Increased elastic microfibrils and thickening of fibroblastic nuclear lamina in canine cutaneous asthenia

dc.contributor.authorBellini, M. H.
dc.contributor.authorCaldini, E. T. E. G.
dc.contributor.authorScapinelli, M. P.
dc.contributor.authorSimoes, M. J. [UNIFESP]
dc.contributor.authorMachado, D. B.
dc.contributor.authorNurmberg, R.
dc.contributor.institutionIPEN CNEN SP
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.date.accessioned2016-01-24T13:52:26Z
dc.date.available2016-01-24T13:52:26Z
dc.date.issued2009-04-01
dc.description.abstractCutaneous asthenia is a hereditary connective tissue disease, primarily of dogs and cats, resembling Ehlers-Danlos syndrome in man. Collagen dysplasia results in skin hyperextensibility, skin and vessel fragility, and poor wound healing. the purpose of this study was to describe the histological findings in a dog with a collagenopathy consistent with cutaneous asthenia. An 8-month-old crossbreed female dog presented with lacerations and numerous atrophic and irregular scars. the skin was hyperextensible and easily torn by the slightest trauma. Ultrastructurally, the dermis was comprised of elaunin and oxytalan microfibrils. These are immature fibres in which the fibrillar component is increased but elastin is reduced. Collagen fibres were profoundly disorganized. the fibrils had a highly irregular outline and a corroded appearance when viewed in cross-section, and were spiralled and fragmented in a longitudinal view. Dermal fibroblasts displayed a conspicuous thickening of the nuclear lamina. Nuclear lamins form a fibrous nucleoskeletal network of intermediate-sized filaments underlying the inner nuclear membrane. Mutations in lamins or lamin-associated proteins cause a myriad of genetic diseases collectively called laminopathies. Disruption of the nuclear lamina seems to affect chromatin organization and transcriptional regulation of gene expression. A common link among all laminopathies may be a failure of stem cells to regenerate mesenchymal tissue. This could contribute to the connective tissue dysplasia seen in cutaneous asthenia.en
dc.description.affiliationIPEN CNEN SP, BR-05508000 São Paulo, Brazil
dc.description.affiliationUniv São Paulo, BR-05508 São Paulo, Brazil
dc.description.affiliationUniversidade Federal de São Paulo, EPM, São Paulo, Brazil
dc.description.affiliationUnifespUniversidade Federal de São Paulo, EPM, São Paulo, Brazil
dc.description.sourceWeb of Science
dc.description.sponsorshipFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.description.sponsorshipIDFAPESP: 07/51204-4
dc.format.extent139-143
dc.identifierhttp://dx.doi.org/10.1111/j.1365-3164.2008.00732.x
dc.identifier.citationVeterinary Dermatology. Malden: Wiley-Blackwell Publishing, Inc, v. 20, n. 2, p. 139-143, 2009.
dc.identifier.doi10.1111/j.1365-3164.2008.00732.x
dc.identifier.issn0959-4493
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/31442
dc.identifier.wosWOS:000264188500012
dc.language.isoeng
dc.publisherWiley-Blackwell
dc.relation.ispartofVeterinary Dermatology
dc.rightsinfo:eu-repo/semantics/restrictedAccess
dc.rights.licensehttp://olabout.wiley.com/WileyCDA/Section/id-406071.html
dc.titleIncreased elastic microfibrils and thickening of fibroblastic nuclear lamina in canine cutaneous astheniaen
dc.typeinfo:eu-repo/semantics/article
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