ICaR-VU
Principal Investigator
Specialization
Role of myofilament proteins in striated muscle function
Name
Coen Ottenheijm, PhD (30-05-1976)
Current position
Assistent Professor of Physiology
Bio sketch
Key Publications (5)
Prizes, awards, grants
The unifying theme of Dr. Ottenheijm’s research concerns the
regulatory pathogenic role of myofilament proteins in striated muscle
contraction.
Dr. Ottenheijm received his doctorate at the dept of Pulmonology at
the Radboud University Nijmegen Medical Center in 2006, where he
investigated the contribution of myofilament dysfunction to diaphragm
weakness in patients with Chronic Obstructive Pulmonary Disease.
His PhD research prompted Dr. Ottenheijm to pursue a postdoctoral
position in the lab of Dr. Henk Granzier at the University of Arizona
(funded by a NWO Rubicon grant), where he focussed on the role of
the giant myofilament proteins titin (the largest protein known to date)
and nebulin in muscle function in health and disease. Currently, Dr
Ottenheijm is working at the dept of Physiology at VUmc (supported
by a NWO VENI grant) and is prinicipal investigator in a collaborative
EU (FP7) funded study investigating novel transgenic mouse models
of nebulin-based nemaline myopathy, and in a Prinses Beatrix
Foundation funded study investigating the role of myofilament
dysfunction in muscular dystrophies. Furthermore, in collaboration
with the depts of Intensive Care Medicine, Surgery and
Anesthesiology at VUmc, Dr Ottenheijm’s research group focusses
on the pathogenesis of diaphragm weakness in conditions associated
with altered diaphragm activity, such as pulmonary hypertension and
mechanical ventilation.
1. Ottenheijm CAC et al. Changes in cross-bridge cycling underlie
muscle weakness in patients with tropomyosin 3-based
myopathy. Hum Mol Genet. 2011 May 15;20(10):2015-25.
2. Welvaart WN et al. & Ottenheijm CAC. Selective diaphragm
muscle weakness following contractile inactivity during thoracic
surgery. Annals of Surgery, 2011. In Press
3. FS de Man et al. & Ottenheijm CAC. Diaphragm muscle
weakness in Pulmonary Arterial Hypertension. Am J Respir Crit
Care Med; 2011. In Press.
4. Ottenheijm CAC and Granzier H. Lifting the nebula: novel
insights into skeletal muscle contractility. Physiology; 2010
Oct;25(5):304-10.
5. Ottenheijm CAC et al. Altered myofilament function depresses
force generation in patients with nebulin-based nemaline
myopathy (NEM2). 2009. J Struct Biol; 2010 May;170(2):334-43.
2011-2015
2011-2015
2011-2012
2010-2013
Prinsess Beatrix Foundation. The Netherlands €250.000
Title: Myofilament-based muscle weakness in
Facioscapulohumeral dystrophy.
Institute for Cardiovascular Research (ICAR-VU,
Netherlands). €250.000
Title: Why do septic patients die from mechanical ventilation?
The role of diaphragm weakness.
Foundation for Building Strength for
Nemaline Myopathies, USA $98.000
Title: Novel mechanisms for muscle weakness in Nemaline
Myopathy.
EU GRANT, 7TH FRAMEWORK PROGRAMME. €434.189
Title: Functional characterization of Nemaline: Myopathy in a
murine model for nebulin mutation: Moving from basic
2008-2011
2006-2008
understanding toward therapeutic interventions. European
Consortium with Dr. S Labeit (EMBL, Heidelberg) and Dr D.
Bendahan (Universitee de la Mediterannee, Marseille).
VENI grant (career development) from NWO (Dutch
Organization for Scientific Research). €208.000
Title: Nebulous Nebulin: expedition to the role of a giant
protein in muscle function and disease.
RUBICON grant (career development) from NWO (Dutch
Organization for Scientific Research). €70.000
Title: Muscle contraction: the role of nebulin.
Hirsch index (1-1-2011)
8
Main contributions to
congresses, memberships of
scientific societies (selection)
Member of the American Thoracic Society and the Biophysical
Society
Selected international activities

Member Editorial Board of the International Journal of Physiology,
Pharmcology and Pathophysiology.

PI in EU seventh framework program 2010: Myopathy in a murine
model for nebulin mutations: Moving from basic understanding
toward therapeutic interventions.

Appointment as Research Assistant Professor at the dept of
Physiology at the University of Arizona, USA.