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Methylprednisolone pulse treatment improves ProSP-C trafficking in twins with SFTPC mutation: An isoform story?

British journal of clinical pharmacology - 1 May 2021

Delestrain Céline, Aissat Abdel, Simon Stéphanie, Tarze Agathe, Duprat Elodie, Nattes Elodie, Costes Bruno, Delattre Valérie, Finet Stéphanie, Fanen Pascale, Epaud Ralph

Abstract excerpt

Mutations in the gene encoding surfactant protein C (SP-C) cause interstitial lung disease (ILD), and glucocorticosteroid (GC) treatment is the most recognized therapy in children. We aimed to decipher the mechanisms behind successful GC treatment in twins carrying a BRICHOS c.566G > A (p.Cys189Tyr) mutation in the SP-C gene (SFTPC). METHODS: The twins underwent bronchoscopy before and after GC treatment and...

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PMID: 33179299
DOI: 10.1111/bcp.14645

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