PhD Scientific Days 2023

Molecular Sciences - Posters L

Nanoscale structural comparison of fibrillin-1 microfibrils isolated from Marfan and non-Marfan syndrome human aorta

Előadó neve

Dr. Sulea, Cristina

Neptun code

KJMBP4

Előadó munkahelye

Department of Biophysics and Radiation Biology, Semmelweis University

Előadó telefonszáma

06206663356

Előadó e-mail címe

cristinasulea@gmail.com

Az előadás címe

Nanoscale structural comparison of fibrillin-1 microfibrils isolated from Marfan and non-Marfan syndrome human aorta

Szerző(k) neve és munkahelye

Cristina M. Sulea1,2,3, Zsolt Mártonfalvi1, Csilla Csányi1, Dóra Haluszka1, Miklós Pólos2,3, Bence Ágg2,3,4, Roland Stengl2,3, Kálmán Benke2,3,5, Zoltán Szabolcs2,3, Miklós S. Z. Kellermayer1
1 Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary
2 Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary
3 Hungarian Marfan Foundation, 1122 Budapest, Hungary
4 Department of Pharmacology and Pharmacotherapy, Semmelweis University, 1089 Budapest, Hungary
5 Department of Cardiac Surgery, University Hospital Halle (Saale), 06120 Halle (Saale), Germany

Bemutatás módja

Poszter

Szekció

Molecular Sciences - Posters L

Language of the presentation

English

Preferred session

Molecular Sciences

Összefoglaló szövege

Introduction: Fibrillin-1 microfibrils are essential elements of the extracellular matrix serving as a scaffold for the deposition of elastin and endowing connective tissues with tensile strength and elasticity. Mutations in the fibrillin-1 gene (FBN1) are linked to Marfan syndrome (MFS), a systemic connective tissue disorder that, besides other heterogeneous symptoms, usually manifests in life-threatening aortic complications. The aortic involvement may be explained by a dysregulation of microfibrillar function and, conceivably, alterations in the microfibrils’ supramolecular structure.
Aims: To perform a nanoscale structural characterization of fibrillin-1 microfibrils isolated from human aortic samples with different FBN1 gene mutations and to compare them with microfibrillar assemblies purified from non-MFS human aortic samples.
Methods: Aortic wall samples were obtained from patients undergoing specific cardiovascular surgical interventions. Fibrillin-rich microfibrils were extracted by bacterial collagenase digestion and purified by size-exclusion chromatography. Atomic force microscopy was employed to visualize and study the microfibrillar assemblies.
Results: Fibrillin-1 microfibrils displayed a characteristic “beads-on-a-string” appearance. The microfibrillar assemblies were investigated for bead geometry (height, length, and width), interbead region height, and periodicity. MFS fibrillin-1 microfibrils had a slightly higher mean bead height, but the bead length and width, as well as the interbead height, were significantly smaller in the MFS group. The mean periodicity varied around 50–52 nm among samples.
Conclusion: The data suggest an overall thinner and presumably more frail structure for the MFS fibrillin-1 microfibrils, which may play a role in the development of MFS-related aortic symptomatology.
Funding: This research was supported by grants from the Hungarian National Research, Development and Innovation Office (K135360 to M.S.Z.K. and project TKP2021-EGA-23) and the New National Excellence Program of the Ministry for Culture and Innovation from the source of the National Research, Development, and Innovation Fund (ÚNKP-22-3-I-SE-49 to C.M.Ș.). Project no. RRF-2.3.1-21-2022-00003 (National Cardiovascular Laboratory) has been implemented with the support provided by the European Union.

University and Doctoral School

Semmelweis University, Doctoral School of Theoretical and Translational Medicine

Supervisor

Prof. Zoltán Szabolcs M.D., Ph.D. and Prof. Miklós S. Z. Kellermayer 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

11:00

End

11:05

Authors (legacy)

Cristina M. Sulea1,2,3, Zsolt Mártonfalvi1, Csilla Csányi1, Dóra Haluszka1, Miklós Pólos2,3, Bence Ágg2,3,4, Roland Stengl2,3, Kálmán Benke2,3,5, Zoltán Szabolcs2,3, Miklós S. Z. Kellermayer1
1 Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary
2 Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary
3 Hungarian Marfan Foundation, 1122 Budapest, Hungary
4 Department of Pharmacology and Pharmacotherapy, Semmelweis University, 1089 Budapest, Hungary
5 Department of Cardiac Surgery, University Hospital Halle (Saale), 06120 Halle (Saale), Germany