Muscular dystrophies are a heterogenous group of inherited single-gene disorders, characterized clinically by progressive muscle weakness and wasting. In many cases the molecular basis of a muscular dystrophy is a disruption of the cytoskeleton - extracellular - matrix link, caused by a lack of expression of components of the dystrophin-glycoproteincomplex (DGC).
According to a current model this results in a destabilization of the entire DGC, thereby affecting the structural integrity of the muscle membrane. Duchenne muscular dystrophy (DMD), a severe X-chromosome-linked myopathy, is caused by mutations in the dystrophin gene. Nevertheless, several lines of evidence indicate that dystrophin deficiency is only the first step of a complex pathogenic cascade and much of the late pathology seems to result from ongoing cycles of muscle fiberde- and regeneration. In this respect, characterization of key control components of the de-/regeneration system would open new paths to interfere with the dystrophic process.
Further Information: PDF
Bayerische Patentallianz GmbH
Phone: +49 89 5480177-0
email@example.com | TechnologieAllianz e.V.
New Lithium Salts of Pentafluorophenylamide Anions as Electrolytes in Lithium Ionic Batteries
18.04.2017 | TechnologieAllianz e.V.
Gratings on glass surfaces
28.03.2017 | TechnologieAllianz e.V.
Thin-film solar cells made of crystalline silicon are inexpensive and achieve efficiencies of a good 14 percent. However, they could do even better if their shiny surfaces reflected less light. A team led by Prof. Christiane Becker from the Helmholtz-Zentrum Berlin (HZB) has now patented a sophisticated new solution to this problem.
"It is not enough simply to bring more light into the cell," says Christiane Becker. Such surface structures can even ultimately reduce the efficiency by...
A study in the journal Bulletin of Marine Science describes a new, blood-red species of octocoral found in Panama. The species in the genus Thesea was discovered in the threatened low-light reef environment on Hannibal Bank, 60 kilometers off mainland Pacific Panama, by researchers at the Smithsonian Tropical Research Institute in Panama (STRI) and the Centro de Investigación en Ciencias del Mar y Limnología (CIMAR) at the University of Costa Rica.
Scientists established the new species, Thesea dalioi, by comparing its physical traits, such as branch thickness and the bright red colony color, with the...
Scientists have succeeded in observing the first long-distance transfer of information in a magnetic group of materials known as antiferromagnets.
An international team of researchers has mapped Nemo's genome, providing the research community with an invaluable resource to decode the response of fish to...
Graphene is considered a promising candidate for the nanoelectronics of the future. In theory, it should allow clock rates up to a thousand times faster than today’s silicon-based electronics. Scientists from the Helmholtz Zentrum Dresden-Rossendorf (HZDR) and the University of Duisburg-Essen (UDE), in cooperation with the Max Planck Institute for Polymer Research (MPI-P), have now shown for the first time that graphene can actually convert electronic signals with frequencies in the gigahertz range – which correspond to today’s clock rates – extremely efficiently into signals with several times higher frequency. The researchers present their results in the scientific journal “Nature”.
Graphene – an ultrathin material consisting of a single layer of interlinked carbon atoms – is considered a promising candidate for the nanoelectronics of the...
03.09.2018 | Event News
27.08.2018 | Event News
17.08.2018 | Event News
18.09.2018 | Materials Sciences
18.09.2018 | Materials Sciences
18.09.2018 | Information Technology