Results 41 to 50 of about 17,793 (302)

Onasemnogene Abeparvovec in Type I Spinal Muscular Atrophy: 24‐Month Follow‐Up From the Italian Registry

open access: yesAnnals 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

Mesin Chopper untuk Meningkatkan Kualitas dan Kuantitas Pakan Usaha Ternak Kambing “Arrahman”

open access: yesWarta LPM
The "Arrahman" partner is located in Kerten, Krincing Village, Secang District, Magelang manages around 200 to 300 goats. The process of preparing cattle fodder such as elephant grass and corn stalks is done manually using a knife.
Rany Puspita Dewi   +2 more
doaj   +1 more source

Design, Construction and Evaluation of a Silage Corn Chopper Equipped with Electromotor Power Unit [PDF]

open access: yesJournal of Agricultural Machinery, 2018
Introduction In most small and medium-size traditional animal farms, silage corn is chopped manually. In order to prepare appropriate chopper for small animal production firms, a small electrical powered chopper was designed and developed. Materials and
R Hadipiour Rokni   +2 more
doaj   +1 more source

A low-offset low-noise sigma-delta modulator with pseudorandom chopper-stabilization technique

open access: yes, 2011
This paper presents a low-offset low-noise sigma-delta modulator with pseudorandom chopper-stabilization technique. The comparison of the noise-cancellation ability with the correlated double sampling and chopper-stabilization techniques is demonstrated;
Chen, Hsin-Liang; Chen, Po-Sheng; Chiang, Jen-Shiun   +1 more
core   +1 more source

Compound Heterozygote Friedreich Ataxia Patients With Covert Proximal FXN Gene Deletions

open access: yesAnnals 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

A 250 °C Low-Power, Low-Temperature-Drift Offset Chopper-Stabilized Operational Amplifier with an SC Notch Filter for High-Temperature Applications

open access: yesApplied Sciences
This paper proposes a three-stage op amp based on the SOI (silicon-on-insulator) process, which achieves a low offset voltage and temperature coefficient across a wide temperature range from −40 °C to 250 °C.
Zhong Yang   +5 more
doaj   +1 more source

A 17 Year Old With Developmental Delay Presenting With Increasing Confusion and Imbalance

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Methylmalonic acidemia is an autosomal recessive genetic disorder primarily caused by defects in methylmalonyl‐CoA mutase and cobalamin (vitamin B12) metabolism. These defects disrupt the tricarboxylic acid cycle and oxidative phosphorylation, leading to the abnormal accumulation of metabolic products such as methylmalonic acid, propionic acid,
Wei Zhao, Yingli Zhang, Hongliang Zheng
wiley   +1 more source

Chopper-Stabilized Instrumentation Amplifier with Automatic Frequency Tuning Loop

open access: yesMicromachines, 2018
A variable-gain chopper-stabilized instrumentation amplifier (chopper IA), which employs a low pass filter (LPF) to attenuate the up-converted noise at the chopping frequency, is presented. The circuit is designed and fabricated with Taiwan Semiconductor
Chen-Mao Wu   +3 more
doaj   +1 more source

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

open access: yesAnnals 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

AC-Chopper-Based Inrush Current Suppressor in a Wind Power Generation System with Squirrel-Cage Induction Machines

open access: yesEnergies, 2018
This paper proposes the inrush current suppressor using an AC chopper in a large-capacity wind power generation system (WPGS) with two squirrel-cage induction machines (SCIMs), which are switched over depending on the wind speed. The input side of the AC
Sho Shibata   +3 more
doaj   +1 more source

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