Results 61 to 70 of about 3,903 (211)

Unraveling A4GALT Mechanism and Its Modulation With Adamantyl‐Galactosylceramide Analogues: Advancing Fabry Disease Therapeutic Strategies

Angewandte Chemie, EarlyView.
A 310‐helix‐mediated conformational switch promotes a front‐face SNi‐like catalysis by human A4GALT. Mechanism‐guided design identifies AdaGalCer as a selective modulator of globotriaosylceramide (Gb3) biosynthesis, opening a clear route toward new Fabry disease therapeutics.
Nicky de Koster, Òscar Vidal‐Gironès, Rowan de Graaf, Ken Kok, Lindsey T. Lelieveld, Jackelien Hoogervorst, Maria J. Ferraz, José Pablo Rivas‐Fernández, Rob F. Lammers, Herman S. Overkleeft, Johannes M. F. G. Aerts, Rolf G. Boot, Carme Rovira, Marta Artola   +13 more
wiley   +2 more sources

Extreme‐Thickness Meta‐Membrane for Controlling Terahertz Vectorial Beams

Advanced Science, EarlyView.
We propose ultra‐thin (λ/30) transmissive terahertz metasurfaces combining near‐field resonant and detour phases in coupled‐slit meta‐atoms, enabling continuous phase and polarization control beyond conventional designs. Based on leaky‐mode theory, scalar and vectorial terahertz Bessel beams at 0.39 THz are experimentally demonstrated, with ...
Yufei Song, Yixiang Xu, Yikai Xu, Yuanxi Liu, Shaojie Ma, Qiong He, Zhuo Wang, Lei Zhou   +7 more
wiley   +1 more source

Quantum statistics of light transmitted through an intracavity Rydberg medium

New Journal of Physics, 2014
We theoretically investigate the quantum statistical properties of light transmitted through an atomic medium with strong optical nonlinearity induced by Rydberg–Rydberg van der Waals interactions.
A Grankin, E Brion, E Bimbard, R Boddeda, I Usmani, A Ourjoumtsev, P Grangier   +6 more
doaj   +1 more source

Stress Accumulation Induced by Ion Exchange for Synchronous Modulation of Mode and Wavelength in Microlasers

Advanced Science, EarlyView.
This work demonstrates a paradigm‐shifting strategy to simultaneously achieve broadband wavelength tunability and single‐mode control within a single perovskite microwire structure. Using a facile heating‐assisted solid–solid anion‐exchange strategy, we synthesized phase‐pure CsPbClxBr3‐x microwires that exhibit the widest reported continuous lasing ...
Bingwang Yang, Lingling Sun, Jinhui Wang, Jitao Li, Peng Wan, Daning Shi, Caixia Kan, Mingming Jiang   +7 more
wiley   +1 more source

Patient-reported experience with Fabry disease and its management in the real-world setting: results from a double-blind, cross-sectional survey of 280 respondents

Orphanet Journal of Rare Diseases
Background Fabry disease (FD) is a rare X-linked lysosomal storage disorder with a heterogeneous clinical presentation. Patients with FD may exhibit early signs/symptoms including neuropathic pain, gastrointestinal complaints, and dermatologic ...
Lisa Berry, Jerry Walter, Jack Johnson, Julia Alton, Janet Powers, Xavier Llòria, Irene Koulinska, Meghan McGee, Dawn Laney   +8 more
doaj   +1 more source

Ultracold atoms in disordered potentials: elastic scattering time in the strong scattering regime

New Journal of Physics, 2019
We study the elastic scattering time ${\tau }_{{\rm{s}}}$ of ultracold atoms propagating in optical disordered potentials in the strong scattering regime, going beyond the recent work of Richard et al (2019 Phys. Rev. Lett. 122 100403).
Adrien Signoles, Baptiste Lecoutre, Jérémie Richard, Lih-King Lim, Vincent Denechaud, Valentin V Volchkov, Vasiliki Angelopoulou, Fred Jendrzejewski, Alain Aspect, Laurent Sanchez-Palencia, Vincent Josse   +10 more
doaj   +1 more source

Super‐Resolution and High‐Data‐Density Acoustic Meta‐Hologram via Amplitude and Phase Coupling

Advanced Science, EarlyView.
A super‐resolution acoustic meta‐hologram utilizes amplitude‐phase coupling under energy conservation constraints to overcome the traditional diffraction limit. This conceptually advanced approach achieves high‐fidelity acoustic field reconstruction and dense information transmission, unlocking new potentials for high‐resolution acoustic imaging and ...
Xiao Guo, Xinzong Wang, Guoshen Tang, Haohan Zeng, Siqi Fan, Xinghao Hu, Youyu Mo, Zhenyu He, Tingting Li, Hui Xu, Jiao Shen, Haiyan Fan, Xiaoxiang Gao, Yifan Zhu, Hui Zhang, Badreddine Assouar   +15 more
wiley   +1 more source

Efficient Polarization‐Entangled Photon‐Pair Generation by a Fiber‐In‐Line van der Waals Material

Advanced Science, EarlyView.
Telecom‐wavelength polarization‐entangled photon pairs are generated in a van der Waals‐material‐integrated all‐fiber device via spontaneous parametric down‐conversion. The demonstrated fiberized quantum light source bridges emerging 2D nonlinear materials and practical quantum photonic systems, opening new opportunities for scalable fiber‐based ...
Jungseok Choi, Seongju Ha, Seungjae Lim, Joohyeon Ahn, Jaekyoung Kim, Jong Hyuk Yim, Nam Hun Park, Youngdong Yoo, Jae‐Ung Lee, Hee Su Park, Sang Min Lee, Dong‐Il Yeom   +11 more
wiley   +1 more source

In Vitro and In Vivo Amenability to Migalastat in Fabry Disease

Molecular Therapy: Methods & Clinical Development, 2020
Migalastat (1-deoxygalactonojirimycin) is approved for the treatment of Fabry disease (FD) in patients with an amenable mutation. Currently, there are at least 367 amenable and 711 non-amenable mutations known, based on an in vitro good laboratory ...
Malte Lenders, Franciska Stappers, Eva Brand   +2 more
doaj   +1 more source

Physics‐Aware Machine‐Learning‐Driven Inverse Design of Broadband Ultra‐Open Acoustic Metamaterials

Advanced Science, EarlyView.
A physics‐aware machine‐learning framework enables inverse design of ultra‐open acoustic silencers by decoupling spectral and radial design spaces. The approach rapidly identifies broadband, compact, and highly ventilated architectures, while revealing hidden linear design rules that link geometry, impedance matching, and acoustic performance.
Zhiwei Yang, Mengyu Li, Xiaohang Xie, Ao Chen, Thomas G. Bifano, Xin Zhang   +5 more
wiley   +1 more source