Julien Ochala

Summary

Affiliation: Uppsala University
Country: Sweden

Publications

  1. ncbi Nemaline myopathy-related skeletal muscle α-actin (ACTA1) mutation, Asp286Gly, prevents proper strong myosin binding and triggers muscle weakness
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Sweden
    PLoS ONE 7:e45923. 2012
  2. ncbi Congenital myopathy-causing tropomyosin mutations induce thin filament dysfunction via distinct physiological mechanisms
    Julien Ochala
    Department of Neuroscience, Uppsala University, Uppsala, Sweden
    Hum Mol Genet 21:4473-85. 2012
  3. ncbi Maintenance of muscle mass, fiber size, and contractile function in mice lacking the Z-disc protein myotilin
    Julien Ochala
    Department of Clinical Neurophysiology, Uppsala University Hospital, Sweden
    Ups J Med Sci 114:235-41. 2009
  4. ncbi Disrupted myosin cross-bridge cycling kinetics triggers muscle weakness in nebulin-related myopathy
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University Hospital, Entrance 85, 3rd Floor, SE 751 85 Uppsala, Sweden
    FASEB J 25:1903-13. 2011
  5. ncbi Diaphragm muscle weakness in an experimental porcine intensive care unit model
    Julien Ochala
    Department of Neuroscience, Uppsala University, Uppsala, Sweden
    PLoS ONE 6:e20558. 2011
  6. ncbi EMD 57033 partially reverses ventilator-induced diaphragm muscle fibre calcium desensitisation
    Julien Ochala
    Department of Clinical Neurophysiology, Uppsala University Hospital, Uppsala, Sweden
    Pflugers Arch 459:475-83. 2010
  7. ncbi A myopathy-linked tropomyosin mutation severely alters thin filament conformational changes during activation
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Uppsala, Sweden
    Proc Natl Acad Sci U S A 107:9807-12. 2010
  8. ncbi Factors underlying the early limb muscle weakness in acute quadriplegic myopathy using an experimental ICU porcine model
    Julien Ochala
    Department of Neuroscience, Uppsala University, Uppsala, Sweden
    PLoS ONE 6:e20876. 2011
  9. ncbi Mechanisms underlying the sparing of masticatory versus limb muscle function in an experimental critical illness model
    Sudhakar Aare
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Uppsala, Sweden
    Physiol Genomics 43:1334-50. 2011
  10. ncbi Effects of a preferential myosin loss on Ca2+ activation of force generation in single human skeletal muscle fibres
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, University Hospital, Uppsala, Sweden
    Exp Physiol 93:486-95. 2008

Collaborators

Detail Information

Publications18

  1. ncbi Nemaline myopathy-related skeletal muscle α-actin (ACTA1) mutation, Asp286Gly, prevents proper strong myosin binding and triggers muscle weakness
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Sweden
    PLoS ONE 7:e45923. 2012
    ..To counterbalance such negative events, future potential therapeutic strategies may focus on the inappropriate actin-actin interface or myosin binding...
  2. ncbi Congenital myopathy-causing tropomyosin mutations induce thin filament dysfunction via distinct physiological mechanisms
    Julien Ochala
    Department of Neuroscience, Uppsala University, Uppsala, Sweden
    Hum Mol Genet 21:4473-85. 2012
    ..Therefore, administration of troponin activators may constitute a promising therapeutic approach in the future...
  3. ncbi Maintenance of muscle mass, fiber size, and contractile function in mice lacking the Z-disc protein myotilin
    Julien Ochala
    Department of Clinical Neurophysiology, Uppsala University Hospital, Sweden
    Ups J Med Sci 114:235-41. 2009
    ..Myotilin location and interactions with other Z-disc proteins are clearly defined, but its role in the regulation of muscle structure and function remains unknown. The present study aims at investigating this specific role of myotilin...
  4. ncbi Disrupted myosin cross-bridge cycling kinetics triggers muscle weakness in nebulin-related myopathy
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University Hospital, Entrance 85, 3rd Floor, SE 751 85 Uppsala, Sweden
    FASEB J 25:1903-13. 2011
    ..To reverse such a negative cascade of events, future potential therapeutic interventions should, therefore, focus on the triggering component, the altered myosin cross-bridge cycling kinetics...
  5. ncbi Diaphragm muscle weakness in an experimental porcine intensive care unit model
    Julien Ochala
    Department of Neuroscience, Uppsala University, Uppsala, Sweden
    PLoS ONE 6:e20558. 2011
    ..Unexpectedly, sepsis, CS or NMBA have no significant additive effects, suggesting that mechanical ventilation and sedation are the triggering factors leading to diaphragm weakness in the intensive care unit...
  6. ncbi EMD 57033 partially reverses ventilator-induced diaphragm muscle fibre calcium desensitisation
    Julien Ochala
    Department of Clinical Neurophysiology, Uppsala University Hospital, Uppsala, Sweden
    Pflugers Arch 459:475-83. 2010
    ..Thus, EMD 57033 partly restored the Ca(2+) activation of cells, reducing VIDD. This finding offers a strong basis for evaluating the effect of Ca(2+) sensitisers on diaphragm function in vivo...
  7. ncbi A myopathy-linked tropomyosin mutation severely alters thin filament conformational changes during activation
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Uppsala, Sweden
    Proc Natl Acad Sci U S A 107:9807-12. 2010
    ....
  8. ncbi Factors underlying the early limb muscle weakness in acute quadriplegic myopathy using an experimental ICU porcine model
    Julien Ochala
    Department of Neuroscience, Uppsala University, Uppsala, Sweden
    PLoS ONE 6:e20876. 2011
    ..These results suggest that the rapid declines in CMAP amplitude and in force generation capacity are triggered by independent mechanisms with significant clinical and therapeutic implications...
  9. ncbi Mechanisms underlying the sparing of masticatory versus limb muscle function in an experimental critical illness model
    Sudhakar Aare
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Uppsala, Sweden
    Physiol Genomics 43:1334-50. 2011
    ..spinal nerve innervated muscles during exposure to the ICU intervention...
  10. ncbi Effects of a preferential myosin loss on Ca2+ activation of force generation in single human skeletal muscle fibres
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, University Hospital, Uppsala, Sweden
    Exp Physiol 93:486-95. 2008
    ..Thus, a change in Ca(2+) activation of the contractile apparatus in patients with preferential myosin loss is proposed as an additional factor contributing to the muscle function impairment in these patients...
  11. ncbi Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres
    Julien Ochala
    Department of Clinical Neurophysiology, Uppsala University Hospital, Entrance 85, 3rd Floor, SE 751 85 Uppsala, Sweden
    J Physiol 581:1283-92. 2007
    ....
  12. ncbi A myopathy-related actin mutation increases contractile function
    Johan Lindqvist
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Sweden
    Acta Neuropathol 123:739-46. 2012
    ..Overall, at the cell level, these divergent mechanisms conduct to an improved steady-state force production. Such results provide new surprising scientific insights and crucial information for future therapeutic strategies...
  13. ncbi Preferential skeletal muscle myosin loss in response to mechanical silencing in a novel rat intensive care unit model: underlying mechanisms
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Uppsala, Sweden
    J Physiol 589:2007-26. 2011
    ....
  14. ncbi Defective regulation of contractile function in muscle fibres carrying an E41K beta-tropomyosin mutation
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, University Hospital, Entrance 85, 3rd Floor, SE 751 85 Uppsala, Sweden
    J Physiol 586:2993-3004. 2008
    ....
  15. ncbi Ca2+ sensitizers: An emerging class of agents for counterbalancing weakness in skeletal muscle diseases?
    Julien Ochala
    Department of Clinical Neurophysiology, Uppsala University Hospital, Sweden
    Neuromuscul Disord 20:98-101. 2010
    ..Therefore, in the future, under certain conditions, these agents may represent an emerging class of agents to enhance the quality of life of patients suffering from skeletal muscle weakness...
  16. ncbi Gene expression and muscle fiber function in a porcine ICU model
    Varuna C Banduseela
    Department of Clinical Neurophysiology, Uppsala University Hospital, SE 751 85 Uppsala, Sweden
    Physiol Genomics 39:141-59. 2009
    ..To our knowledge, this is the first study that isolates the effect of immobilization and mechanical ventilation in an ICU condition from various other cofactors...
  17. ncbi Thin filament proteins mutations associated with skeletal myopathies: defective regulation of muscle contraction
    Julien Ochala
    Department of Neuroscience, Clinical Neurophysiology, University Hospital, Entrance 85, SE 751 85, Uppsala, Sweden
    J Mol Med (Berl) 86:1197-204. 2008
    ..With an enhanced understanding of the cellular and molecular mechanisms underlying muscle weakness in patients carrying such mutations, better therapy strategies can be developed to improve the quality of life...
  18. ncbi Sparing of muscle mass and function by passive loading in an experimental intensive care unit model
    Guillaume Renaud
    Department of Neuroscience, Clinical Neurophysiology, University Hospital, Entrance 85, 3rd Floor, SE 751 85 Uppsala, Sweden
    J Physiol 591:1385-402. 2013
    ....