PhD Scientific Days 2022

Translational Medicine II. (Poster discussion will take place in the Aula during the Coffee Break)

Nanoscale features of Marfan and non-Marfan aorta-derived fibrillin-1 microfibrils

Előadó neve

Dr. Șulea, Cristina M.

Előadó munkahelye

Department of Cardiac Surgery, Semmelweis University Heart and Vascular Center, Budapest, Hungary

Előadó telefonszáma

+36705776688

Előadó e-mail címe

cristinasulea@gmail.com

Az előadás címe

Nanoscale features of Marfan and non-Marfan aorta-derived fibrillin-1 microfibrils

Szerző(k) neve és munkahelye

C.M. Șulea1,2,3, Zs. Mártonfalvi4, M.Cs. Csányi4, D. Haluszka4, M. Pólos2,3, K. Benke2,3, Z. Szabolcs2,3, M.S.Z. Kellermayer4
1 Semmelweis University, Doctoral School of Theoretical and Translational Medicine, Budapest, Hungary
2 Department of Cardiac Surgery, Semmelweis University Heart and Vascular Center, Budapest, Hungary
3 Hungarian Marfan Foundation, Budapest, Hungary
4 Semmelweis University, Department of Biophysics and Radiation Biology, Budapest, Hungary

Bemutatás módja

Poszter

Szekció

Translational Medicine II. (Poster discussion will take place in the Aula during the Coffee Break)

Language of the presentation

English

Preferred session

Theoretical and Translational Medicine

Összefoglaló szövege

Introduction: Fibrillin microfibrils are essential structures of the extracellular matrix that play a fundamental role in multiple physiological functions of connective tissues. They are abundant in highly dynamic tissues, such as the aorta, where fibrillin-1 microfibrils are involved in vascular elasticity. Their biological relevance is reflected by the linkage of mutations in the fibrillin-1-encoding FBN1 gene to a group of connective tissue disorders known as fibrillinopathies, the most notable of which is Marfan syndrome, which is associated with skeletal, ocular and cardiovascular defects. Aortic involvement, in the form of aortic dilation, dissection, or rupture, represents the main cause of mortality among Marfan syndrome patients.
Aims: To assess and compare the structural and mechanical characteristics of fibrillin-1 microfibrils isolated from human Marfan and non-Marfan aortic tissue.
Method: Aortic wall samples were obtained from patients undergoing specific cardiovascular surgical interventions. The deposition of fibrillin-1 in the extracellular matrix was analyzed by using two-photon microscopy. Fibrillin-rich microfibrils were extracted by bacterial collagenase digestion and purified by size-exclusion chromatography. Atomic force microscopy was used to visualize and study the microfibrillar assemblies.
Results: In both groups, fibrillin-1 microfibrils of various lengths were observed, exhibiting the typical “beads-on-a-string” appearance. A mean periodicity of approximately 52 nm was recorded. Following initial measurements, no significant differences regarding nanostructural features were identified between the two groups.
Conclusion: We theorize that mutations in the FBN1 gene lead to nanomechanical variations in the Marfan fibrillin-1 microfibrils rather than morphological modifications. Further measurements are expected to reveal the microfibril-level characteristics that lead to the development symptoms involving the Marfan aorta.
Funding: This research was funded by a grant from the Hungarian National Research, Development and Innovation Office (K135360).

University and Doctoral School

Semmelweis University, Doctoral School of Theoretical and Translational Medicine

Supervisor

Prof. Szabolcs Zoltán M.D., Ph.D.

Publication of my abstract

I give consent to the publication of my abstract on the website of the congress.

Kind

Szabad

Status

elfogadva

Accepted presentation method

poszter

Előadás fájl jóváhagyás

nem rendelkezett róla

Előadó

6939

Start

13:45

End

13:50

Authors (legacy)

C.M. Șulea1,2,3, Zs. Mártonfalvi4, M.Cs. Csányi4, D. Haluszka4, M. Pólos2,3, K. Benke2,3, Z. Szabolcs2,3, M.S.Z. Kellermayer4
1 Semmelweis University, Doctoral School of Theoretical and Translational Medicine, Budapest, Hungary
2 Department of Cardiac Surgery, Semmelweis University Heart and Vascular Center, Budapest, Hungary
3 Hungarian Marfan Foundation, Budapest, Hungary
4 Semmelweis University, Department of Biophysics and Radiation Biology, Budapest, Hungary