Results 81 to 90 of about 206,047 (258)

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

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

Mechanochemical Synthesis and Characterization of Nanostructured ErB4 and NdB4 Rare‐Earth Tetraborides

Advanced Engineering Materials, Volume 27, Issue 6, March 2025.
ErB4 and NdB4 nanostructured powders are produced by mechanochemical synthesis. 5 h mechanical alloying and 4 M HCl acid leaching are used in the production. ErB4 and NdB4 powders exhibit maximum magnetization of 0.4726 emu g−1 accompanied with an antiferromagnetic‐to‐paramagnetic phase transition at about TN = 18 K and 0.132 emu g−1 with a maximum at ...
Burçak Boztemur, Faruk Kaya, Bora Derin, Mustafa Lütfi Öveçoğlu, Ju Li, Duygu Ağaoğulları   +5 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

S-Benzyl-L-cysteine Inhibits Growth and Photosynthesis, and Triggers Oxidative Stress in Ipomoea grandifolia

Agronomy
L-cysteine, a precursor of essential components for plant growth, is synthesized by the cysteine synthase complex, which includes O-acetylserine(thiol) lyase (OAS-TL) and serine acetyltransferase.
Danielly Caroline Inacio Martarello, Luiz Henryque Escher Grizza, Marcela de Paiva Foletto-Felipe, Ana Paula da Silva Mendonça, Renato Polimeni Constantin, Ana Paula Ferro, Wanderley Dantas dos Santos, Rodrigo Polimeni Constantin, Rogerio Marchiosi, Osvaldo Ferrarese-Filho   +9 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

Role of Plastocyanin in response to photosynthetic electron transport in Solanum nigrum under Cd stress

Plant Stress
Plastocyanin (PC), an essential electron carrier located between the cytochrome b6f (cytb6f) complex and PSI in photosynthetic electron transport, remains inadequately understood regarding its regulatory role in electron transfer under heavy metal stress.
Jiaxuan Xiang, Ziqiang Fu, Hongyang Xu, Xinhao Huang, Fan Zhu   +4 more
doaj   +1 more source

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

Low‐Angle Grain Boundaries and Re‐Segregation in Single‐Crystalline Ni‐Base Superalloys

Advanced Engineering Materials, EarlyView.
This work demonstrates that Re‐segregation at low‐angle grain boundaries (LAGBs) in Ni‐base superalloys is influenced by misorientation angle. Advanced microscopy and atom probe tomography reveal that higher misorientation angles increases Re‐segregation.
Alireza B. Parsa, Aparna Saksena, Alesander Kostka, Pascal Thome, Felicitas Scholz, Alexandre Mussi, Christoph Somsen, Baptiste Gault, Jan Frenzel, Gunther Eggeler   +9 more
wiley   +1 more source

Field Report from Collaborative Research Center 1625: Heterogeneous Research Data Management Using Ontology Representations

Advanced Engineering Materials, EarlyView.
A unified research data management framework for heterogeneous materials data is presented. The system integrates multimodal datasets using ontologies and knowledge graphs, enabling interoperability and FAIR (findable, accessible, interoperable, reusable) data principles. By linking data across scales and workflows, it supports reproducible, Artifitial
Doaa Mohamed, Samuel García Vázquez, Behnam Mardani, Victor Dudarev, Alfred Ludwig, Maribel Acosta, Markus Stricker   +6 more
wiley   +1 more source