Julien Ochala

Julien Ochala

Lektor

Medlem af:

    Since starting as a group leader in 2010, Julien Ochala has set up a productive and expanding research laboratory. In 2020, he has gathered a H-index of 25 with 56 peer-reviewed publications in journals such as Nature Reviews Neurology, Development, PNAS, Annals of Neurology or Acta Neuropathologica. Additionally, besides acting as external examiner for PhD theses, senior editor (Experimental Physiology and Frontiers in Physiology) or referee for scientific journals and funding bodies, he has regularly been invited to give research seminars at institutions including Institute of Myology in Paris, UCSD and University of Edinburgh.

     

    Primære forskningsområder

    In the Ochala group, the research work focuses on (i) the understanding of the cellular and molecular mechanisms causing acquired and genetic muscle diseases; and (ii) providing sufficient knowledge to identify drug targets and design novel therapeutic interventions (including exercise). For that, his laboratory uses primarily human muscle tissue but also animal samples together with a portfolio of techniques ranging from single molecule biophysics to whole muscle physiology and from X-ray diffraction to high-resolution confocal microscopy allowing deep mechanistic studies.

     

    Aktuel forskning

    Congenital myopathies: Mechanisms and Therapies
    Skeletal muscles are composed of numerous cells called muscle fibres, the basic units for generating muscle strength and, ultimately, movement. These fibres feature a large number of proteins, known as contractile proteins. In normal muscle fibres, these proteins are well-organised allowing an optimal production of force. In certain muscle disorders termed congenital myopathies, these proteins are not encoded properly and mutant molecules are then expressed. Patients with these molecular defects suffer from deleterious muscle weakness. The mechanisms by which these mutant proteins induce skeletal muscle weakness are currently not known. In the present research project, we, therefore, investigate how the mutants affect (i) protein function; (ii) the generation of the force of muscle fibres; and (iii) the consequences on the cellular architecture. Deciphering these mechanisms allows us to design effective therapeutic interventions. Here are some of our collaborators for this line of research: Prof Heinz Jungbluth (King’s), Prof Jocelyn Laporte (IGBMC), Prof Henk Granzier (University of Arizona), Prof John Vissing (University of Copenhagen), Prof Anders Oldfors (Gothenburg University), Prof Carina Wallgren-Pettersson (University of Helsinki), Prof Juan Jesus Vilchez (University of Valencia), Prof Edmar Zanoteli (University of Sao Paulo), Prof Mike Regnier (University of Washington), Dr Norma Romero (Institute of Myology), Dr Michael Lawlor (Wisconsin College of Medicine), Dr Hiroyuki Iwamoto (SPring8), and many more…
     
    Missing LINC to healthy ageing
    During the last few decades, human lifespan has tremendously been extended. However, this longer lifespan has not been associated with longer healthspan but rather with accrued morbidities and diseases. What triggers the deterioration of the healthspan remains to be studied. Based on premature ageing syndromes where lifespan and healthspan are both rapidly altered due to mutations in one particular group of proteins found in the membrane of cell nuclei (LINC complex proteins), the present project aims to precisely define whether defects in these particular proteins occur in normal ageing and what they induce across the molecular and cellular scales using animal and human skeletal muscle cells of different ages as model systems. This work is done I collaboration with: Prof Stephen Harridge (King’s), Dr Matthew Stroud (King’s), Prof Brian Kennedy (NUS Medicine), Prof Marco Narici (University of Padova), Dr Abigail Mackey (University of Copenhagen), Dr Andrew Philp (Garvan Institute), Dr Dawn Lowe (University of Minnesota), Gant Luxton (University of Minnesota), and many more…
     

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