Abstract
One of the systems that are potentially affected in mitochondrial disorders, but hardly get systematically investigated, are the arteries. One of the phenotypic manifestations in arteries is atherosclerosis. This review focuses on the current knowledge and recent advances of mitochondrial atherosclerosis. We conducted a systematic literature review via PubMed using appropriate search terms.Atherosclerosis in mitochondrial disorders may result from a primary pathomechanism or a secondary one due to mitochondrial diabetes, arterial hypertension, or hyperlipidemia. Anecdotal reports show that primary atherosclerosis can be a phenotypic feature of mitochondrial disorders. Predominantly, patients carrying mutations in mtDNA-located genes may develop primary mitochondrial atherosclerosis. Though not systematically investigated, it is conceivable that primary mitochondrial atherosclerosis results from increased oxidative stress, mitophagy, metabolic breakdown, or lactic acidosis. Mitochondrial disorder patients with primary mitochondrial atherosclerosis should receive not only antithrombotic medication but also antioxidants and cofactors.Atherosclerosis in mitochondrial disorders may occur even in the absence of classical atherosclerosis risk factors, suggesting that atherosclerosis can be a primary manifestation of the metabolic defect. Though primary atherosclerosis in mitochondrial disorders has not been systematically investigated, anecdotal data indicate that mitochondrial dysfunction can be a mechanism for the development of primary, mitochondrial atherosclerosis. These patients require antioxidants and cofactors in addition to antithrombotic medication.
Highlights
BackgroundSince the first recognition of mitochondrial disorders (MIDs), it had been noticed that they sooner or later become multisystem diseases, affecting all organs or tissues, including the arteries [1]
Increased oxidative stress explains the pathogenesis of primary ASCL, as it leads to lipid peroxidation, damage of mitochondrial DNA, mitochondrial dysfunction, damage of endothelial cells, vascular smooth muscle cells (VSMC), erythrocytes, thrombocytes, and, lastly, to apoptosis via either the receptor-mediated pathway or the mitochondrion-mediated pathway and the activation of the caspase cascade [24]
Though primary and secondary ASCL in MIDs have not been systematically investigated, anecdotal data currently available indicate that mitochondrial dysfunction can be a mechanism for the development of ASCL
Summary
Since the first recognition of mitochondrial disorders (MIDs), it had been noticed that they sooner or later become multisystem diseases, affecting all organs or tissues, including the arteries [1]. Affection of the arteries manifests either as macro-angiopathy or micro-angiopathy [1]. Mitochondrial microangiopathy manifests as leukoencephalopathy, migraine-like headache, or peripheral retinopathy [1]. Mitochondrial macroangiopathy manifests clinically as ectasia of arteries, aneurysm formation, dissection, spontaneous arterial rupture, or atherosclerosis (ASCL) [1]. This systematic review focuses on the current knowledge and recent advances of mitochondrial ASCL. Its scientific content comprises the confirmation that mitochondrial atherosclerosis exists, that the absence of classical risk factors for ASCL does not exclude it, and that patients with mitochondrial ASCL should receive antioxidants and cofactors in addition to antithrombotic management
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