Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas

Abstract 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 cha...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Sabine Kling, Emilio A. Torres-Netto, Hormoz Abdshahzadeh, Edgar M. Espana, Farhad Hafezi
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/d51032ee07ed4614b016d10a480413b9
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:d51032ee07ed4614b016d10a480413b9
record_format	dspace
spelling	oai:doaj.org-article:d51032ee07ed4614b016d10a480413b92021-12-02T16:38:25ZCollagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas10.1038/s41598-021-96775-w2045-2322https://doaj.org/article/d51032ee07ed4614b016d10a480413b92021-08-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-96775-whttps://doaj.org/toc/2045-2322Abstract 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.Sabine KlingEmilio A. Torres-NettoHormoz AbdshahzadehEdgar M. EspanaFarhad HafeziNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-8 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Sabine Kling Emilio A. Torres-Netto Hormoz Abdshahzadeh Edgar M. Espana Farhad Hafezi Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas
description	Abstract 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	article
author	Sabine Kling Emilio A. Torres-Netto Hormoz Abdshahzadeh Edgar M. Espana Farhad Hafezi
author_facet	Sabine Kling Emilio A. Torres-Netto Hormoz Abdshahzadeh Edgar M. Espana Farhad Hafezi
author_sort	Sabine Kling
title	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_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_sort	collagen v insufficiency in a mouse model for ehlers danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/d51032ee07ed4614b016d10a480413b9
work_keys_str_mv	AT sabinekling collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT emilioatorresnetto collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT hormozabdshahzadeh collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT edgarmespana collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas AT farhadhafezi collagenvinsufficiencyinamousemodelforehlersdanlossyndromeaffectsviscoelasticbiomechanicalpropertiesexplainingthinandbrittlecorneas
_version_	1718383602812583936

Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas

Ejemplares similares