Results 131 to 140 of about 7,750,015 (291)

Early Clinical, Imaging, and Pathological Characteristics of SRPK3/TTN‐Digenic Myopathy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective SRPK3/TTN‐digenic myopathy was recently established as a skeletal muscle myopathy caused by digenic inheritance. This study characterizes the early clinical presentation of SRPK3/TTN‐digenic myopathy in one previously reported and seven newly identified pediatric patients.
Rotem Orbach, Sandra Donkervoort, Carola Hedberg‐Oldfors, Giovanni Baranello, Dimah Saade, Precilla D'Souza, Ruchee Patel, Eva Michael, A. Reghan Foley, Diana Bharucha‐Goebel, S. Jin Haugland, Meghan McAnally, Omer Abdul Hamid, Katherine Chao, Ellen F. Macnamara, Alan H. Beggs, Anna Sarkozy, Juliane Mueller, Steven A. Moore, Richard S. Finkel, Cynthia J. Tifft, Francesco Muntoni, Anders Oldfors, Carsten G. Bönnemann   +23 more
wiley   +1 more source

Highly Regioselective 1,3-Dipolar Cycloaddition of Nitrilimines and Thioaurones Towards Spiro-2-Pyrazolines: Synthesis, Characterization, and Mechanistic Study

Reactions
In this paper, we report a highly regioselective 1,3-dipolar cycloaddition (1,3-DC) reaction of nitrilimines with thioaurone derivatives that afforded the hitherto unreported spiropyrazolines. Spectroscopic and spectrometric data were utilized to confirm
Mohamed Bakhouch, Bouchra Es-Sounni, Ayoub Ouaddi, Khaoula Oudghiri, Mohammed Chalkha, Lahoucine Bahsis, Taoufiq Benali, Mohamed Bourass, Rabiaa Fdil, Mohamed Akhazzane, Mohamed El Yazidi   +10 more
doaj   +1 more source

Plasma EV Proteomics Identifies ECM Remodeling and Inflammatory Proteins LUM and C7 as Candidate Biomarkers in FSHD

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Facioscapulohumeral muscular dystrophy (FSHD) is one of the most debilitating and common muscular dystrophies. Despite its severity, no approved therapy exists for FSHD patients. However, several therapeutic candidates are currently under development, and some have recently entered clinical trials, marking the need for reliable ...
Mustafa Bilal Bayazit, Chiranth K. Nagaraj, Jackson S. Newell, Kim Truc Nguyen, Xilal Y. Rima, Jacob Doon‐Ralls, Eduardo Reátegui, Jeffrey M. Statland, Rabi Tawil, Kevin M. Flanigan, Scott Q. Harper, Nizar Y. Saad   +11 more
wiley   +1 more source

A Workflow to Accelerate Microstructure‐Sensitive Fatigue Life Predictions

Advanced Engineering Materials, EarlyView.
This study introduces a workflow to accelerate predictions of microstructure‐sensitive fatigue life. Results from frameworks with varying levels of simplification are benchmarked against published reference results. The analysis reveals a trade‐off between accuracy and model complexity, offering researchers a practical guide for selecting the optimal ...
Luca Loiodice, Krzysztof S. Stopka, Michael D. Sangid   +2 more
wiley   +1 more source

First-principles study of electrolyte components stability and orientation on MoS2 surfaces: implications for SEI formation in sodium-ion batteries

Frontiers in Batteries and Electrochemistry
The interfacial stability between electrolyte components and electrode materials plays a crucial role in the formation of the solid electrolyte interphase (SEI) in sodium-ion batteries.
Luis A. Selis, Luis A. Selis, Utkarsh Vijay, Diego E. Galvez-Aranda   +3 more
doaj   +1 more source

Microstructural Evolution and Vacancy Defect Formation in Mn–Mo–Ni RPV Steel Under Low Cycle Fatigue: Insights From EBSD and PALS

Advanced Engineering Materials, EarlyView.
Low‐cycle fatigue damage in Mn–Mo–Ni reactor pressure vessel steel is examined using a combined electron backscatter diffraction and positron annihilation lifetime spectroscopy approach. The study correlates texture evolution, dislocation substructure development, and vacancy‐type defect formation across uniform, necked, and fracture regions, providing
Apu Sarkar, Dhanadeep Dutta, G. Bharat Reddy   +2 more
wiley   +1 more source

Are diurnal variations in glymphatic clearance driven by circadian regulation of Aquaporin-4 expression?

Fluids and Barriers of the CNS
Aquaporin-4 (AQP4) is a key astrocytic water channel protein predominantly localized to perivascular endfeet, where it is believed to play a crucial role in brain fluid homeostasis and glymphatic waste clearance.
Mina Martine Frey, Negar Zohoorian, Nadia Skauli, Ole Petter Ottersen, Mahmood Amiry-Moghaddam   +4 more
doaj   +1 more source

Localization of Electronic Wave Functions on Quasiperiodic Lattices

, 1998
We study electronic eigenstates on quasiperiodic lattices using a tight-binding Hamiltonian in the vertex model. In particular, the two-dimensional Penrose tiling and the three-dimensional icosahedral Ammann-Kramer tiling are considered. Our main interest concerns the decay form and the self-similarity of the electronic wave functions, which we compute
Rieth, Thomas, Grimm, Uwe, Schreiber, Michael   +2 more
openaire   +2 more sources

Thermodynamic Pathways of Nonequilibrium Solidification in Wire‐Arc Additive Manufacturing Fe‐Based Multicomponent Alloy Structures

Advanced Engineering Materials, EarlyView.
Geometry‐driven thermal behavior in wire‐arc additive manufacturing (WAAM) influences microstructural evolution during nonequilibrium solidification of a chemically complex Fe–Cr–Nb–W–Mo–C nanocomposite system. By comparing different deposits configurations, distinct entropy–cooling rate correlations, segregation, and carbide evolution are revealed ...
Blanca Palacios, Anthony Gonzalez, Aleksander Aleman, Tyler Dolmetsch, Tanaji Paul, Arvind Agarwal   +5 more
wiley   +1 more source

Modeling of the General Trends of Reactivity and Regioselectivity in Cyclopentadiene–Nitroalkene Diels–Alder Reactions

Molecules
This study presents a theoretical investigation of the electronic properties of mono- and pentasubstituted cyclopentadiene analogs and variously substituted conjugated nitroalkenes bearing electron-donating and electron-withdrawing groups.
Adrianna Fałowska, Stanisław Grzybowski, Daniel Kapuściński, Karol Sambora, Agnieszka Łapczuk   +4 more
doaj   +1 more source