IPSC derived cardiac fibroblasts of DMD patients show compromised actin microfilaments, metabolic shift and pro-fibrotic phenotype

Abstract Duchenne muscular dystrophy (DMD) is a severe form of muscular dystrophy caused by mutations in the dystrophin gene. We characterized which isoforms of dystrophin were expressed by human induced pluripotent stem cell (hiPSC)-derived cardiac fibroblasts obtained from control and DMD patients...

Full description

Bibliographic Details
Published in:	Biology Direct
Main Authors:	Salwa Soussi, Lesia Savchenko, Davide Rovina, Jason S. Iacovoni, Andrea Gottinger, Maxime Vialettes, Josè-Manuel Pioner, Andrea Farini, Sara Mallia, Martina Rabino, Giulio Pompilio, Angelo Parini, Olivier Lairez, Aoife Gowran, Nathalie Pizzinat
Format:	Article
Language:	English
Published:	BMC 2023-07-01
Subjects:	Duchenne Human induced pluripotent stem cell Fibroblasts Mitochondrial oxidation Actin microfilaments
Online Access:	https://doi.org/10.1186/s13062-023-00398-2

Similar Items

Correction: IPSC derived cardiac fibroblasts of DMD patients show compromised actin microfilaments, metabolic shift and pro-fibrotic phenotype
by: Salwa Soussi, et al.
Published: (2023-10-01)

The influence of the endocannabinoid system on the profibrotic profile of cardiac fibroblasts associated with Duchenne Muscular Dystrophy
by: Lesia Savchenko, et al.
Published: (2024-11-01)

Visualization of Cell Membrane Tension Regulated by the Microfilaments as a “Shock Absorber” in Micropatterned Cells
by: Xianmeng Wang, et al.
Published: (2023-06-01)

Dynamic changes and transcriptome analyses reveal the microfilament skeleton response to water stress in thalli of Neopyropia yezoensis inhabiting the intertidal zone
by: Jiqiang Yin, et al.
Published: (2025-03-01)

Microfilament cytoskeletons in BY-2 protoplasts and broad bean mesophyll protoplasts and the effect of latrunculin B on their distribution
by: YE Lu-fei, et al.
Published: (2010-09-01)

Amorphous silica nanoparticles cause abnormal cytokinesis and multinucleation through dysfunction of the centralspindlin complex and microfilaments
by: Liyan Xiao, et al.
Published: (2023-08-01)

Mechanical Sensing Element PDLIM5 Promotes Osteogenesis of Human Fibroblasts by Affecting the Activity of Microfilaments
by: Xiaolan Huang, et al.
Published: (2021-05-01)

The role of the cytoskeleton in fibrotic diseases
by: Caoyuan Niu, et al.
Published: (2024-10-01)

Beam theory of cellular microfilaments based on coarse-grained molecular force field
by: Kun Huang, et al.
Published: (2024-03-01)

Extracellular AMP Inhibits Pollen Tube Growth in <i>Picea meyeri</i> via Disrupted Calcium Gradient and Disorganized Microfilaments
by: Junhui Zhou, et al.
Published: (2024-12-01)

Role of microfilament depolymerization in osteogenic differentiation of bone marrow mesenchymal stem cells promoted by low intensity pulsed ultrasound
by: YANG Li, et al.
Published: (2022-05-01)

miR-196a enhances polymerization of neuronal microfilaments through suppressing IMP3 and upregulating IGF2 in Huntington’s disease
by: Han-In Yang, et al.
Published: (2022-12-01)

The Endocannabinoid System and Cannabidiol: Past, Present, and Prospective for Cardiovascular Diseases
by: Martina Rabino, et al.
Published: (2021-09-01)

Chorein sensitive microtubule organization in tumor cells
by: Saad Alkahtani, et al.
Published: (2023-09-01)

Reprogramming of dermal fibroblasts from a Duchenne muscular dystrophy patient carrying a deletion of exons 45–50 into an induced pluripotent stem cell line (CCMi005-A)
by: Davide Rovina, et al.
Published: (2022-10-01)

Mechanisms of regulation electric and contractile activity smooth muscle cells: the role of cytoskeleton
by: M. A. Medvedev, et al.
Published: (2008-12-01)

Exploring the maternal inheritance transmitted by the oocyte to its progeny
by: Verlhac, Marie-Hélène
Published: (2024-06-01)

Effect of low temperature on profilins and ADFs transcription and actin cytoskeleton reorganization in Arabidopsis
by: T. T. Fan, et al.
Published: (2015-12-01)

Effect of Filament Fineness on Composite Yarn Residual Torque
by: Sarıoğlu Esin, et al.
Published: (2018-03-01)

Organization of the Cytoskeleton in Ectopic Foci of the Endometrium with Rare Localization
by: Konstantin A. Toniyan, et al.
Published: (2021-08-01)

Impact of Marine Drugs on Cytoskeleton-Mediated Reproductive Events
by: Francesco Silvestre, et al.
Published: (2010-03-01)

Plant strategy to adapt to life in water: peculiarities of root system organization in Sagittaria sagittifolia and Alisma plantago-aquatica (Alismataceae)
by: Ovcharenko Yu., et al.
Published: (2025-06-01)

Porosity Assessment of Melt Electrospun PLA
by: Emilija Zdraveva
Published: (2025-01-01)

Derivation of the Duchenne muscular dystrophy patient-derived induced pluripotent stem cell line lacking DMD exons 49 and 50 (CCMi001DMD-A-3, ∆49, ∆50)
by: Gabriella Spaltro, et al.
Published: (2017-12-01)

Modulation of rice grain shape and appearance by the GS10-encoded long coiled-coil protein
by: Yanmin Luo, et al.
Published: (2025-02-01)

Cell and Tissue Nanomechanics: From Early Development to Carcinogenesis
by: Mikhail E. Shmelev, et al.
Published: (2022-02-01)

Novel MYO1D Missense Variant Identified Through Whole Exome Sequencing and Computational Biology Analysis Expands the Spectrum of Causal Genes of Laterality Defects
by: Rabab Said Alsafwani, et al.
Published: (2021-09-01)

Altered cytoskeleton dynamics in patient-derived iPSC-based model of PCDH19 clustering epilepsy
by: Rossella Borghi, et al.
Published: (2025-01-01)

Special Issue: Pathophysiology and Therapeutic Perspectives in DMD: The Well-Defined Role of the Immune System
by: Andrea Farini
Published: (2021-12-01)

Cytochalasins as Modulators of Stem Cell Differentiation
by: Luca Pampanella, et al.
Published: (2024-02-01)

Cytochalasin B Modulates Nanomechanical Patterning and Fate in Human Adipose-Derived Stem Cells
by: Eva Bianconi, et al.
Published: (2022-05-01)

Nano-Structured Ridged Micro-Filaments (≥100 µm Diameter) Produced Using a Single Step Strategy for Improved Bone Cell Adhesion and Proliferation in Textile Scaffolds
by: Nemeshwaree Behary, et al.
Published: (2022-06-01)

Research progress on the roles of actin-depolymerizing factor in plant stress responses
by: Yongwang Sun, et al.
Published: (2023-11-01)

Anti-fibrotic, muscle-promoting antibody-drug conjugates for the improvement and treatment of DMD
by: Anas Odeh, et al.
Published: (2025-05-01)

The mechanical mechanism of angiotensin II induced activation of hepatic stellate cells promoting portal hypertension
by: Yiheng Zhang, et al.
Published: (2024-06-01)

Plasmolysis: Loss of Turgor and Beyond
by: Ingeborg Lang, et al.
Published: (2014-11-01)

Mechanical properties of polycaprolactone microfilaments for muscular tissue engineering
by: Laura Rojas-Rojas, et al.
Published: (2023-03-01)

Generation of four human induced pluripotent stem cell lines from COVID-19 hospitalized patients with increased levels of cardiac Troponin in the acute infection phase developing or not myocarditis
by: Martina Rabino, et al.
Published: (2023-03-01)

Analysis of effector/memory regulatory T cells from arrhythmogenic cardiomyopathy patients identified IL-32 as a novel player in ACM pathogenesis
by: Salwa Soussi, et al.
Published: (2025-02-01)

Evaluation of Biomechanical and Chemical Properties of Gamma-Irradiated Polycaprolactone Microfilaments for Musculoskeletal Tissue Engineering Applications
by: Laura Rojas-Rojas, et al.
Published: (2022-01-01)