Results 41 to 50 of about 13,148 (299)
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Objective Onasemnogene abeparvovec (OA) is an AAV9‐based gene therapy for spinal muscular atrophy type I (SMA I). Real‐world outcomes show increased response variability compared to clinical trials, and follow‐up data beyond 12–18 months are limited.
Marika Pane +43 more
wiley +1 more source
Compound Heterozygote Friedreich Ataxia Patients With Covert Proximal FXN Gene Deletions
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT We present Friedreich ataxia patients with frataxin gene deletions. Data and records were collected at the Children's Hospital of Philadelphia from patients enrolled in the FACOMS natural history study. Patients with proximal deletions initially diagnosed with only one GAA expanded allele had more severe disease than their homozygous expansion
Michael P. Lazaropoulos +5 more
wiley +1 more source
Animal, 2010 This study examined the effects of physical form and stage of maturity at harvest of whole-crop barley silage (WCBS) on feed intake, eating and rumination activity, diet selection and faecal particle size in dairy steers.
B.-O. Rustas +3 more
doaj +1 more source
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 +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 +8 more
wiley +1 more source
Animal, 2013 The study examined the effects of physical form and harvest time of alfalfa silage on eating and ruminating activity and particle size distribution in feed boli, rumen content and faeces in dry cows. The alfalfa crop was harvested at two stages of growth
L.F. Kornfelt +2 more
doaj +1 more source
Chop Chop: Byzantine Atomic Broadcast to the Network Limit
CoRR, 2023 At the heart of state machine replication, the celebrated technique enabling decentralized and secure universal computation, lies Atomic Broadcast, a fundamental communication primitive that orders, authenticates, and deduplicates messages. This paper presents Chop Chop, a Byzantine Atomic Broadcast system that uses a novel authenticated memory pool to
Martina Camaioni +5 more
openaire +3 more sources
Experimental Characterization of Mycelium‐Based Composites Under Multiple Loading Conditions
Advanced Engineering Materials, EarlyView.This study examines the mechanical response of mycelium‐based composites under compression, shear, and tension using mechanical testing and imaging methods. The comparison between unpressed and hot‐pressed specimens shows that hot pressing is associated with higher compression and shear stiffnesses.
Shaghayegh Elahi +5 more
wiley +1 more source
Advanced Functional Materials, EarlyView.Bandgap‐engineered AlGaAs/GaAs heterostructures exhibit wavelength‐selective dual‐polarity photoelectrochemistry, switching from photocathodic to photoanodic response depending on excitation wavelength. The polarity transition is governed by band‐selective absorption, built‐in electric‐field‐driven carrier transport, and interfacial charge‐transfer ...
Yukai Mao +9 more
wiley +1 more source
The Influence of Chopping Duration on the Degree of Proteins Extraction
Scientific Papers Animal Science and Biotechnologies, 2023 The quality of meat preparations, in terms of binding compositions, is directly influenced by the degree of extractibility of structural proteins, which in turn is influenced by a number of factors such as quality of raw and auxiliary materials, how ...
Daniela Ianițchi +5 more
doaj