Helena M. Viola YOU? Author Swipe

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Novel Peptide Therapeutics Targeting the L-Type Calcium Channel Prevent Hypertrophic Cardiomyopathy by Decreasing Mitochondrial Energetics Open

Teagan Er, Daniel C. Reinke, Alice J. Francis, Tanya Solomon, Helena M. Viola , et al. · 2025

Mitochondrial dysfunction is considered to drive the development of hypertrophic cardiomyopathy (HCM). In search of a preventative HCM therapy, we explored the efficacy of amino acid peptide variants that could alter the L-type Ca²⁺

Gene editing and cardiac disease modelling for the interpretation of genetic variants of uncertain significance in congenital heart disease Open

Vanessa S. Fear, Catherine A. Forbes, Nicole C. Shaw, Kathryn O. Farley, Jessica L. Mantegna , et al. · 2023

Cytoskeletal disarray increases arrhythmogenic vulnerability during sympathetic stimulation in a model of hypertrophic cardiomyopathy Open

Henrietta Cserné Szappanos, Helena M. Viola, Danica W. Ito, Seakcheng Lim, Melissa Mangala , et al. · 2023

Familial hypertrophic cardiomyopathy (FHC) patients are advised to avoid strenuous exercise due to increased risk of arrhythmias. Mice expressing the human FHC-causing mutation R403Q in the myosin heavy chain gene ( MYH6 ) recapitulate the…

A maladaptive feedback mechanism between the extracellular matrix and cytoskeleton contributes to hypertrophic cardiomyopathy pathophysiology Open

Helena M. Viola, Caitlyn Richworth, Tanya Solomon, Ian L. Chin, Henrietta Cserné Szappanos , et al. · 2023

A common genetic variant of a mitochondrial RNA processing enzyme predisposes to insulin resistance Open

Giulia Rossetti, Judith A. Ermer, Maike Stentenbach, Stefan J. Siira, Tara R. Richman , et al. · 2021

A variant in an RNA processing enzyme predisposes to insulin resistance by reducing calcium release and insulin secretion.

Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan Open

Tara R. Richman, Judith A. Ermer, Stefan J. Siira, Irina Kuznetsova, Christopher A. Brosnan , et al. · 2021

Changes in the rate and fidelity of mitochondrial protein synthesis impact the metabolic and physiological roles of mitochondria. Here we explored how environmental stress in the form of a high‐fat diet modulates mitochondrial translation …

Characterization and validation of a preventative therapy for hypertrophic cardiomyopathy in a murine model of the disease Open

Helena M. Viola, Ashay Shah, Victoria Johnstone, Henrietta Cserné Szappanos, Mark P. Hodson , et al. · 2020

Significance Hypertrophic cardiomyopathy affects 1:500 of the general population. Current drug therapy is used to manage symptoms in patients. There is an unmet need for treatments that can prevent the cardiomyopathy. Here we identify biom…

Preventative therapeutic approaches for hypertrophic cardiomyopathy Open

Tanya Solomon, Aleksandra Filipovska, Livia C. Hool, Helena M. Viola · 2020

Sarcomeric gene mutations are associated with the development of hypertrophic cardiomyopathy (HCM). Current drug therapeutics for HCM patients are effective in relieving symptoms, but do not prevent or reverse disease progression. Moreover…

Lack of Strategic Funding and Long-Term Job Security Threaten to Have Profound Effects on Cardiovascular Researcher Retention in Australia Open

Rachel E. Climie, Jason Wu, Anna C. Calkin, Niamh Chapman, Sally C Inglis , et al. · 2020

Fidelity and coordination of mitochondrial protein synthesis in health and disease Open

Danielle L. Rudler, Laetitia A. Hughes, Helena M. Viola, Livia C. Hool, Oliver Rackham , et al. · 2020

The evolutionary acquisition of mitochondria has given rise to the diversity of eukaryotic life. Mitochondria have retained their ancestral α‐proteobacterial traits through the maintenance of double membranes and their own circular genome.…

A dendronized polymer variant that facilitates safe delivery of a calcium channel antagonist to the heart Open

Helena M. Viola, Ashay Shah, Jessica A. Kretzmann, Cameron W. Evans, Marck Nörret , et al. · 2020

Therapeutic approaches for myocardial ischemia-reperfusion injury (MI) have been ineffective due to limited bioavailability and poor specificity. We have previously shown that a peptide that targets the α-interaction domain of the cardiac …

145 Oxidative Stress Does not Contribute to the Development of Hypertrophic Cardiomyopathy in a Murine Model of the Disease Open

Helena M. Viola, Livia C. Hool · 2020

095 Characterising the Phenotypic Switch in a Murine Model of Hypertrophic Cardiomyopathy Open

Tanya Solomon, Elif Eroğlu, N. Andharia, Henrietta Cserné Szappanos, Livia C. Hool , et al. · 2020

Fidelity of translation initiation is required for coordinated respiratory complex assembly Open

Danielle L. Rudler, Laetitia A. Hughes, Kara L. Perks, Tara R. Richman, Irina Kuznetsova , et al. · 2019

The initiation of mitochondrial protein synthesis fine-tunes the assembly of respiratory complexes and energy production.

TGF‐β‐induced fibrotic stress increases G‐quadruplex formation in human fibroblasts Open

Priyanka Toshniwal, Michelle Nguyen, Aurore Guédin, Helena M. Viola, Diwei Ho , et al. · 2019

Scar formation after wound healing is a major medical problem. A better understanding of the dynamic nuclear architecture of the genome during wound healing could provide insights into the underlying pathophysiology and enable novel therap…

Myoglobinopathy is an adult-onset autosomal dominant myopathy with characteristic sarcoplasmic inclusions Open

Montse Olivé, Martin Engvall, Gianina Ravenscroft, Macarena Cabrera‐Serrano, Hong Jiao , et al. · 2019

Impaired calcium handling and mitochondrial metabolic dysfunction as early markers of hypertrophic cardiomyopathy Open

Helena M. Viola, Livia C. Hool · 2019

Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder, characterised by myocyte remodeling, disorganisation of sarcomeric proteins, impaired energy metabolism and altered cardiac contractility. Gene mutations encoding cardiac …

Optimal Design of Dendrimer Nanotechnology to Deliver a Peptide as a Therapeutic to the Heart Open

Arshad Ali Shah, Helena M. Viola, Marck Nörret, Jessica A. Kretzmann, K. Swaminathan Iyer , et al. · 2019

Misregulation of Mitochondrial Protein Synthesis Leads to Cardiomyopathy Open

Laetitia A. Hughes, Danielle L. Rudler, Kara L. Perks, Tara R. Richman, Inna Kusnetsova , et al. · 2019

The Role of Extracellular Matrix Stiffness on Cardiac Metabolic Activity Open

Tanya Solomon, Yu Suk Choi, Livia C. Hool, Helena M. Viola · 2019

Concerted regulation of mitochondrial and nuclear non‐coding <span>RNA</span> s by a dual‐targeted <span>RN</span> ase Z Open

Stefan J. Siira, Giulia Rossetti, Tara R. Richman, Kara L. Perks, Judith A. Ermer , et al. · 2018

A platform for discovery of functional cell-penetrating peptides for efficient multi-cargo intracellular delivery Open

Katrin Hoffmann, Nadia Milech, Suzy M. Juraja, Paula Cunningham, Shane R. Stone , et al. · 2018

A Morpholino Oligomer Therapy Regime That Restores Mitochondrial Function and Prevents mdx Cardiomyopathy Open

Helena M. Viola, Victoria Johnstone, A.M. Adams, Sue Fletcher, Livia C. Hool · 2018

PTCD1 Is Required for 16S rRNA Maturation Complex Stability and Mitochondrial Ribosome Assembly Open

Kara L. Perks, Giulia Rossetti, Irina Kuznetsova, Laetitia A. Hughes, Judith A. Ermer , et al. · 2018

The regulation of mitochondrial RNA life cycles and their roles in ribosome biogenesis and energy metabolism are not fully understood. We used CRISPR/Cas9 to generate heart- and skeletal-muscle-specific knockout mice of the pentatricopepti…

Real-Time Bioimpedance Sensing of Antifibrotic Drug Action in Primary Human Cells Open

Maryam Parviz, Priyanka Toshniwal, Helena M. Viola, Livia C. Hool, Mark W. Fear , et al. · 2017

Fibrotic diseases are among the most serious health issues with severe burdens due to their chronic nature and a large number of patients suffering from the debilitating effects and long-term sequelae. Collagenase treatment is a nonsurgica…

Adult-onset obesity is triggered by impaired mitochondrial gene expression Open

Kara L. Perks, Nicola Ferreira, Tara R. Richman, Judith A. Ermer, Irina Kuznetsova , et al. · 2017

Reduction in an RNA binding protein impairs mitochondrial biogenesis and alters cell signaling, resulting in obesity.

Auto-regulation in the powerhouse Open

Helena M. Viola, Livia C. Hool · 2017

Mitochondrial flashes have a central role in ensuring that ATP levels remain constant in heart cells.

Dystrophic Cardiomyopathy—Potential Role of Calcium in Pathogenesis, Treatment and Novel Therapies Open

Victoria Johnstone, Helena M. Viola, Livia C. Hool · 2017

Duchenne muscular dystrophy (DMD) is caused by defects in the DMD gene and results in progressive wasting of skeletal and cardiac muscle due to an absence of functional dystrophin. Cardiomyopathy is prominent in DMD patients, and contribut…

Manipulating L-Type Calcium Channel Activity Alters Mitochondrial Function and Prevents Hypertrophic Cardiomyopathy in a Troponin I Mutant Mouse Model Open

Helena M. Viola, Victoria Johnstone, Christopher Semsarian, Livia C. Hool · 2017

Treatment of Adult mdx Mice with Phosphorodiamidate Morpholino Oligomer Restores Cardiac Mitochondrial Energetics and Prevents the Dystrophic Cardiomyopathy Open

Helena M. Viola, Victoria Johnstone, A.M. Adams, Sue Fletcher, Livia C. Hool · 2017