Nebulin, a multi-functional giant

Roles of Nebulin Family Members in the Heart

(2015) Marie-Louise Bang et al.   CIRCULATION JOURNAL

Leiomodin-3-deficient mice display nemaline myopathy with fast-myofiber atrophy

(2015) L. Tian et al.   Disease Models & Mechanisms

Severe myopathy in mice lacking the MEF2/SRF-dependent gene leiomodin-3

(2015) Bercin K. Cenik et al.   JOURNAL OF CLINICAL INVESTIGATION

Knockout of Lmod2 results in shorter thin filaments followed by dilated cardiomyopathy and juvenile lethality

(2015) Christopher T. Pappas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

How Leiomodin and Tropomodulin use a common fold for different actin assembly functions

(2015) Malgorzata Boczkowska et al.   Nature Communications

LMOD3: the “missing link” in nemaline myopathy?

(2015) Sarah Sandaradura et al.   Oncotarget

Effect of levosimendan on the contractility of muscle fibers from nemaline myopathy patients with mutations in the nebulin gene

(2015) Josine M de Winter et al.   Skeletal Muscle

Mutation Update: The Spectra of Nebulin Variants and Associated Myopathies

(2014) Vilma-Lotta Lehtokari et al.   HUMAN MUTATION

Leiomodin 3 and tropomodulin 4 have overlapping functions during skeletal myofibrillogenesis

(2014) C. U. Nworu et al.   JOURNAL OF CELL SCIENCE

KLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathy

(2014) Ankit Garg et al.   JOURNAL OF CLINICAL INVESTIGATION

Leiomodin-3 dysfunction results in thin filament disorganization and nemaline myopathy

(2014) Michaela Yuen et al.   JOURNAL OF CLINICAL INVESTIGATION

The nebulin SH3 domain is dispensable for normal skeletal muscle structure but is required for effective active load bearing in mouse

(2013) D. L. Yamamoto et al.   JOURNAL OF CELL SCIENCE

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

(2013) Josine Marieke de Winter et al.   JOURNAL OF MEDICAL GENETICS

Identification of Xin-repeat proteins as novel ligands of the SH3 domains of nebulin and nebulette and analysis of their interaction during myofibril formation and remodeling

(2013) Stefan Eulitz et al.   MOLECULAR BIOLOGY OF THE CELL

A two-segment model for thin filament architecture in skeletal muscle

(2013) David S. Gokhin et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

In vivo and in vitro investigations of heterozygous nebulin knock-out mice disclose a mild skeletal muscle phenotype

(2013) C. Gineste et al.   NEUROMUSCULAR DISORDERS

Expression of multiple nebulin isoforms in human skeletal muscle and brain

(2012) Jenni Laitila et al.   MUSCLE & NERVE

Thin-filament length correlates with fiber type in human skeletal muscle

(2011) David S. Gokhin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

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

(2011) Julien Ochala et al.   FASEB JOURNAL

The desmin coil 1B mutation K190A impairs nebulin Z-disc assembly and destabilizes actin thin filaments

(2011) G. M. Conover et al.   JOURNAL OF CELL SCIENCE

Nemaline Myopathies

(2011) Carina Wallgren-Pettersson et al.   Seminars in Pediatric Neurology

Nebulin, a major player in muscle health and disease

(2010) Siegfried Labeit et al.   FASEB JOURNAL

Isolation of Nebulin from Rabbit Skeletal Muscle and Its Interaction with Actin

(2010) Ryo Chitose et al.   JOURNAL OF BIOMEDICINE AND BIOTECHNOLOGY

Nebulin regulates actin filament lengths by a stabilization mechanism

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

Leiomodin-2 is an antagonist of tropomodulin-1 at the pointed end of the thin filaments in cardiac muscle

(2010) T. Tsukada et al.   JOURNAL OF CELL SCIENCE

Reduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle

(2010) P. Tonino et al.   JOURNAL OF CELL SCIENCE

Differential splicing of the large sarcomeric protein nebulin during skeletal muscle development

(2010) Danielle Buck et al.   JOURNAL OF STRUCTURAL BIOLOGY

A nucleator arms race: cellular control of actin assembly

(2010) Kenneth G. Campellone et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Lifting the Nebula: Novel Insights into Skeletal Muscle Contractility

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

Nebulin and N-WASP Cooperate to Cause IGF-1-Induced Sarcomeric Actin Filament Formation

(2010) K. Takano et al.   SCIENCE

The Nebulin family: an actin support group

(2010) Christopher T. Pappas et al.   TRENDS IN CELL BIOLOGY

Reduced thin filament length in nebulin-knockout skeletal muscle alters isometric contractile properties

(2009) David S. Gokhin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL 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

The vertebrate muscle Z-disc: sarcomere anchor for structure and signalling

(2009) Pradeep K. Luther   JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY

Nanomechanics of Full-Length Nebulin: An Elastic Strain Gauge in the Skeletal Muscle Sarcomere

(2009) Vamsi K. Yadavalli et al.   LANGMUIR

CORE-ROD MYOPATHY CAUSED BY MUTATIONS IN THE NEBULIN GENE

(2009) N. B. Romero et al.   NEUROLOGY

Sarcoplasmic reticulum calcium uptake and speed of relaxation are depressed in nebulin-free skeletal muscle

(2008) Coen A. C. Ottenheijm et al.   FASEB JOURNAL

Nebulin Interacts with CapZ and Regulates Thin Filament Architecture within the Z-Disc

(2008) Christopher T. Pappas et al.   MOLECULAR BIOLOGY OF THE CELL

A Myopathy-linked Desmin Mutation Perturbs Striated Muscle Actin Filament Architecture

(2008) Gloria M. Conover et al.   MOLECULAR BIOLOGY OF THE CELL