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miR-29 Regulates Type VII Collagen in Recessive Dystrophic Epidermolysis Bullosa.
Vanden Oever, Michael; Muldoon, Daniel; Mathews, Wendy; McElmurry, Ron; Tolar, Jakub.
Afiliación
  • Vanden Oever M; Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota, USA.
  • Muldoon D; Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota, USA.
  • Mathews W; Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota, USA.
  • McElmurry R; Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota, USA.
  • Tolar J; Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota, USA; Stem Cell Institute, University of Minnesota, Minneapolis, Minnesota, USA. Electronic address: tolar003@umn.edu.
J Invest Dermatol ; 136(10): 2013-2021, 2016 10.
Article en En | MEDLINE | ID: mdl-27328306
Recessive dystrophic epidermolysis bullosa (RDEB) is a complex inherited skin disorder caused by loss-of-function mutations in the COL7A1 gene. For an effective treatment of this disorder to be realized, both a thorough understanding of the regulation of COL7A1 and an understanding of the underlying nature of the complications of RDEB are needed. Currently, both posttranscriptional regulation of COL7A1 and the underlying causes of fibrosis in RDEB patients are poorly understood. Here, we describe a mechanism of regulation, to our knowledge previously unknown, by which micro RNA-29 (miR-29) regulates COL7A1 in a complex network: both directly through targeting its 3' untranslated region at two distinct seed regions and indirectly through targeting an essential transcription factor required for basal COL7A1 expression, SP1. In turn, miR-29 itself is regulated by SP1 activity and transforming growth factor-ß signaling. RDEB mice express high levels of transforming growth factor-ß and significantly lower miR-29 compared with wild-type cohorts. The sustained decrease in miR-29 in RDEB skin leads to an increase of miR-29 target genes expressed in the skin, including profibrotic extracellular matrix collagens. Collectively, we identify miR-29 as an important factor in both regulating COL7A1 and inhibiting transforming growth factor-ß-mediated fibrosis.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Epidermólisis Ampollosa Distrófica / Factor de Crecimiento Transformador beta / Colágeno Tipo VII / MicroARNs Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Revista: J Invest Dermatol Año: 2016 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Epidermólisis Ampollosa Distrófica / Factor de Crecimiento Transformador beta / Colágeno Tipo VII / MicroARNs Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Revista: J Invest Dermatol Año: 2016 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos