Connective Tissue Disorders and Cardiovascular Complications: The Indomitable Role of Transforming Growth Factor-β Signaling

Wheeler, Jason B.; Ikonomidis, John S.; Jones, Jeffrey A.

doi:10.1007/978-3-030-80614-9_7

Cited by 11 publications

(9 citation statements)

References 143 publications

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“…However, patients with acute aortic dissection were included in their study, compared to our study, and TGF-β1 concentrations in this subgroup differed the most from the control group. In contrast to the Rueda-Martínez et al study we found a significant 2-fold decrease in the levels of TGF-β1 in the blood of patients with aortic dilatation compared to the reference group ( 28 ). Although we found significantly different values of TGF-β1 between DAA and NDAA compared to the reference group, we did not find a correlation between concentrations of TGF-β1 and ascending aorta dilatation size.…”

Section: Discussioncontrasting

confidence: 99%

“…Several studies have found that the TGF-β1 signaling pathway is important in the development of abdominal and thoracic aortic aneurysms (2,18,19,26,27). These findings in patients with connective tissue disorders have been published (28). Hilebrand et al reported elevated total TGF-β1 levels in the entire spectrum of genetic aortic syndromes (27).…”

Section: Discussionmentioning

confidence: 76%

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Transforming growth factor serum concentrations in patients with proven non-syndromic aortopathy

Karalko

Pojar

Žaloudková

et al. 2022

Front. Cardiovasc. Med.

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BackgroundThe mechanism underlying aortic dilatation is still unknown. Vascular dilatation is thought to be the result of progressive aortic media degeneration caused by defective vascular matrix hemostasis, including TGF-β1 dysregulation. The goal of this study is to draw attention to the potential utility of TGF-β1 as a diagnostic marker in non-syndromic patients with aortic dilatation.MethodsTGF-β1 levels in plasma were measured in 50 patients who had undergone surgery and had a tricuspid or bicuspid aortic valve as well as a normal or dilated ascending aorta. A pathologist also examined thirty resected aorta samples. To specify the reference range of TGF-β1, a control group of 40 volunteers was enrolled in this study.ResultsWe discovered a significant difference in TGF-β1 levels between patients with aortic dilatation and the control group (32.5 vs. 63.92; P < 0.001), as well as between patients with non-dilated aorta but with aortic valve disease, and the control group (27.68 vs. 63.92; P < 0.001). There was no difference between the dilated ascending aorta group and the non-dilated ascending aorta group. We found a poor correlation between TGF-β1 levels and ascending aorta diameter as well as the grade of ascending aorta histopathological abnormalities.ConclusionTGF-β1 concentration does not meet the criteria to be a specific marker of aortic dilatation, but it is sensitive to aortic valvulopathy-aortopathy. A larger patient cohort study is needed to confirm these findings.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 76%

Transforming growth factor serum concentrations in patients with proven non-syndromic aortopathy

Karalko

Pojar

Žaloudková

et al. 2022

Front. Cardiovasc. Med.

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“…Regnase-1 is a well-known member of the molecular machinery involved in cancer, as it forms part of the anti-inflammatory molecular machinery of defense. 3 The authors rationalize that this endoribonuclease might be involved in Marfan syndrome aortopathy because (1) its expression is regulated by TGF-β, a crucial aggravating driver of aortopathy, 4 (2) it is a negative regulator of oxidative stress, another known actor in TAAD progression, 5 and (3) its intrinsic anti-inflammatory properties, where the role of inflammation is becoming increasingly more evident in the progression of the aortopathy. 6 …”

Section: Main Textmentioning

confidence: 99%

Navigating toward gene therapy in Marfan syndrome: A hope for halting aortic aneurysm

Egea

2024

Molecular Therapy - Methods & Clinical Development

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“…Fibrillin-1 monomers form the structure that helps generate mature elastic bers from tropoelastin [51]. In these circumstances, micro brils act as a scaffold for elastin and support the assembly process with the formation of a functional architecture of elastic bers [26,50].…”

Section: Introductionmentioning

confidence: 99%

Skin mast cells in Marfan syndrome: specific emphasis on connective tissue remodeling

Atiakshin,

Nikolaeva,

Gritsevskaya

et al. 2024

Arch Dermatol Res

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Marfan syndrome (MFS) is a hereditary condition accompanied by disorders in the structural and regulatory properties of the connective tissue, including elastic bers, due to a mutation in the FBN1 gene and the synthesis of abnormal brillin 1 glycoprotein. Despite the high potential of mast cells (MCs) to remodel the extracellular matrix (ECM), their pathogenetic signi cance in MFS has not been consideredyet. An analysis of the skin MC population in children with Marfan syndrome revealed a considerably increased number of intraorganic populations with preservation of the speci c protease Tryptase + Chymase + CPA3 + pro le typical of the skin. The features of the MC histotopography phenotype in MFS consisted of closer colocalization with elastic bers, smooth muscle cells and broblasts. MCs formed many intradermal clusters that synchronized the activity of cell functions in the stromal landscape of the tissue microenvironment with the help of spatial architectonics, including the formation of cell chains and the creation of brous niches. In MCs, the expression of speci c proteases, TGF-β and heparin increased with targeted secretion of biologically active substances to the dermal elastic bers, which, in MFS, had speci c structural features, including abnormal variability in thickness along the entire length, alternation of thickened and thinned areas, and uneven surface topography. The paper discusses the potential role of MCs in strain analysis (tensometry) of the tissue microenvironment in MFS. Thus, quantitative and qualitative rearrangements of the skin MC population in MFS are aimed at altering the stromal landscape of the connective tissue. The results obtained should be taken into account when managing clinical signs of MFS manifested in other pathogenetically critical structures of internal organs, including the aorta, tendons, cartilage and parenchymal organs.

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Connective Tissue Disorders and Cardiovascular Complications: The Indomitable Role of Transforming Growth Factor-β Signaling

Cited by 11 publications

References 143 publications

Transforming growth factor serum concentrations in patients with proven non-syndromic aortopathy

Transforming growth factor serum concentrations in patients with proven non-syndromic aortopathy

Navigating toward gene therapy in Marfan syndrome: A hope for halting aortic aneurysm

Skin mast cells in Marfan syndrome: specific emphasis on connective tissue remodeling

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