Henk Granzier Selected Research

nebulin

1/2020	Triggering typical nemaline myopathy with compound heterozygous nebulin mutations reveals myofilament structural changes as pathomechanism.
1/2020	In vivo characterization of skeletal muscle function in nebulin-deficient mice.
9/2019	Failure to identify modifiers of NEBULIN-related nemaline myopathy in two pre-clinical models of the disease.
1/2019	Deleting nebulin's C-terminus reveals its importance to sarcomeric structure and function and is sufficient to invoke nemaline myopathy.
1/2019	Omecamtiv mecarbil lowers the contractile deficit in a mouse model of nebulin-based nemaline myopathy.
1/2017	Muscle weakness in respiratory and peripheral skeletal muscles in a mouse model for nebulin-based nemaline myopathy.
1/2015	Effect of levosimendan on the contractility of muscle fibers from nemaline myopathy patients with mutations in the nebulin gene.
6/2013	Troponin activator augments muscle force in nemaline myopathy patients with nebulin mutations.
6/2013	Deleting exon 55 from the nebulin gene induces severe muscle weakness in a mouse model for nemaline myopathy.
1/2013	Fast skeletal muscle troponin activation increases force of mouse fast skeletal muscle and ameliorates weakness due to nebulin-deficiency.

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Henk Granzier Research Topics

Disease

20	Nemaline Myopathies (Nemaline Myopathy) 01/2020 - 07/2009
17	Muscle Weakness 01/2020 - 02/2003
11	Heart Failure 01/2021 - 09/2002
9	Hypertrophy 01/2019 - 11/2003
8	Dilated Cardiomyopathy (Cardiomyopathy, Congestive) 05/2019 - 02/2002
7	Cardiomyopathies (Cardiomyopathy) 01/2022 - 09/2005
7	Muscular Diseases (Myopathy) 01/2020 - 05/2011
4	Limb-girdle muscular dystrophy type 2A 11/2019 - 11/2008
4	Atrophy 01/2018 - 02/2007
4	Heart Diseases (Heart Disease) 12/2016 - 12/2002
3	Sudden Death 01/2022 - 08/2011
3	Neoplasms (Cancer) 01/2021 - 09/2011
3	Muscular Dystrophies (Muscular Dystrophy) 04/2011 - 02/2004
2	Cardiotoxicity 01/2021 - 11/2019
2	Muscular Atrophy (Muscle Atrophy) 01/2021 - 01/2020
2	Fibrosis (Cirrhosis) 01/2021 - 09/2009
2	familial dilated cardiomyopathy 05/2019 - 02/2002
2	Cardiac Sudden Death (Sudden Cardiac Arrest) 05/2019 - 08/2011
1	Cardiac Arrhythmias (Arrythmia) 01/2022
1	Heart Injuries 01/2021
1	Minicore Myopathy with External Ophthalmoplegia 01/2020
1	Disease Progression 01/2020
1	Fatigue 01/2020
1	Rare Diseases (Rare Disease) 01/2020
1	Wounds and Injuries (Trauma) 01/2019
1	Critical Illness (Critically Ill) 01/2018
1	Diabetes Mellitus 01/2018
1	Congenital Structural Myopathies (Centronuclear Myopathy) 10/2013
1	Neuromuscular Diseases (Neuromuscular Disease) 06/2013
1	Heart Arrest (Cardiac Arrest) 02/2013
1	Ventricular Remodeling 11/2012
1	Hypertension (High Blood Pressure) 11/2012
1	Ehlers-Danlos Syndrome (Syndrome, Ehlers-Danlos) 04/2012
1	Arrhythmogenic Right Ventricular Dysplasia (Arrhythmogenic Right Ventricular Cardiomyopathy) 08/2011
1	Ischemia 11/2010
1	Reperfusion Injury 11/2010
1	Myocardial Ischemia (Ischemic Heart Diseases) 11/2010
1	Mitral Valve Insufficiency (Mitral Regurgitation) 10/2010
1	Inborn Genetic Diseases (Disease, Hereditary) 08/2010
1	Aortic Valve Stenosis (Aortic Stenosis) 08/2009
1	Desmin-Related Myofibrillar Myopathy 05/2009
1	Lymphoma (Lymphomas) 03/2007
1	Diastolic Heart Failure 02/2007

Drug/Important Bio-Agent (IBA)

36	ConnectinIBA 01/2021 - 02/2002
19	nebulinIBA 01/2020 - 07/2009
17	Proteins (Proteins, Gene)FDA Link 01/2021 - 02/2002
8	Protein Isoforms (Isoforms)IBA 01/2020 - 09/2002
3	RNA (Ribonucleic Acid)IBA 01/2022 - 10/2016
3	Myosins (Myosin)IBA 01/2020 - 11/2012
3	CalpainIBA 11/2019 - 08/2010
3	CalciumIBA 01/2015 - 02/2007
2	Messenger RNA (mRNA)IBA 01/2020 - 09/2011
2	Contractile ProteinsIBA 01/2018 - 06/2013
2	Peptide Hydrolases (Proteases)FDA Link 04/2011 - 08/2010
2	CollagenIBA 10/2010 - 01/2007
2	Crystallins (Crystallin)IBA 05/2009 - 02/2007
1	Recombinant DNA (Recombinant DNA Research)IBA 01/2022
1	PorinsIBA 01/2021
1	Doxorubicin (Adriamycin)FDA LinkGeneric 01/2021
1	Malate Dehydrogenase (Dehydrogenase, Malate)IBA 01/2021
1	Succinate Dehydrogenase (Fumarate Reductase)IBA 01/2021
1	AnthracyclinesIBA 01/2021
1	Matrix Metalloproteinase InhibitorsIBA 01/2021
1	Guanosine Monophosphate (5' Guanylic Acid)IBA 01/2020
1	Cyclic GMP-Dependent Protein Kinases (Protein Kinase G)IBA 01/2020
1	pyruvate dehydrogenase kinase 4IBA 05/2019
1	Insulin-Like Growth Factor I (IGF-1)IBA 01/2019
1	omecamtiv mecarbilIBA 01/2019
1	Glutathione Transferase (Glutathione S-Transferase)IBA 01/2019
1	LIM Domain ProteinsIBA 12/2016
1	Desoxycorticosterone Acetate (DOCA)FDA Link 10/2016
1	Simendan (Levosimendan)IBA 01/2015
1	Phosphotransferases (Kinase)IBA 07/2013
1	TroponinIBA 06/2013
1	Glycoproteins (Glycoprotein)IBA 04/2012
1	tenascin XIBA 04/2012
1	TropomyosinIBA 05/2011
1	Matrix Metalloproteinases (MMPs)IBA 11/2010
1	N- hydroxy- 5- ethoxymethyloxy- 2- methyl- 4- (4- phenoxybenzoyl)aminopentanamideIBA 11/2010
1	Matrix Metalloproteinase 2 (Gelatinase A)IBA 11/2010
1	Chymases (Chymase)IBA 10/2010
1	Fibronectins (Fibronectin)IBA 10/2010
1	Mutant Proteins (Protein, Mutant)IBA 08/2010
1	SuspensionsIBA 01/2010
1	Isoproterenol (Isoprenaline)FDA LinkGeneric 09/2009
1	Angiotensin IIIBA 09/2009
1	GelsIBA 08/2009
1	Sepharose (Agarose)IBA 08/2009
1	Cysteine Proteases (Cysteine Protease)IBA 11/2008
1	ProteomeIBA 03/2007
1	Carrier Proteins (Binding Protein)IBA 02/2007

Therapy/Procedure

3	Denervation 10/2018 - 11/2003
2	Therapeutics 06/2013 - 05/2011
1	Drug Therapy (Chemotherapy) 01/2021
1	Artificial Respiration (Mechanical Ventilation) 01/2018
1	Length of Stay 01/2018
1	Mechanical Ventilators (Ventilator) 01/2018
1	Positive-Pressure Respiration (Positive End-Expiratory Pressure) 01/2018
1	Thoracotomy 12/2011
1	Heart Transplantation (Grafting, Heart) 08/2011