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  1. Article ; Online: Super relaxed myosins loosen up to different cues in cardiac and skeletal muscle sarcomeres.

    Claassen, Wout J / Ottenheijm, Coen A C

    The Journal of general physiology

    2023  Volume 155, Issue 7

    MeSH term(s) Sarcomeres ; Cues ; Myosins ; Muscle, Skeletal/physiology ; Heart
    Chemical Substances Myosins (EC 3.6.4.1)
    Language English
    Publishing date 2023-05-16
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Comment
    ZDB-ID 3118-5
    ISSN 1540-7748 ; 0022-1295
    ISSN (online) 1540-7748
    ISSN 0022-1295
    DOI 10.1085/jgp.202213292
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Book ; Thesis: Diaphragm dysfunction in COPD

    Ottenheijm, Coen Adri Charles

    involvement of the myofilaments

    2006  

    Author's details door Coen Adri Charles Ottenheijm
    Language English
    Size 152 S. : Ill., graph. Darst.
    Publishing country Netherlands
    Document type Book ; Thesis
    Thesis / German Habilitation thesis Nijmegen, Univ., Diss., 2006
    HBZ-ID HT016844203
    ISBN 90-9020471-7 ; 978-90-9020471-0
    Database Catalogue ZB MED Medicine, Health

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    Shelf markLoan statusLocation
    2011 E 2425 order for loan Magazin

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  3. Article ; Online: Titin N2A: More than a signaling node?

    van der Pijl, Robbert J / Ottenheijm, Coen A C

    The Journal of general physiology

    2021  Volume 153, Issue 7

    MeSH term(s) Connectin ; Muscle Proteins ; Sarcomeres
    Chemical Substances Connectin ; Muscle Proteins
    Language English
    Publishing date 2021-06-03
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Comment
    ZDB-ID 3118-5
    ISSN 1540-7748 ; 0022-1295
    ISSN (online) 1540-7748
    ISSN 0022-1295
    DOI 10.1085/jgp.202112904
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  4. Article ; Online: Nebulin: big protein with big responsibilities.

    Yuen, Michaela / Ottenheijm, Coen A C

    Journal of muscle research and cell motility

    2020  Volume 41, Issue 1, Page(s) 103–124

    Abstract: Nebulin, encoded by NEB, is a giant skeletal muscle protein of about 6669 amino acids which forms an integral part of the sarcomeric thin filament. In recent years, the nebula around this protein has been largely lifted resulting in the discovery that ... ...

    Abstract Nebulin, encoded by NEB, is a giant skeletal muscle protein of about 6669 amino acids which forms an integral part of the sarcomeric thin filament. In recent years, the nebula around this protein has been largely lifted resulting in the discovery that nebulin is critical for a number of tasks in skeletal muscle. In this review, we firstly discussed nebulin's role as a structural component of the thin filament and the Z-disk, regulating the length and the mechanical properties of the thin filament as well as providing stability to myofibrils by interacting with structural proteins within the Z-disk. Secondly, we reviewed nebulin's involvement in the regulation of muscle contraction, cross-bridge cycling kinetics, Ca
    MeSH term(s) Humans ; Muscle Proteins/pharmacology ; Muscle Proteins/therapeutic use ; Muscle, Skeletal/drug effects
    Chemical Substances Muscle Proteins ; nebulin (02X6KNJ5EE)
    Language English
    Publishing date 2020-01-25
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 283053-x
    ISSN 1573-2657 ; 0142-4319
    ISSN (online) 1573-2657
    ISSN 0142-4319
    DOI 10.1007/s10974-019-09565-3
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Small molecule drugs to improve sarcomere function in those with acquired and inherited myopathies.

    Claassen, Wout J / Baelde, Rianne J / Galli, Ricardo A / de Winter, Josine M / Ottenheijm, Coen A C

    American journal of physiology. Cell physiology

    2023  Volume 325, Issue 1, Page(s) C60–C68

    Abstract: Muscle weakness is a hallmark of inherited or acquired myopathies. It is a major cause of functional impairment and can advance to life-threatening respiratory insufficiency. During the past decade, several small-molecule drugs that improve the ... ...

    Abstract Muscle weakness is a hallmark of inherited or acquired myopathies. It is a major cause of functional impairment and can advance to life-threatening respiratory insufficiency. During the past decade, several small-molecule drugs that improve the contractility of skeletal muscle fibers have been developed. In this review, we provide an overview of the available literature and the mechanisms of action of small-molecule drugs that modulate the contractility of sarcomeres, the smallest contractile units in striated muscle, by acting on myosin and troponin. We also discuss their use in the treatment of skeletal myopathies. The first of three classes of drugs discussed here increase contractility by decreasing the dissociation rate of calcium from troponin and thereby sensitizing the muscle to calcium. The second two classes of drugs directly act on myosin and stimulate or inhibit the kinetics of myosin-actin interactions, which may be useful in patients with muscle weakness or stiffness.
    MeSH term(s) Humans ; Sarcomeres/physiology ; Calcium ; Muscle Contraction/physiology ; Muscle Weakness ; Myosins/genetics ; Troponin
    Chemical Substances Calcium (SY7Q814VUP) ; Myosins (EC 3.6.4.1) ; Troponin
    Language English
    Publishing date 2023-05-22
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 392098-7
    ISSN 1522-1563 ; 0363-6143
    ISSN (online) 1522-1563
    ISSN 0363-6143
    DOI 10.1152/ajpcell.00047.2023
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Comprehensive phenotypic characterization of an allelic series of zebrafish models of NEB-related nemaline myopathy.

    Fabian, Lacramioara / Karimi, Esmat / Farman, Gerrie P / Gohlke, Jochen / Ottenheijm, Coen A C / Granzier, Hendrikus L / Dowling, James J

    Human molecular genetics

    2024  

    Abstract: Nemaline myopathy (NM) is a rare congenital neuromuscular disorder characterized by muscle weakness and hypotonia, slow gross motor development, and decreased respiratory function. Mutations in at least twelve genes, all of each encode proteins that are ... ...

    Abstract Nemaline myopathy (NM) is a rare congenital neuromuscular disorder characterized by muscle weakness and hypotonia, slow gross motor development, and decreased respiratory function. Mutations in at least twelve genes, all of each encode proteins that are either components of the muscle thin filament or regulate its length and stability, have been associated with NM. Mutations in Nebulin (NEB), a giant filamentous protein localized in the sarcomere, account for more than 50% of NM cases. At present, there remains a lack of understanding of whether NEB genotype influences nebulin function and NM-patient phenotypes. In addition, there is a lack of therapeutically tractable models that can enable drug discovery and address the current unmet treatment needs of patients. To begin to address these gaps, here we have characterized five new zebrafish models of NEB-related NM. These mutants recapitulate most aspects of NEB-based NM, showing drastically reduced survival, defective muscle structure, reduced contraction force, shorter thin filaments, presence of electron-dense structures in myofibers, and thickening of the Z-disks. This study represents the first extensive investigation of an allelic series of nebulin mutants, and thus provides an initial examination in pre-clinical models of potential genotype-phenotype correlations in human NEB patients. It also represents the first utilization of a set of comprehensive outcome measures in zebrafish, including correlation between molecular analyses, structural and biophysical investigations, and phenotypic outcomes. Therefore, it provides a rich source of data for future studies exploring the NM pathomechanisms, and an ideal springboard for therapy identification and development for NEB-related NM.
    Language English
    Publishing date 2024-03-17
    Publishing country England
    Document type Journal Article
    ZDB-ID 1108742-0
    ISSN 1460-2083 ; 0964-6906
    ISSN (online) 1460-2083
    ISSN 0964-6906
    DOI 10.1093/hmg/ddae033
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  7. Article ; Online: A bright light on myosin to study skeletal muscle relaxation.

    Galli, Ricardo A / Ottenheijm, Coen A C

    The Journal of physiology

    2020  Volume 598, Issue 22, Page(s) 5001–5002

    MeSH term(s) Animals ; Deoxyadenine Nucleotides ; Mice ; Muscle Relaxation ; Muscle, Skeletal ; Myosins
    Chemical Substances Deoxyadenine Nucleotides ; Myosins (EC 3.6.4.1) ; 2'-deoxyadenosine triphosphate (K8KCC8SH6N)
    Language English
    Publishing date 2020-10-01
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 3115-x
    ISSN 1469-7793 ; 0022-3751
    ISSN (online) 1469-7793
    ISSN 0022-3751
    DOI 10.1113/JP280664
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  8. Article ; Online: Diaphragm Activity during Expiration in Ventilated Critically Ill Patients.

    de Vries, Heder J / Jonkman, Annemijn H / Holleboom, Minke C / de Grooth, Harm J / Shi, Zhonghua / Ottenheijm, Coen A / de Man, Angelique M / Tuinman, Pieter R / Heunks, Leo

    American journal of respiratory and critical care medicine

    2024  Volume 209, Issue 7, Page(s) 881–883

    MeSH term(s) Humans ; Diaphragm ; Critical Illness/therapy ; Respiration, Artificial ; Thorax ; Exhalation
    Language English
    Publishing date 2024-04-03
    Publishing country United States
    Document type Letter
    ZDB-ID 1180953-x
    ISSN 1535-4970 ; 0003-0805 ; 1073-449X
    ISSN (online) 1535-4970
    ISSN 0003-0805 ; 1073-449X
    DOI 10.1164/rccm.202310-1845LE
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  9. Article ; Online: Tirasemtiv enhances submaximal muscle tension in an Acta1:p.Asp286Gly mouse model of nemaline myopathy.

    Galli, Ricardo A / Borsboom, Tamara C / Gineste, Charlotte / Brocca, Lorenza / Rossi, Maira / Hwee, Darren T / Malik, Fady I / Bottinelli, Roberto / Gondin, Julien / Pellegrino, Maria-Antonietta / de Winter, Josine M / Ottenheijm, Coen A C

    The Journal of general physiology

    2024  Volume 156, Issue 4

    Abstract: Nemaline myopathies are the most common form of congenital myopathies. Variants in ACTA1 (NEM3) comprise 15-25% of all nemaline myopathy cases. Patients harboring variants in ACTA1 present with a heterogeneous disease course characterized by stable or ... ...

    Abstract Nemaline myopathies are the most common form of congenital myopathies. Variants in ACTA1 (NEM3) comprise 15-25% of all nemaline myopathy cases. Patients harboring variants in ACTA1 present with a heterogeneous disease course characterized by stable or progressive muscle weakness and, in severe cases, respiratory failure and death. To date, no specific treatments are available. Since NEM3 is an actin-based thin filament disease, we tested the ability of tirasemtiv, a fast skeletal muscle troponin activator, to improve skeletal muscle function in a mouse model of NEM3, harboring the patient-based p.Asp286Gly variant in Acta1. Acute and long-term tirasemtiv treatment significantly increased muscle contractile capacity at submaximal stimulation frequencies in both fast-twitch extensor digitorum longus and gastrocnemius muscle, and intermediate-twitch diaphragm muscle in vitro and in vivo. Additionally, long-term tirasemtiv treatment in NEM3 mice resulted in a decreased respiratory rate with preserved minute volume, suggesting more efficient respiration. Altogether, our data support the therapeutic potential of fast skeletal muscle troponin activators in alleviating skeletal muscle weakness in a mouse model of NEM3 caused by the Acta1:p.Asp286Gly variant.
    MeSH term(s) Humans ; Animals ; Mice ; Myopathies, Nemaline/drug therapy ; Myopathies, Nemaline/genetics ; Muscle Tonus ; Actins/genetics ; Muscle, Skeletal ; Disease Models, Animal ; Troponin ; Imidazoles ; Pyrazines
    Chemical Substances Actins ; CK-2017357 ; Troponin ; Imidazoles ; Pyrazines
    Language English
    Publishing date 2024-02-20
    Publishing country United States
    Document type Journal Article
    ZDB-ID 3118-5
    ISSN 1540-7748 ; 0022-1295
    ISSN (online) 1540-7748
    ISSN 0022-1295
    DOI 10.1085/jgp.202313471
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Inspiratory Muscle Training in Nemaline Myopathy.

    van Kleef, Esmee S B / Langer, Daniel / van Engelen, Baziel G M / Ottenheijm, Coen A C / Voermans, Nicol C / Doorduin, Jonne

    Journal of neuromuscular diseases

    2023  Volume 10, Issue 5, Page(s) 825–834

    Abstract: Background: Respiratory muscle weakness is a common feature in nemaline myopathy. Inspiratory muscle training (IMT) is an intervention that aims to improve inspiratory muscle strength.: Objective: The aim of this controlled before-and-after pilot ... ...

    Abstract Background: Respiratory muscle weakness is a common feature in nemaline myopathy. Inspiratory muscle training (IMT) is an intervention that aims to improve inspiratory muscle strength.
    Objective: The aim of this controlled before-and-after pilot study was to investigate if IMT improves respiratory muscle strength in patients with nemaline myopathy.
    Methods: Nine patients (7 females; 2 males, age 36.6±20.5 years) with respiratory muscle weakness and different clinical phenotypes and genotypes were included. Patients performed eight weeks of sham IMT followed by eight weeks of active threshold IMT. The patients trained twice a day five days a week for 15 minutes at home. The intensity was constant during the training after a gradual increase to 30% of maximal inspiratory pressure (MIP).
    Results: Active IMT significantly improved MIP from 43±15.9 to 47±16.6 cmH2O (p = 0.019). The effect size was 1.22. There was no significant effect of sham IMT. Sniff nasal inspiratory pressure, maximal expiratory pressure, spirometry, and diaphragm thickness and thickening showed no significant improvements.
    Conclusions: This pilot study shows that threshold IMT is feasible in patients with nemaline myopathy and improves inspiratory muscle strength. Our findings provide valuable preliminary data for the design of a larger, more comprehensive trial.
    MeSH term(s) Male ; Female ; Humans ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Breathing Exercises ; Myopathies, Nemaline ; Pilot Projects ; Respiratory Therapy ; Diaphragm ; Muscle Weakness
    Language English
    Publishing date 2023-07-10
    Publishing country Netherlands
    Document type Journal Article
    ISSN 2214-3602
    ISSN (online) 2214-3602
    DOI 10.3233/JND-221665
    Database MEDical Literature Analysis and Retrieval System OnLINE

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