title: Limited impact of fibromodulin deficiency on the development of experimental skin fibrosis.
abstract: Excessive production of collagen is the hallmark of fatal diseases of fibrosis such as systemic sclerosis. Overexpression of the proteoglycan fibromodulin (FMOD) has been associated with improved wound healing and scarless repair. In this study, we have investigated the consequences of FMOD deficiency on the development of experimental skin fibrosis. Using immunohistochemistry, we identified FMOD in both human and murine fibrotic skin. In the bleomycin model of skin fibrosis, FMOD(-/-) mice developed skin fibrosis to a similar degree compared to FMOD(+/+) mice. Analysis of skin ultrastructure using transmission electron microscopy revealed a significant reduction in collagen fibril diameter in FMOD(-/-) but not FMOD(+/+) mice following fibrosis. We conclude that the impact of FMOD deficiency on the development of experimental skin fibrosis is limited.
authors.affiliation: Department of Clinical Sciences Lund, Section of Rheumatology and Molecular Skeletal Biology, Lund University, Lund, Sweden.
authors.forename: Kristofer
authors.initials: K
authors.lastname: Andréasson
authors.affiliation: Department of Experimental Medical Science, Lund University, Lund, Sweden.
authors.forename: Renata
authors.initials: R
authors.lastname: Gustafsson
authors.affiliation: Department of Experimental Medical Science, Lund University, Lund, Sweden.
authors.forename: Kristina
authors.initials: K
authors.lastname: Rydell-Törmänen
authors.affiliation: Department of Experimental Medical Science, Lund University, Lund, Sweden.
authors.forename: Gunilla
authors.initials: G
authors.lastname: Westergren-Thorsson
authors.affiliation: Department of Clinical Sciences Lund, Section of Rheumatology and Molecular Skeletal Biology, Lund University, Lund, Sweden.
authors.forename: Tore
authors.initials: T
authors.lastname: Saxne
authors.affiliation: Department of Clinical Sciences Lund, Section of Rheumatology and Molecular Skeletal Biology, Lund University, Lund, Sweden.
authors.forename: Roger
authors.initials: R
authors.lastname: Hesselstrand
mesh_terms: D000293:Adolescent; D000818:Animals; D001761:Bleomycin; D016022:Case-Control Studies; D002648:Child; D004195:Disease Models, Animal; D000071235:Fibromodulin / Q000172:deficiency*; D005355:Fibrosis; D006801:Humans; D008810:Mice, Inbred C57BL; D012595:Scleroderma, Systemic / Q000209:etiology / Q000378:metabolism*; D012867:Skin / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure
chemical_list: C000604330:FMOD protein, human; C000604332:Fmod protein, mouse; D001761:Bleomycin; D000071235:Fibromodulin
keywords: bleomycin; collagen; extracellular matrix; skin; systemic sclerosis