Troponin activator augments muscle force in nemaline myopathy patients with nebulin mutations

Safety, tolerability and pharmacodynamics of a skeletal muscle activator in amyotrophic lateral sclerosis

(2012) Jeremy Shefner et al.   Amyotrophic Lateral Sclerosis

Congenital myopathy-causing tropomyosin mutations induce thin filament dysfunction via distinct physiological mechanisms

(2012) J. Ochala et al.   HUMAN MOLECULAR GENETICS

Activation of fast skeletal muscle troponin as a potential therapeutic approach for treating neuromuscular diseases

(2012) Alan J Russell et al.   NATURE MEDICINE

The Calcium Sensitizer Levosimendan Improves Human Diaphragm Function

(2011) Jonne Doorduin et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Disrupted myosin cross-bridge cycling kinetics triggers muscle weakness in nebulin-related myopathy

(2011) Julien Ochala et al.   FASEB JOURNAL

Novel mutations in NEB cause abnormal nebulin expression and markedly impaired muscle force generation in severe nemaline myopathy

(2011) Michael W Lawlor et al.   Skeletal Muscle

Dominant Mutations in KBTBD13, a Member of the BTB/Kelch Family, Cause Nemaline Myopathy with Cores

(2010) Nyamkhishig Sambuughin et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Nebulin regulates actin filament lengths by a stabilization mechanism

(2010) Christopher T. Pappas et al.   JOURNAL OF CELL BIOLOGY

Lifting the Nebula: Novel Insights into Skeletal Muscle Contractility

(2010) Coen A. C. Ottenheijm et al.   PHYSIOLOGY

A Nebulin Ruler Does Not Dictate Thin Filament Lengths

(2009) Angelica Castillo et al.   BIOPHYSICAL JOURNAL

Nebulin plays a direct role in promoting strong actin-myosin interactions

(2009) Marie-Louise Bang et al.   FASEB JOURNAL

Thin filament length dysregulation contributes to muscle weakness in nemaline myopathy patients with nebulin deficiency

(2009) C. A.C. Ottenheijm et al.   HUMAN MOLECULAR GENETICS

Nebulin Alters Cross-bridge Cycling Kinetics and Increases Thin Filament Activation

(2009) Murali Chandra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Altered myofilament function depresses force generation in patients with nebulin-based nemaline myopathy (NEM2)

(2009) Coen A.C. Ottenheijm et al.   JOURNAL OF STRUCTURAL BIOLOGY

Levosimendan Enhances Force Generation of Diaphragm Muscle from Patients with Chronic Obstructive Pulmonary Disease

(2008) Hieronymus W. H. van Hees et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Defective regulation of contractile function in muscle fibres carrying an E41K β-tropomyosin mutation

(2008) Julien Ochala et al.   JOURNAL OF PHYSIOLOGY-LONDON