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Importance of Timely Treatment Initiation in Infantile-Onset Pompe Disease, a Single-Centre Experience
(MDPI, 2021-11-09)
Abstract
Classic infantile Pompe disease (IPD) is a rare lysosomal storage disorder characterized by severe hypertrophic cardiomyopathy and profound muscle weakness. Without treatment, death occurs within the first 2 years ...
Slow Folding of a Helical Protein: Large Barriers, Strong Internal Friction, or a Shallow, Bumpy Landscape?
(American Chemical Society, 2020-10-15)
The rate at which a protein molecule folds is determined by opposing energetic and entropic contributions to the free energy that shape the folding landscape. Delineating the extent to which they impact the diffusional ...
Reflecting Laser-Driven Shocks in Diamond in the Megabar Pressure Range
(Chinese Laser Press and Cambridge University Press, 2021-01-08)
In this work we present experimental results on the behavior of diamond at megabar pressure. The experiment was performed using the PHELIX facility at GSI in Germany to launch a planar shock into solid multi-layered diamond ...
The stochastic self-consistent harmonic approximation: calculating vibrational properties of materials with full quantum and anharmonic effects
(IOP Publishing Ltd, 2021-07-13)
[EN] The efficient and accurate calculation of how ionic quantum and thermal fluctuations impact
the free energy of a crystal, its atomic structure, and phonon spectrum is one of the main
challenges of solid state physics, ...
Graphene Oxide–Silver Nanoparticle Nanohybrids: Synthesis, Characterization, and Antimicrobial Properties
(MDPÎ, 2020-02-21)
Drug resistance of pathogenic microorganisms has become a global public health problem, which has prompted the development of new materials with antimicrobial properties. In this context, antimicrobial nanohybrids are an ...
Effect of Organic Modifier and Clay Content on Non-Isothermal Cold Crystallization and Melting Behavior of Polylactide/Organovermiculite Nanocomposites
(MDPI, 2020-02-07)
In clay/polymer nanocomposites, the crystallization behavior and kinetics of the polymer can be affected by the presence of clay, its content and the degree of miscibility between the clay and the polymer matrix. The effect ...
Selective ultrasensitive optical fiber nanosensors based on plasmon resonance energy transfer
(American Chemical Society, 2020-04-03)
The facet of optical fibers coated with nanostructures enable the development of ultraminiature and sensitive (bio)chemical sensors. The reported sensors until now lack of specificity and the fabrication methods offer poor ...
Weak or Strong Sustainability in Rural Land Use Planning? Assessing Two Case Studies through Multi-Criteria Analysis
(MDPI, 2020-03-19)
This paper addresses the debate regarding weak versus strong sustainability in the field of rural land use planning. Both concepts correspond to opposing paradigms on sustainability and both their fundamentals of economic ...
Refractive-Index Profile Reconstruction in Graded-Index Polymer Optical Fibers Using Raman Spectroscopy
(MDPI, 2020-05-14)
This work reports a novel method to create a 3D map of the refractive index of different graded-index polymer optical fibers (GI-POF), measuring the Raman spectra at different points of their transverse sections. Raman ...
Foreword Special Issue Cell Cycle and Regulation
(MDPI, 2020-02-27)
The process of cell division is critical to the growth and development of an organism. As a fertilized egg develops into a mature organism, tissues undergo cellular renewal or commit to terminal differentiation and leave ...