Cargando…
Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas
Ehlers–Danlos syndrome (EDS) is a genetic disease leading to abnormalities in mechanical properties of different tissues. Here we quantify corneal biomechanical properties in an adult classic EDS mouse model using two different measurement approaches suited for murine corneal mechanical characteriza...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8405695/ https://www.ncbi.nlm.nih.gov/pubmed/34462473 http://dx.doi.org/10.1038/s41598-021-96775-w |
_version_ | 1783746380878053376 |
---|---|
author | Kling, Sabine Torres-Netto, Emilio A. Abdshahzadeh, Hormoz Espana, Edgar M. Hafezi, Farhad |
author_facet | Kling, Sabine Torres-Netto, Emilio A. Abdshahzadeh, Hormoz Espana, Edgar M. Hafezi, Farhad |
author_sort | Kling, Sabine |
collection | PubMed |
description | Ehlers–Danlos syndrome (EDS) is a genetic disease leading to abnormalities in mechanical properties of different tissues. Here we quantify corneal biomechanical properties in an adult classic EDS mouse model using two different measurement approaches suited for murine corneal mechanical characterization and relate differences to stromal structure using Second Harmonic Generation (SHG) microscopy. Quasi-static Optical Coherence Elastography (OCE) was conducted non-invasively during ambient pressure modulation by − 3 mmHg. 2D-extensometry measurements was conducted invasively consisting of a pre-conditioning cycle, a stress-relaxation test and a rupture test. In a total of 28 eyes from a Col5a1(+/−) mouse model and wild-type C57BL/6 littermates (wt), Col5a1(+/−) corneas were thinner when compared to wt, (125 ± 11 vs 148 ± 10 μm, respectively, p < 0.001). Short-term elastic modulus was significantly increased in OCE (506 ± 88 vs 430 ± 103 kPa, p = 0.023), and the same trend was observed in 2D-extensometry (30.7 ± 12.1 kPa vs 21.5 ± 5.7, p = 0.057). In contrast, in stress relaxation tests, Col5a1(+/−) corneas experienced a stronger relaxation (55% vs 50%, p = 0.01). SHG microscopy showed differences in forward and backward scattered signal indicating abnormal collagen fibrils in Col5a1(+/−) corneas. We propose that disturbed collagen fibril structure in Col5a1(+/−) corneas affects the viscoelastic properties. Results presented here support clinical findings, in which thin corneas with global ultrastructural alterations maintain a normal corneal shape. |
format | Online Article Text |
id | pubmed-8405695 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-84056952021-09-01 Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas Kling, Sabine Torres-Netto, Emilio A. Abdshahzadeh, Hormoz Espana, Edgar M. Hafezi, Farhad Sci Rep Article Ehlers–Danlos syndrome (EDS) is a genetic disease leading to abnormalities in mechanical properties of different tissues. Here we quantify corneal biomechanical properties in an adult classic EDS mouse model using two different measurement approaches suited for murine corneal mechanical characterization and relate differences to stromal structure using Second Harmonic Generation (SHG) microscopy. Quasi-static Optical Coherence Elastography (OCE) was conducted non-invasively during ambient pressure modulation by − 3 mmHg. 2D-extensometry measurements was conducted invasively consisting of a pre-conditioning cycle, a stress-relaxation test and a rupture test. In a total of 28 eyes from a Col5a1(+/−) mouse model and wild-type C57BL/6 littermates (wt), Col5a1(+/−) corneas were thinner when compared to wt, (125 ± 11 vs 148 ± 10 μm, respectively, p < 0.001). Short-term elastic modulus was significantly increased in OCE (506 ± 88 vs 430 ± 103 kPa, p = 0.023), and the same trend was observed in 2D-extensometry (30.7 ± 12.1 kPa vs 21.5 ± 5.7, p = 0.057). In contrast, in stress relaxation tests, Col5a1(+/−) corneas experienced a stronger relaxation (55% vs 50%, p = 0.01). SHG microscopy showed differences in forward and backward scattered signal indicating abnormal collagen fibrils in Col5a1(+/−) corneas. We propose that disturbed collagen fibril structure in Col5a1(+/−) corneas affects the viscoelastic properties. Results presented here support clinical findings, in which thin corneas with global ultrastructural alterations maintain a normal corneal shape. Nature Publishing Group UK 2021-08-30 /pmc/articles/PMC8405695/ /pubmed/34462473 http://dx.doi.org/10.1038/s41598-021-96775-w Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Article Kling, Sabine Torres-Netto, Emilio A. Abdshahzadeh, Hormoz Espana, Edgar M. Hafezi, Farhad Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
title | Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
title_full | Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
title_fullStr | Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
title_full_unstemmed | Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
title_short | Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
title_sort | collagen v insufficiency in a mouse model for ehlers danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8405695/ https://www.ncbi.nlm.nih.gov/pubmed/34462473 http://dx.doi.org/10.1038/s41598-021-96775-w |
work_keys_str_mv | AT klingsabine collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT torresnettoemilioa collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT abdshahzadehhormoz collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT espanaedgarm collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT hafezifarhad collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas |