A single-cell transcriptome analysis on aneurysmal human aortic tissue suggested that mitochondrial dysfunction and increased chromatin oxidative phosphorylation (OXPHOS) were found in TAA tissues and insufficient ATP production might not be sufficient for the contractile activities of human aortic smooth muscle cells (HAoSMCs) ( Li et al., 2020). Recent studies found that mitochondrial dysfunction was also related to the development of arterial aneurysm formation ( Cooper et al., 2021 van der Pluijm et al., 2018 Oller et al., 2021). Mitochondrial dysfunction has been closely linked to a variety of cardiovascular disorders, such as heart failure and atherosclerosis. However, the underlying mechanism through which insufficient NOTCH1 induces aortopathy remains to be explored. NOTCH1 insufficiency has been observed in the population with BAV ( Balistreri et al., 2018 Harrison et al., 2019 Malashicheva et al., 2020 Sciacca et al., 2013). In particular, genetic factors are considered to play a pivotal role in the disease progression ( Isselbacher et al., 2016 Prakash et al., 2014). Multiple factors, such as genetics and hemodynamics, are involved in the etiologies of BAV-TAA. At present, the understanding of pathophysiological mechanisms of BAV-TAA is incomplete, which leads to the absence of effective pharmaceutical therapy to alleviate aortopathy progression ( Lindeman and Matsumura, 2019). The current clinical management mainly relies on prophylactic surgical repair of the notably dilated aorta ( Coady et al., 2010). BAV-TAA poses a severe health threat to a large population because progressive aneurysmal dilation can potentially develop into lethal dissection or rupture ( Goldfinger et al., 2014 Olsson et al., 2006). BAV arises from incomplete separation or fusion of the aortic valve cusps and is associated with an approximately 40% risk of developing thoracic aortic aneurysm (TAA), namely, bicuspid aortopathy ( Verma and Siu, 2014). Introductionīicuspid aortic valve (BAV) disease is the most common congenital cardiovascular abnormality and is found in nearly 1.4% of the general population ( Garg et al., 2005 Michelena et al., 2011 Verma and Siu, 2014).
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also provides a platform to screen for drugs and examine the molecular mechanisms at play in aortic diseases. The aorta-on-a-chip model developed by Abudupataer et al. These compounds could potentially benefit individuals with deficient aortic valves, but experiments in animals and clinical trials would be needed first to confirm the results and assess safety. Applying drugs that tweak mitochondrial activity helped tissues from patients with bicuspid aortic valves to work better. then reduced NOTCH1 levels in healthy samples, which made the muscle tissue less able to contract and reduced the activity of the mitochondria. They also created an ‘aorta-on-a-chip’ model where aortic muscle cells were grown in the laboratory under conditions resembling those found in the body – including the rhythmic strain that the aorta is under because of the heart beating. analyzed the proteins present in diseased and healthy aortic muscle cells, confirming a lower production of NOTCH1 and impaired mitochondria in diseased tissues. To answer this question, Abudupataer et al. However, it is not known how these findings are connected or linked with the aneurysms developing. Problems in the mitochondria – the structures that power up a cell – are also observed. Recent studies have highlighted that many individuals with bicuspid aortic valves also have lower levels of a protein known as NOTCH1, which plays a key signalling role for cells.
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This is partly because the biological processes involved in the aneurysms worsening and bursting open is unclear. Open-heart surgery is currently the only way to treat these bulges (or ‘aneurysms’), as no drug exists that could slow down disease progression. This defect is also associated with bulges on the aorta which progressively weaken the artery, sometimes causing it to rupture. These valves may harden or become leaky, forcing the heart to work harder. Nearly 1.4% of people around the world are born with ‘bicuspid’ aortic valves that only have two flaps. Its three flaps (or ‘cusps’) are pushed open when the blood exits the heart, and they shut tightly so it does not flow back in the incorrect direction. The aortic valve, which sits at the entrance of the aorta, is a key component of this system. To function properly, the heart must remain a one-way system, pumping out oxygenated blood into the aorta – the largest artery in the body – so it can be distributed across the organism.