The Role of Mitochondria and the Phospholipid Cardiolipin in Health and Disease

Learn more about the role of Mitochondria and the Phospholipid Cardiolipin in health and disease from this brief high level overview.

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Metabolism is the set of life-sustaining chemical processes that combine the air we breathe and the food we eat into cellular energy. Central to cellular metabolism is the Mitochondrial Network, which generates energy in the form of Adenosine Triphosphate, or ATP, along the electron transport chain.

Mitochondria are essential for many aspects of life, influencing energy production, cell signaling, and cell survival.

Mitochondria support the function of every organ system in the body; including the heart, skeletal muscles, and eyes. Mitochondria contain and outer and inner membrane. In healthy tissue, the inner membrane has folds, known as Cristae, which project into the matrix and house the proteins involved in energy production. The main building blocks of these membrane bi-layers, are Phospholipids. Cardiolipin is a signature Phospholipid within the inner membrane and is essential for nearly every aspect of Mitochondrial function. Cardiolipin influences energy production along the electron transport chain, the movement of molecules in and out of the Mitochondria, and the ability of Mitochondria to replicate, network, and turn over. Cardiolipin has a unique structure, influencing the curvature of Cristae, and the assembly of protein complexes involved in energy production. Cardiolipin-dependent assembly at the electron transport chain facilitates optimal electron transfer necessary for cellular energy production.

Mitochondrial Dysfunction is central to disease etiology in many different pathologies. Features of Mitochondrial Dysfunction include alterations to the inner membrane structure, and can include damage and loss of Cardiolipin; disrupting the production of protein complexes embedded in the membrane, including the respiratory complexes. As a result of this, electrons are more likely to leak and generate reactive oxygen species rather than move along the respiratory chain, which can lead to further damage of Mitochondrial lipids and proteins. Mitochondrial Dysfunction that can include damage or loss of Cardiolipin have been implicated in the etiology in a variety of pathological conditions, including cardiomyopathies, skeletal muscle weakness, and vision loss.

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At Stealth BioTherapeutics, our work is personal. We believe strongly in the potential of our science to deliver new treatment solutions and improve quality of life for patients with diseases involving mitochondrial dysfunction. Many of the diseases we are targeting currently have no FDA-approved treatments, presenting a significant unmet medical need that we hope to help address. Our team works closely with patients and healthcare professionals to achieve our mission of developing therapies for rare and common age-related diseases involving mitochondrial dysfunction. We collaborate closely with patient advocacy to understand how these diseases impact the daily lives of patients and to better inform our clinical development efforts. We also partner with leading institutions, physicians and scientists to develop our mitochondrial therapies. In preclinical studies, elamipretide has been shown to restore mitochondrial function at the cellular level with resulting improvement in the function of organ systems such as skeletal muscle, eye, brain, heart and kidney. Our hope is that this investigational therapy will help improve the prognosis and quality of life for patients and families whose lives are touched by mitochondrial disease.