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

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

Author Bellini, M. H. Google Scholar
Caldini, E. T. E. G. Google Scholar
Scapinelli, M. P. Google Scholar
Simoes, M. J. Autor UNIFESP Google Scholar
Machado, D. B. Google Scholar
Nurmberg, R. Google Scholar
Institution IPEN CNEN SP
Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
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.
Language English
Sponsor Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
Grant number FAPESP: 07/51204-4
Date 2009-04-01
Published in Veterinary Dermatology. Malden: Wiley-Blackwell Publishing, Inc, v. 20, n. 2, p. 139-143, 2009.
ISSN 0959-4493 (Sherpa/Romeo, impact factor)
Publisher Wiley-Blackwell
Extent 139-143
Access rights Closed access
Type Article
Web of Science ID WOS:000264188500012

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