Results 91 to 100 of about 588,596 (269)

Onasemnogene Abeparvovec in Patients With SMA: Interim Results of the RESTORE Registry in Japan

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective There are limited real‐world data regarding the safety and effectiveness of onasemnogene abeparvovec (OA; Zolgensma) infusion, a one‐time gene replacement therapy, for Japanese patients with spinal muscular atrophy (SMA). We aimed to improve understanding of the real‐world outcomes for OA in Japan.
Kayoko Saito, Kamal Benguerba, Ken Tsuchida, Kazushige Yazawa, Isao Tsumiyama, Hiromitsu Kayama, Sandra P. Reyna, Farid Khan, Richard S. Finkel   +8 more
wiley   +1 more source

White Matter Microstructural Abnormalities in Neonatal Onset Genetic Epilepsy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Recent evidence indicates that epilepsy is associated with abnormal white matter. If seizures alter white matter, then the impact upon network function, epileptogenesis, and cognition could be pronounced in neonates undergoing rapid developmental myelination. Neonates with epilepsy due to nonstructural genetic causes provide a unique
Amanda G. Sandoval Karamian, Tianjia Zhu, Hao Huang, Shavonne L. Massey, Mark P. Fitzgerald, Darshana Parikh, Arastoo Vossough, Ingo Helbig, Nicholas S. Abend   +8 more
wiley   +1 more source

Research Review on Inhibitors of Lumpy Skin Disease Virus: From Biological Characteristics, Drug Repurposing, to Computer-Aided Drug Design

International Journal of Microbiology
This review summarizes the research progress on inhibitors of lumpy skin disease virus (LSDV), focusing on its biological characteristics and key proteins involved in the viral replication cycle.
Leilei ZHAO, Jing QU, Miao AN, Yang LIU, Xiubo LI, Yiming LIU, Chen PENG   +6 more
doaj   +1 more source

Prominent Movement Disorders in RNU2‐2‐Related Spliceosomopathy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Pediatric movement disorders often overlap with neurodevelopmental diseases, suggesting shared molecular mechanisms. Variants in small nuclear RNA (snRNA) genes encoding spliceosome components have recently been associated with neurodevelopmental disorders, termed “RNUopathies.” We analyzed genome sequencing data from 14 patients with ...
Magdalena Krygier, Ugo Sorrentino, Matias Wagner, Marta Zawadzka, Anna Lemska, Maria Mazurkiewicz‐Bełdzińska, Michael Zech   +6 more
wiley   +1 more source

Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs, Marcel Naumann, Annaliis Lehto, Hanna Schön, Jens Kurth, Johannes Prudlo, Andreas Hermann, Elisabeth Kasper   +7 more
wiley   +1 more source

What Do Large Language Models Know About Materials?

Advanced Engineering Materials, EarlyView.
If large language models (LLMs) are to be used inside the material discovery and engineering process, they must be benchmarked for the accurateness of intrinsic material knowledge. The current work introduces 1) a reasoning process through the processing–structure–property–performance chain and 2) a tool for benchmarking knowledge of LLMs concerning ...
Adrian Ehrenhofer, Thomas Wallmersperger, Gianaurelio Cuniberti   +2 more
wiley   +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

Milk Disposition Kinetics, Residue and Efficacy of Rifaximin After Intramammary Administration in Lactating Cow

Antibiotics
Background: Rifaximin is a non-aminoglycoside antibiotic utilized for the treatment of mastitis in cows, but its milk disposition kinetics, residue, and bacteriological status in lactating cow milk have hardly been reported.
Na Yu, Yaoxin Tang, Weifeng Zhao, Junhao Xiang, Jing Qu, Hao Wu, Yiming Liu   +6 more
doaj   +1 more source

A Knowledge‐Based Approach for Understanding and Managing Additive Manufacturing Data

Advanced Engineering Materials, EarlyView.
Additive manufacturing processes generate a large amount of data. Effectively managing, understanding, and retrieving information from this data remains a major challenge. Therefore, we propose an ontology‐based approach to integrate heterogeneous data, enable semantic queries, and support decision‐making.
Mina Abd Nikooie Pour, Prithwish Tarafder, Anton Wiberg, Huanyu Li, Johan Moverare, Patrick Lambrix   +5 more
wiley   +1 more source

Understanding the Stochastic Nature of Process Parameter Development of Blown Powder Laser Beam Directed Energy Deposition Additive Manufacturing of Pure Molybdenum

Advanced Engineering Materials, EarlyView.
Identified through the use of statistical design of experiments and metallographic investigation, this study exposes the stochastic origins of intergranular cracks in blown powder laser beam directed energy deposition additive manufacturing of pure molybdenum. It further demonstrates a successful crack mitigation approach with direct correlation to the
Nathaniel J. Lies, Daniel E. Driemeyer, Aaron P. Stebner   +2 more
wiley   +1 more source