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Characterization of the Mitochondrial Phenotype Associated with Primary Biliary Cholangitis
DownloadSpring 2017
Primary Biliary Cholangitis (PBC) is a chronic liver disease characterized by the immune-mediated destruction of intra-hepatic bile ducts. The autoimmune nature of PBC involves humoral and cell-mediated responses that target endogenous mitochondrial proteins. It is thought that the breakdown of...
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Chronic Fatigue Mechanisms in Autoimmune Diseases: Lessons from Primary Biliary Cholangitis and Systemic Sclerosis
DownloadFall 2023
Systemic sclerosis (SSc) is an autoimmune disease affecting the body's connective tissues, resulting in progressive fibrosis and vasculopathy. In some cases, individuals with SSc may also develop primary biliary cholangitis (PBC), another autoimmune disease characterized by damage to their...
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Spring 2013
Mitochondria generate reactive oxygen species (mROS) and metabolic substrates like alpha-ketogluterate (αKG), in proportion to oxygen, which target extra-mitochondrial effectors to coordinate a cellular response. For example, acute hypoxia decreases mROS, resulting in pulmonary artery smooth...
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The metabolic basis of cell identity in the pulmonary arteries and the right ventricle in pulmonary arterial hypertension
DownloadFall 2023
Pulmonary arterial hypertension (PAH) is an incurable disease characterized by profound pulmonary vascular remodeling, including the development of plexogenic lesions (which is the hallmark of PAH) that results in lumen obliteration, raising the pulmonary vascular resistance (PVR) and the right...
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Understanding Heart Failure in Patients with Dilated Cardiomyopathy: A Direct Tissue Analysis from Explanted Human Hearts
DownloadFall 2022
Background Chronic heart failure (HF) remains a rising global epidemic that affects both adults and children population. The causes of HF are heterogeneous, and syndromes are often complicated by concomitant disorders such as iron deficiency (ID) and diabetes. Comorbidities not only complicate...