Charge Transfer

Advanced Functional Materials, EarlyView.
A buried‐junction DSPEC design is introduced that leverages cascade charge transfer to enhance efficiency, stability, and versatility. This approach facilitates effective charge transfer and minimizes recombination losses, leading to significant improvements.
Jun‐Hyeok Park, Kyounglim Kim, Jinyoung Lee, Sarang Kim, Gwangsu Yoon, So Yeon Yoon, Jeong Kyeong Lee, Ji‐Wook Jang, Tae‐Hyuk Kwon   +8 more
wiley   +1 more source

Interference Effect of Shock Wave on Tip Leakage Vortex in a Transonic Variable Nozzle Turbine

International Journal of Turbomachinery, Propulsion and Power
The tip leakage flow at both sides of the nozzle vane is an important factor for the reduction in turbine aerothermal performance. A strong shock wave is generated at the trailing edge of the nozzle vane under transonic condition, which can interfere ...
Xinguo Lei, Qin Luo, Hanzhi Zhang
doaj   +1 more source

Ideal Molecular Sieving with a Dense MOF for Helium Upgrading with Highly Diffusion Selective Mixed Matrix Membranes

Advanced Functional Materials, EarlyView.
The separation of Helium gas from natural gas is challenging but highly important. MIL‐116(Ga), a “non‐porous” metal–organic framework is used as a molecular sieve to separate He from CH4. Druse‐like MIL‐116(Ga) particles are integrated into polysulfone mixed matrix membranes.
Ayisha Komal, Laura Calderón‐Rodríguez, Oksana Smirnova, Eren Grossmann, Aparna Binu Varghese, Karen Marlenne Garcia Alvarez, Andreas Schneemann, Thomas Hoyer, Ralf Wyrwa, Felix Helmut Schacher, Alexander Knebel   +10 more
wiley   +1 more source

Advancing Electronic Application of Coordination Solids: Enhancing Electron Transport and Device Integration via Surface‐Mounted MOFs (SURMOFs)

Advanced Functional Materials, EarlyView.
The layer‐by‐layer (LbL) assembly of coordination solids, enabled by the surface‐mounted metal‐organic framework (SURMOF) platform, is on the cusp of generating the organic counterpart of the epitaxy of inorganics. The programmable and sequential SURMOF protocol, optimized by machine learning (ML), is suited for accessing high‐quality thin films of ...
Zhengtao Xu, Christof Wöll, Stefan Bräse   +2 more
wiley   +1 more source