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Researchers from the Institute’s NMR laboratory at the SFRMBM 2023 conference

The 6th conference of the French Society of Magnetic Resonance in Biology and Medicine (SFRMBM) was held in Paris from 27 to 29 March 2023. Its objective: to present the latest methodological advances and innovative applications in magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS). The communications focused on developments in physics and technology … [Read more]

A new pathophysiological mechanism to explain asymmetry in mitochondrial myopathies

Mitochondrial diseases are a heterogeneous group of disorders caused by alterations in mitochondrial DNA (missense mutations, sporadic large-scale deletions or mutations in nuclear maintenance genes). In these rare and complex diseases, symptoms can vary from person to person and between different tissues in a individual. More than 350 genes have been identified. A team of … [Read more]

Quantitative MRI to assess the evolution of muscle condition

Clinical trials related to dystrophinopathies are increasingly using quantitative MRI to assess the evolution of muscle status in patients with these neuromuscular diseases. In this prospective study, researchers from the NMR laboratory (NIC, Institute of Myology) and a clinician (Reference Centre for MNM Paris-Est, Institute of Myology) aimed to establish the sensitivity of the quantification … [Read more]

A new clinical trial on DM1 at the Institute of Myology

The double-blind, placebo-controlled phase I/II ACHIEVE trial is evaluating DYNE-101. This drug candidate for Steinert’s disease is being developed by the company Dyne Therapeutics. It is an antisense oligonucleotide coupled to an antibody fragment targeting the hTfR1 receptor (present in large quantities on the surface of muscle cells) administered by intravenous infusion. The Institute of … [Read more]

The effectiveness of therapies in DMD depends on the territorial distribution of dystrophin

The distribution of dystrophin in the muscle fibre is not uniform. It is dependent on the cell nuclei, thus creating small functional units at the sarcolemmal level. Researchers from the University of Versailles Saint-Quentin-en-Yvelines, with the contribution of Genethon and the Institute of Myology, have carried out experiments in transgenic mice which have shown a … [Read more]

Standards now available for muscle fibre size and type during growth

The evolution of muscle fibre morphometry during growth is poorly understood. French researchers and engineers involving scientists from the Institute of Myology have used a new image analysis algorithm (Carpaccio.Cloud) to better measure the evolution of several quantitative parameters over time: 482 images, corresponding to 83 biopsied deltoid muscles and representing a total of 33,094 … [Read more]

The induction of self-tolerance is back on the agenda in myasthenia gravis

According to preclinical work by Spanish researchers, carried out with the help of Rozen Le Panse’s team at the Institute of Myology : intraperitoneal injection of acetylcholine receptors (RACh) encapsulated in liposomes rich in phosphatidylserine decreases the anti-RACh antibody level and improves physical performance (Global clinical score or GCS) in an myasthenia gravis experimental mouse … [Read more]

Electrophysiological studies are useful in differentiating CMS

Researchers from the Institute of Myology analysed electroneuromyography data from 120 patients with congenital myasthenic syndromes (CMS) and compared them with the results of genetic studies: five distinct profiles could be established from the measurement of CMAPs and repetitive stimulation of three nerves (accessory nerve, radial nerve, deep fibular nerve) at a frequency of 3 … [Read more]

Myasthenia gravis: the role of thymic macrophages is essential

Myasthenia gravis (MG) is a neuromuscular disease related to the deleterious action of autoantibodies directed against elements of the neuromuscular junction, most often against the acetylcholine receptor (AChR) which is located in the postsynaptic region. The thymus plays a key role in this pathology (AChR-MG) and is characterised by an IFN-β-related type I interferon (IFN) … [Read more]

Anti-fibrotic drug therapies in skeletal muscle diseases

Fibrosis is defined as an excessive accumulation of extracellular matrix. It can affect many organs, including the lungs, liver, heart, skin, kidneys and muscles. Muscle fibrosis is the result of a defective regeneration process, unresolved inflammation or chronic damage. Many muscle diseases have a combination of high levels of fibrosis and muscle wasting and weakness. … [Read more]