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Institut de la Myologie

Newsletter #63

Cell migration and evaluation of physical activity

Welcome to our summer issue of the Newsletter!

In this edition, two interviews highlight promising results from research teams at the Institute of Myology on both basic and clinical research aspects: the mechanisms of cell migration and improved evaluation of physical activity of patients. Stéphane Vassilopoulos describes the identification of new cell structures used by the cells as hooks to attach to other structures in order to move around. In our second interview, Jean-Yves Hogrel discusses the results of a pilot study that included 5 inflammatory myopathies patients who wore wrist accelerometers to record the global movement of the patients throughout the day. The results are encouraging, and this very promising pilot study will now be extended to a larger patient cohort.

Save the date! Telethon 2017 will take place over the weekend of December 8th and 9th 2017. Zazie, a french singer, will be the ambassador along with four families. Please visit the 31st Telethon dedicated website (in french) for further details.

Until our next issue in September, we wish you all an enjoyable and safe summer!

At the Institute
A novel mechanism of cell migration linked to clathrin/AP-2 tubes Stéphane Vassilopoulos (INSERM Researcher at the Myology Research Center at the Institute of Myology) participated in the discovery of a new mode of adhesion and movement of the cell in a three-dimensional collagen matrix. Read more

Evaluation of physical activity by accelerometry: a promising measure in patients with neuromuscular pathology Clinicians and researchers at the Institute have just published an article concerning the use of accelerometers to assess the impact of a treatment by measuring the daily physical activity of patients with idiopathic inflammatory myopathies (IIM). Jean-Yves Hogrel (Director of the Laboratory of Physiology and Neuromuscular Evaluation) discusses the results of this pilot study. Read more

The Summer School of Myology: 20 years later! In 20 years, the Summer School of Myology has trained more than 850 physicians from 5 continents and representing 70 nations. These physicians and researchers form today an international network of myology experts. Read more

Our latest news
DM1: reducing CTG repeats The CRISPR/Cas9 system for eliminating abnormal CTG repeats in DM1 is effective in cellular models. Read more

The 1st European consensus for starting and stopping ERT in adult patients with Pompe disease A systematic review of the literature was undertaken to determine the effectiveness of ERT in adult patients on a range of clinical outcome measures and quality of life. Read more

Higher risk of device-related complications after cardioverter defibrillator implantations in DM1 patients This study compared the rate of device-related complications between pacemaker (23 DM1 patients) and implantable cardioverter defibrillators (46 matched controls) implantation. Read more

Wide spectrum of clinical severity in affected female carriers of MTM1 mutations The analysis of a large cohort of 43 cases highlights a wide spectrum of clinical severity ranging from severe neonatal and generalized weakness, similar to XLMTM male, to milder adult forms. Read more

More breaking news
SMA: European marketing authorisation for Spinraza™ (nusinersen) Spinraza™ (nusinersen), the first and only treatment for spinal muscular atrophy (SMA), is now available in Europe. Read more

Establishing a standardised corticosteroid treatment for DMD The authors are conducting a clinical trial that compares the 3 most frequently prescribed corticosteroid regimes, standardises treatment of DMD complications and standardises prevention of corticosteroid side effects. Read more

Spinal muscular atrophy
Duchenne muscular dystrophy
Other neuromuscular diseases and basic research
In brief
September 2017: Myology to be taught in the 3rd year of the Bachelor’s degree in Sciences for Health A Myology teaching unit will be launched in September at the Université Paris Descartes (Paris V) in the 3rd year the Bachelor’s degree in Sciences for Health. Read more

Upcoming ENMC workshop Next workshop "Clinical trial readiness for Calpainopathies" will take place from 15 to 17 September 2017. Read more

TGDOC Update - SMA registry, initial guidance available TGDOC chairs have produced an initial guidance document which describes the workshop and provides the list of recommended data items. Read more

Job offer The Tapscott Lab (Seattles, USA) has an opening for a postdoctoral research associate who has interest and expertise in gene transcription in a chromatin context in normal development and disease. Read more

Latest Publications from the Institute
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Every two months, the newsletter from the Institute of Myology informed you on developments in myology research, with a summary of the latest scientifics news, medical, political and community about neuromuscular diseases. You can read our newsletter by subscribing. You can unsubscribe here.
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This is the newsletter of current medical science from the Institute of Myology. It is published every two months. Chief Editor: Marie-Pierre Verrier Editorial Board: Marianne Perreau-Saussine; J. Andoni Urtizberea. Redaction: Racquel N. Cooper; Anne Berthomier. Also participate: Nathalie Haslin; Hala Alameddine; Delphine Alibert. Do you have any questions? Would you like to share some news? Please contact us. © 2017 - AFM - Institut de Myologie. ISSN 1772-9866