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

Show simple item record Bellini, M. H. Caldini, E. T. E. G. Scapinelli, M. P. Simoes, M. J. [UNIFESP] Machado, D. B. Nurmberg, R. 2016-01-24T13:52:26Z 2016-01-24T13:52:26Z 2009-04-01
dc.identifier.citation Veterinary Dermatology. Malden: Wiley-Blackwell Publishing, Inc, v. 20, n. 2, p. 139-143, 2009.
dc.identifier.issn 0959-4493
dc.description.abstract Cutaneous 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.sponsorship Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.format.extent 139-143
dc.language.iso eng
dc.publisher Wiley-Blackwell
dc.relation.ispartof Veterinary Dermatology
dc.rights Acesso restrito
dc.title Increased elastic microfibrils and thickening of fibroblastic nuclear lamina in canine cutaneous asthenia en
dc.type Artigo
dc.contributor.institution IPEN CNEN SP
dc.contributor.institution Universidade de São Paulo (USP)
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.description.affiliation IPEN CNEN SP, BR-05508000 São Paulo, Brazil
dc.description.affiliation Univ São Paulo, BR-05508 São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, EPM, São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, EPM, São Paulo, Brazil
dc.description.sponsorshipID FAPESP: 07/51204-4
dc.identifier.doi 10.1111/j.1365-3164.2008.00732.x
dc.description.source Web of Science
dc.identifier.wos WOS:000264188500012


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