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Título:	GSE4 reverts fibrosis in bleomycin treated rats with Idipathic pulmonary fibrosis
Autor:	Cortijo, Julio; Manguan-García, Cristina CSIC ORCID; Pintado-Berninches, Laura CSIC ORCID ; Sastre, Leandro CSIC ORCID; Egusquiaguirre, Susana P.; Igartua, Manuela; Perona Abellón, Rosario CSIC ORCID
Fecha de publicación:	2015
Citación:	VIII Reunión Anual CIBERER (2015)
Resumen:	Pulmonary fibrosis (sporadic-IPF and familial pulmonary fibrosis-FPF) has in common an altered wound healing that induces a tissue elderly aging and a devastating morbility and mortality in adults (mean survival 3-4 years from diagnosis). Two proteins parts of the telomerase complex have been found mutated in both IPF and FPF. We have approach the development of a therapy for idiopathic pulmonary fibrosis (IPF-FPF) based on the reactivation of the telomerase activity. GSE24-2 an internal domain of dyskerin is able to reactivate telomerase activity in DKC-mutated cells derived from patients with dyskeratosis congenita (DC). This molecule has been recently approved by EMA as an orphan drug for the treatment of this disease (Gestelmir). We have use a rat model for IPF by intratracheal administration of bleomycin. Administration of PLGA/PEI nanoparticles loaded with a shorter derived peptide of GSE24.2 called GSE4, is able to both prevent and revert fibrosis in the bleomycin treated rats by image CT. Quantification of SPECT comparative images between IPF and IPF GSE4 indicate that there is a bad distribution of the radiocontrast in IPF that is reverted by GSE4. Examination of the lung lobules in the treated animals reveal an histological reversion of the colagen deposition when the rats are treated with GSE4 loaded nanoparticles.
Descripción:	Resumen del trabajo presentado a la VIII Reunión Científica Anual del Centro de Investigación Biomédica En Red de Enfermedades Raras, celebrada en San Lorenzo del Escorial (Madrid) los días 12 y 13 de marzo de 2015.-- et al.
URI:	http://hdl.handle.net/10261/152745
Aparece en las colecciones:	(IIBM) Comunicaciones congresos