List of works - H.L. Granzier

A new congenital multicore titinopathy associated with fast myosin heavy chain deficiency

scientific article published on 19 April 2020

Altered contractility of skeletal muscle in mice deficient in titin's M-band region

scientific article

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

scientific article

Altered titin expression, myocardial stiffness, and left ventricular function in patients with dilated cardiomyopathy

scientific article

Cardiac titin: an adjustable multi-functional spring

scientific article

Cardiac titin: structure, functions and role in disease

scientific article published on 7 July 2006

Changes in cross-bridge cycling underlie muscle weakness in patients with tropomyosin 3-based myopathy.

scientific article

Deleting exon 55 from the nebulin gene induces severe muscle weakness in a mouse model for nemaline myopathy

scientific article published on 28 May 2013

Different molecular mechanics displayed by titin's constitutively and differentially expressed tandem Ig segments.

scientific article

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

scientific article

Dimerization of the cardiac ankyrin protein CARP: implications for MARP titin-based signaling

scientific article

Downsizing the molecular spring of the giant protein titin reveals that skeletal muscle titin determines passive stiffness and drives longitudinal hypertrophy

article

Dynamic distribution of muscle-specific calpain in mice has a key role in physical-stress adaptation and is impaired in muscular dystrophy.

scientific article

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

scientific article published on 28 April 2015

Experimentally increasing titin compliance in a novel mouse model attenuates the Frank-Starling mechanism but has a beneficial effect on diastole.

scientific article

Expressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function

scientific article published on 28 January 2020

Expression of distinct classes of titin isoforms in striated and smooth muscles by alternative splicing, and their conserved interaction with filamins

scientific article published in August 2006

Fast Skeletal Muscle Troponin Activation Increases Force of Mouse Fast Skeletal Muscle and Ameliorates Weakness Due to Nebulin-Deficiency ⬇️

scientific article published on February 20, 2013

Functional Characterization of the Intact Diaphragm in a Nebulin-Based Nemaline Myopathy (NM) Model-Effects of the Fast Skeletal Muscle Troponin Activator tirasemtiv

scientific article published on 10 October 2019

Genetic variation in titin in arrhythmogenic right ventricular cardiomyopathy-overlap syndromes

scientific article

KBTBD13 is an actin-binding protein that modulates muscle kinetics

scientific article published on 01 February 2020

Late-life restoration of mitochondrial function reverses cardiac dysfunction in old mice

scientific article published on 10 July 2020

Lifting the Nebula: Novel Insights into Skeletal Muscle Contractility ⬇️

scientific article published on October 1, 2010

Mechanics on myocardium deficient in the N2B region of titin: the cardiac-unique spring element improves efficiency of the cardiac cycle

scientific article

Metformin improves diastolic function in an HFpEF-like mouse model by increasing titin compliance

scientific article published on 19 December 2018

MuRF1 is a muscle fiber-type II associated factor and together with MuRF2 regulates type-II fiber trophicity and maintenance.

scientific article

Muscle weakness in respiratory and peripheral skeletal muscles in a mouse model for nebulin-based nemaline myopathy

scientific article published on 25 October 2016

Mutation-specific effects on thin filament length in thin filament myopathy

scientific article published on 13 April 2016

Nebulin alters cross-bridge cycling kinetics and increases thin filament activation: a novel mechanism for increasing tension and reducing tension cost

scientific article published on 07 September 2009

Nebulin and Lmod2 are critical for specifying thin-filament length in skeletal muscle

scientific article published on 11 November 2020

Nebulin and titin modulate cross-bridge cycling and length-dependent calcium sensitivity

scientific article published on 04 April 2019

Nebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity: novel insights in nemaline myopathy

scientific article

Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo

scientific article

Nebulin, a major player in muscle health and disease

scientific article

New insights into the structural roles of nebulin in skeletal muscle

scientific article

Non-proteolytic functions of calpain-3 in sarcoplasmic reticulum in skeletal muscles.

scientific article

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

scientific article published on June 20, 2011

Novel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscle

scientific journal article

Omecamtiv mecarbil lowers the contractile deficit in a mouse model of nebulin-based nemaline myopathy

scientific article published on 13 November 2019

Phosphorylating Titin's Cardiac N2B Element by ERK2 or CaMKIIδ Lowers the Single Molecule and Cardiac Muscle Force

scientific article

Progress on the regulation of myofibrillar function: Part 2

scientific article published on 15 April 2019

Protein changes contributing to right ventricular cardiomyocyte diastolic dysfunction in pulmonary arterial hypertension

scientific article

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

scientific article

Right ventricular diastolic impairment in patients with pulmonary arterial hypertension

scientific article published on 20 September 2013

Role of Titin in Skeletal Muscle Function and Disease ⬇️

scientific article published on January 1, 2010

Sarcomere length-dependent effects on Ca2+-troponin regulation in myocardium expressing compliant titin

scientific article published on 06 December 2018

Sarcomeric dysfunction contributes to muscle weakness in facioscapulohumeral muscular dystrophy

scientific article

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

scientific article published on 23 April 2008

Selective Diaphragm Muscle Weakness After Contractile Inactivity During Thoracic Surgery

scientific article published on 01 December 2011

Shortening of the elastic tandem immunoglobulin segment of titin leads to diastolic dysfunction

scientific article published on 24 May 2013

Single molecule force spectroscopy on titin implicates immunoglobulin domain stability as a cardiac disease mechanism.

scientific article

The giant protein titin: a major player in myocardial mechanics, signaling, and disease

scientific article published on February 2004

The multifunctional Ca(2+)/calmodulin-dependent protein kinase II delta (CaMKIIδ) phosphorylates cardiac titin's spring elements.

scientific article published on 5 December 2012

The sarcomeric protein nebulin: another multifunctional giant in charge of muscle strength optimization

scientific article published on 27 February 2012

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

scientific article published on 04 April 2009

Titin and diaphragm dysfunction in chronic obstructive pulmonary disease

scientific article published on 09 December 2005

Titin and diaphragm dysfunction in mechanically ventilated rats ⬇️

scientific article published on February 11, 2012

Titin and its associated proteins: the third myofilament system of the sarcomere

scientific article

Titin as a modular spring: emerging mechanisms for elasticity control by titin in cardiac physiology and pathophysiology

scientific article

Titin mutations and muscle disease

scientific article published on 27 March 2019

Titin-actin interaction: PEVK-actin-based viscosity in a large animal

scientific article published on 15 November 2011

Titin-based mechanosensing and signaling: role in diaphragm atrophy during unloading? ⬇️

scientific article published on November 12, 2010

Titin-based stiffening of muscle fibers in Ehlers-Danlos Syndrome

scientific article published on 05 January 2012

Titin: an endosarcomeric protein that modulates myocardial stiffness in DCM.

scientific article

Titin: physiological function and role in cardiomyopathy and failure

scientific article

Triggering typical nemaline myopathy with compound heterozygous nebulin mutations reveals myofilament structural changes as pathomechanism

scientific article published on 01 June 2020

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

scientific article

Tuning passive mechanics through differential splicing of titin during skeletal muscle development

scientific article published on October 2009

List of works by H.L. Granzier

A new congenital multicore titinopathy associated with fast myosin heavy chain deficiency

Altered contractility of skeletal muscle in mice deficient in titin's M-band region

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

Altered titin expression, myocardial stiffness, and left ventricular function in patients with dilated cardiomyopathy

Cardiac titin: an adjustable multi-functional spring

Cardiac titin: structure, functions and role in disease

Changes in cross-bridge cycling underlie muscle weakness in patients with tropomyosin 3-based myopathy.

Deleting exon 55 from the nebulin gene induces severe muscle weakness in a mouse model for nemaline myopathy

Different molecular mechanics displayed by titin's constitutively and differentially expressed tandem Ig segments.

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

Dimerization of the cardiac ankyrin protein CARP: implications for MARP titin-based signaling

Downsizing the molecular spring of the giant protein titin reveals that skeletal muscle titin determines passive stiffness and drives longitudinal hypertrophy

Dynamic distribution of muscle-specific calpain in mice has a key role in physical-stress adaptation and is impaired in muscular dystrophy.

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

Experimentally increasing titin compliance in a novel mouse model attenuates the Frank-Starling mechanism but has a beneficial effect on diastole.

Expressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function

Expression of distinct classes of titin isoforms in striated and smooth muscles by alternative splicing, and their conserved interaction with filamins

Fast Skeletal Muscle Troponin Activation Increases Force of Mouse Fast Skeletal Muscle and Ameliorates Weakness Due to Nebulin-Deficiency ⬇️

Functional Characterization of the Intact Diaphragm in a Nebulin-Based Nemaline Myopathy (NM) Model-Effects of the Fast Skeletal Muscle Troponin Activator tirasemtiv

Genetic variation in titin in arrhythmogenic right ventricular cardiomyopathy-overlap syndromes

KBTBD13 is an actin-binding protein that modulates muscle kinetics

Late-life restoration of mitochondrial function reverses cardiac dysfunction in old mice

Lifting the Nebula: Novel Insights into Skeletal Muscle Contractility ⬇️

Mechanics on myocardium deficient in the N2B region of titin: the cardiac-unique spring element improves efficiency of the cardiac cycle

Metformin improves diastolic function in an HFpEF-like mouse model by increasing titin compliance

MuRF1 is a muscle fiber-type II associated factor and together with MuRF2 regulates type-II fiber trophicity and maintenance.

Muscle weakness in respiratory and peripheral skeletal muscles in a mouse model for nebulin-based nemaline myopathy

Mutation-specific effects on thin filament length in thin filament myopathy

Nebulin alters cross-bridge cycling kinetics and increases thin filament activation: a novel mechanism for increasing tension and reducing tension cost

Nebulin and Lmod2 are critical for specifying thin-filament length in skeletal muscle

Nebulin and titin modulate cross-bridge cycling and length-dependent calcium sensitivity

Nebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity: novel insights in nemaline myopathy

Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo

Nebulin, a major player in muscle health and disease

New insights into the structural roles of nebulin in skeletal muscle

Non-proteolytic functions of calpain-3 in sarcoplasmic reticulum in skeletal muscles.

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

Novel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscle

Omecamtiv mecarbil lowers the contractile deficit in a mouse model of nebulin-based nemaline myopathy

Phosphorylating Titin's Cardiac N2B Element by ERK2 or CaMKIIδ Lowers the Single Molecule and Cardiac Muscle Force

Progress on the regulation of myofibrillar function: Part 2

Protein changes contributing to right ventricular cardiomyocyte diastolic dysfunction in pulmonary arterial hypertension

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

Right ventricular diastolic impairment in patients with pulmonary arterial hypertension

Role of Titin in Skeletal Muscle Function and Disease ⬇️

Sarcomere length-dependent effects on Ca2+-troponin regulation in myocardium expressing compliant titin

Sarcomeric dysfunction contributes to muscle weakness in facioscapulohumeral muscular dystrophy

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

Selective Diaphragm Muscle Weakness After Contractile Inactivity During Thoracic Surgery

Shortening of the elastic tandem immunoglobulin segment of titin leads to diastolic dysfunction

Single molecule force spectroscopy on titin implicates immunoglobulin domain stability as a cardiac disease mechanism.

The giant protein titin: a major player in myocardial mechanics, signaling, and disease

The multifunctional Ca(2+)/calmodulin-dependent protein kinase II delta (CaMKIIδ) phosphorylates cardiac titin's spring elements.

The sarcomeric protein nebulin: another multifunctional giant in charge of muscle strength optimization

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

Titin and diaphragm dysfunction in chronic obstructive pulmonary disease

Titin and diaphragm dysfunction in mechanically ventilated rats ⬇️

Titin and its associated proteins: the third myofilament system of the sarcomere

Titin as a modular spring: emerging mechanisms for elasticity control by titin in cardiac physiology and pathophysiology

Titin mutations and muscle disease

Titin-actin interaction: PEVK-actin-based viscosity in a large animal

Titin-based mechanosensing and signaling: role in diaphragm atrophy during unloading? ⬇️

Titin-based stiffening of muscle fibers in Ehlers-Danlos Syndrome

Titin: an endosarcomeric protein that modulates myocardial stiffness in DCM.

Titin: physiological function and role in cardiomyopathy and failure

Triggering typical nemaline myopathy with compound heterozygous nebulin mutations reveals myofilament structural changes as pathomechanism

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

Tuning passive mechanics through differential splicing of titin during skeletal muscle development