ArticleThe Journal of cell biology1998
The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils.
Article in The Journal of cell biology, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
31 citing papers in PubMed, 6 citations in OpenAlex.
- Article
- Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice.Matrix biology : journal of the International Society for Matrix Biology · 2025Article
- Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis.Scientific reports · 2024Article
- Concomitant uterus agenesia and Marfan syndrome with systemic sclerosis: A rare case report.Clinical case reports · 2024Article
- Increased Susceptibility to Glaucomatous Damage in Microfibril Deficient Mice.Investigative ophthalmology & visual science · 2020Article
- Gelatinolytic activity in gingival crevicular fluid and saliva of growing patients with Marfan syndrome: a case-control study.BMC oral health · 2019Article
- Structural and compositional diversity of fibrillin microfibrils in human tissues.The Journal of biological chemistry · 2018Article
- Proteinase activated receptor-2 counterbalances the vascular effects of endothelin-1 in fibrotic tight-skin mice.British journal of pharmacology · 2017Article
- Systemic sclerosis sera affect fibrillin-1 deposition by dermal blood microvascular endothelial cells: therapeutic implications of cyclophosphamide.Arthritis research & therapy · 2013Article
- Genetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and function.Cellular and molecular life sciences : CMLS · 2011Review
- Structure-function relations in an elastase-induced mouse model of emphysema.American journal of respiratory cell and molecular biology · 2011Article
- Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome.Human molecular genetics · 2010Article
- Fibrillin assemblies: extracellular determinants of tissue formation and fibrosis.Fibrogenesis & tissue repair · 2010Article
- Article
- Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome.Science translational medicine · 2010Article
- Clinical significance of serum HMGB-1 and sRAGE levels in systemic sclerosis: association with disease severity.Journal of clinical immunology · 2009Article
- Radiation-induced skin injury in the animal model of scleroderma: implications for post-radiotherapy fibrosis.Radiation oncology (London, England) · 2008Article
- Early emphysema in the tight skin and pallid mice: roles of microfibril-associated glycoproteins, collagen, and mechanical forces.American journal of respiratory cell and molecular biology · 2006Article
- Animal models in scleroderma.Current rheumatology reports · 2005Review
- Autoantibodies to a collagen-specific molecular chaperone, heat-shock protein 47, in systemic sclerosis.Clinical and experimental immunology · 2004Article
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
Mice carrying the Tight skin (Tsk) mutation harbor a genomic duplication within the fibrillin-1 (Fbn 1) gene that results in a larger than normal in-frame Fbn 1 transcript. In this study, the consequences of the Tsk mutation for fibrillin-containing microfibrils have been examined. Dermal fibroblasts from Tsk/+ mice synthesized and secreted both normal fibrillin (approximately 330 kD) and the mutant oversized Tsk fibrillin-1 (approximately 450 kD) in comparable amounts, and Tsk fibrillin-1 was stably incorporated into cell layers. Immunohistochemical and ultrastructural analyses of normal and Tsk/+ mouse skin highlighted differences in the gross organization and distribution of microfibrillar arrays. Rotary shadowing of high Mr preparations from Tsk/+ skin demonstrated the presence of abundant beaded microfibrils. Some of these had normal morphology and periodicity, but others were distinguished by diffuse interbeads, longer periodicity, and tendency to aggregate. The presence of a structurally abnormal population of microfibrils in Tsk/+ skin was unequivocally demonstrated after calcium chelation and in denaturating conditions. Scanning transmission electron microscopy highlighted the presence of more mass in Tsk/+ skin microfibrils than in normal mice skin microfibrils. These data indicate that Tsk fibrillin-1 polymerizes and becomes incorporated into a discrete population of beaded microfibrils with altered molecular organization.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.