Marianna Langione
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View article: Long-term mavacamten exposure reduces force and sarcomere density in a hiPSC model of hypertrophic cardiomyopathy
Long-term mavacamten exposure reduces force and sarcomere density in a hiPSC model of hypertrophic cardiomyopathy Open
Background Mavacamten, a first-in-class allosteric myosin inhibitor, has demonstrated efficacy and safety in obstructive hypertrophic cardiomyopathy (oHCM), notably reducing symptoms, left ventricular outflow obstruction, and wall thicknes…
View article: 3D-Printable Gelatin Methacrylate-Xanthan Gum Hydrogel Bioink Enabling Human Induced Pluripotent Stem Cell Differentiation into Cardiomyocytes
3D-Printable Gelatin Methacrylate-Xanthan Gum Hydrogel Bioink Enabling Human Induced Pluripotent Stem Cell Differentiation into Cardiomyocytes Open
We describe the development of a bioink to bioprint human induced pluripotent stem cells (hiPSCs) for possible cardiac tissue engineering using a gelatin methacrylate (GelMA)-based hydrogel. While previous studies have shown that GelMA at …
View article: Caveolin-3 and Caveolin-1 Interaction Decreases Channel Dysfunction Due to Caveolin-3 Mutations
Caveolin-3 and Caveolin-1 Interaction Decreases Channel Dysfunction Due to Caveolin-3 Mutations Open
Caveolae constitute membrane microdomains where receptors and ion channels functionally interact. Caveolin-3 (cav-3) is the key structural component of muscular caveolae. Mutations in CAV3 lead to caveolinopathies, which result in both mus…
View article: Corrigendum: Calcium handling maturation and adaptation to increased substrate stiffness in human iPSC-derived cardiomyocytes: the impact of full-length dystrophin deficiency
Corrigendum: Calcium handling maturation and adaptation to increased substrate stiffness in human iPSC-derived cardiomyocytes: the impact of full-length dystrophin deficiency Open
[This corrects the article DOI: 10.3389/fphys.2022.1030920.].
View article: Slower Calcium Handling Balances Faster Cross-Bridge Cycling in Human <i>MYBPC3</i> HCM
Slower Calcium Handling Balances Faster Cross-Bridge Cycling in Human <i>MYBPC3</i> HCM Open
Background: The pathogenesis of MYBPC3 -associated hypertrophic cardiomyopathy (HCM) is still unresolved. In our HCM patient cohort, a large and well-characterized population carrying the MYBPC3 :c772G>A variant (p.Glu258Lys, E258K) provid…
View article: Calcium handling maturation and adaptation to increased substrate stiffness in human iPSC-derived cardiomyocytes: The impact of full-length dystrophin deficiency
Calcium handling maturation and adaptation to increased substrate stiffness in human iPSC-derived cardiomyocytes: The impact of full-length dystrophin deficiency Open
Cardiomyocytes differentiated from human induced Pluripotent Stem Cells (hiPSC- CMs) are a unique source for modelling inherited cardiomyopathies. In particular, the possibility of observing maturation processes in a simple culture dish op…
View article: Structure-Function Investigation of the E258K-Mybpc3 Mutation in HCM Patient-Derived Cardiomyocytes
Structure-Function Investigation of the E258K-Mybpc3 Mutation in HCM Patient-Derived Cardiomyocytes Open
View article: The E258K-MYPBC3 Modelled in HCM Patient-derived Cardiomyocytes to Identify the Primary Impact of the Mutation versus the Secondary Changes Due to Cardiac Remodeling
The E258K-MYPBC3 Modelled in HCM Patient-derived Cardiomyocytes to Identify the Primary Impact of the Mutation versus the Secondary Changes Due to Cardiac Remodeling Open
View article: Optical Investigation of Action Potential and Calcium Handling Maturation of hiPSC-Cardiomyocytes on Biomimetic Substrates
Optical Investigation of Action Potential and Calcium Handling Maturation of hiPSC-Cardiomyocytes on Biomimetic Substrates Open
Cardiomyocytes from human induced pluripotent stem cells (hiPSC-CMs) are the most promising human source with preserved genetic background of healthy individuals or patients. This study aimed to establish a systematic procedure for explori…
View article: Loss of Dystrophin Alters Calcium-Handling Maturation in Response to Microenvironment in Hipsc-Cardiomyocytes from Duchenne Muscular Dystrophy Patients
Loss of Dystrophin Alters Calcium-Handling Maturation in Response to Microenvironment in Hipsc-Cardiomyocytes from Duchenne Muscular Dystrophy Patients Open