Duchenne muscular dystrophy (DMD) is a severe degenerative muscle disease caused by mutations in the DMD gene, which encodes dystrophin. Despite its initial description in the late 19th century by French neurologist Guillaume Duchenne de Boulogne, and identification of causal DMD genetic mutations in the 1980s, therapeutics remain challenging. The current standard of care is corticosteroid treatment, which delays the progression of muscle dysfunction but is associated with significant adverse effects.
View Article and Find Full Text PDFThis paper examines the potential persistent effects (scarring) of the COVID-19 pandemic on the economy and the channels through which they may occur. Our findings from a broad set of historical recessions confirm that recessions are associated with persistent output losses and that the greatest scarring has occurred following financial crises. The amount of scarring following pandemic and epidemic recessions in the sample is in between that of typical recessions and financial crises.
View Article and Find Full Text PDFMuscle spindles are sensory organs that detect and mediate both static and dynamic muscle stretch and monitor muscle position, through a specialised cell population, termed intrafusal fibres. It is these fibres that provide a key contribution to proprioception and muscle spindle dysfunction is associated with multiple neuromuscular diseases, aging and nerve injuries. To date, there are few publications focussed on generation and characterisation of intrafusal muscle fibres To this end, current models of skeletal muscle focus on extrafusal fibres and lack an appreciation for the afferent functions of the muscle spindle.
View Article and Find Full Text PDFIntrafusal fibres are a specialised cell population in skeletal muscle, found within the muscle spindle. These fibres have a mechano-sensory capacity, forming part of the monosynaptic stretch-reflex arc, a key component responsible for proprioceptive function. Impairment of proprioception and associated dysfunction of the muscle spindle is linked with many neuromuscular diseases.
View Article and Find Full Text PDFPhilos Trans A Math Phys Eng Sci
December 2011
Uncertainty is pervasive in economic policy-making. Modern economies share similarities with other complex systems in their unpredictability. But economic systems also differ from those in the natural sciences because outcomes are affected by the state of beliefs of the systems' participants.
View Article and Find Full Text PDFThe new synthetic form of microporous crystalline silica, denoted as ITQ-12, shows a high potential for the separation of propane and propene from its mixtures.
View Article and Find Full Text PDFAngew Chem Int Ed Engl
July 1999
A novel topology, consisting of a three-dimensional system of large pore channels, is found for the zeolite ITQ-7 (see picture). This material has been synthesized by combining the structure-directing effects of fluoride ions and a new organic cation purposefully designed to promote the crystallization of a low-density silica phase. ITQ-7 is the least dense crystalline silica phase known so far.
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