Is there a role for autophagy in ascending aortopathy associated with tricuspid or bicuspid aortic valve?. (2nd April 2019)

• Record Type: Journal Article
• Authors: Forte, Amalia; Cipollaro, Marilena; De Feo, Marisa; Della Corte, Alessandro
• Is Part Of: Clinical science. Volume 133:Number 7(2019)
• Page Start: 805
• Page End: 819
• Publication Date: 2019-04-02
• Subjects: autophagy -- aortopathy -- bicuspid aortic valve -- hemodynamics -- pathogenesis; Medicine -- Periodicals; Biochemistry -- Periodicals; 616
• DOI: 10.1042/CS20181092
• Languages: English
• ISSNs: 0143-5221

Abstract

Abstract : Autophagy is a conserved process by which cytoplasmatic elements are sequestered in vesicles and degraded after their fusion with lysosomes, thus recycling the precursor molecules. The autophagy-mediated removal of redundant/harmful/damaged organelles and biomolecules plays not only a replenishing function, but protects against stressful conditions through an adaptive mechanism. Autophagy, known to play a role in several pathological conditions, is now gaining increasing attention also in the perspective of the identification of the pathogenetic mechanisms at the basis of ascending thoracic aortic aneurysm (TAA), a localized or diffused dilatation of the aorta with an abnormal widening greater than 50 percent of the vessel's normal diameter. TAA is less frequent than abdominal aortic aneurysm (AAA), but is encountered with a higher percentage in patients with congenital heart disease or known genetic syndromes. Several biological aspects of TAA pathophysiology remain to be elucitated and therapeutic needs are still widely unmet. One of the most controversial and epidemiologically important forms of TAA is that associated with the congenital bicuspid malformation of the aortic valve (BAV). Dysregulated autophagy in response, for example, to wall shear stress alterations, has been demonstrated to affect the phenotype of vascular cells relevant to aortopathy, with potential consequences on signaling, remodeling, and angiogenesis. The most recent findings and hypotheses concerning the multiple aspects of autophagy and of its dysregulation are summarized, both in general and in the context of the different vascular cell types and of TAA progression, with particular reference to BAV-related aortopathy.