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

Author: AissatAbdel, CostesBruno, DelattreValérie, DelestrainCéline, DupratElodie, EpaudRalph, FanenPascale, FinetStéphanie, NattesElodie, SimonStéphanie, TarzeAgathe

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Overview

Glucocorticosteroid (GC) treatment effectively improves proSP-C processing and reduces SFTPC mRNA levels in twins with a BRICHOS mutation in the SP-C gene, potentially by favoring the expression of a less stable proSP-C isoform.
Paper Details　
Original Abstract of the Article :
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.Cys189T...See full text at original site
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引用元：
https://doi.org/10.1111/bcp.14645

データ提供：米国国立医学図書館（NLM）

Methylprednisolone Pulse Treatment Improves ProSP-C Trafficking in Twins with SFTPC Mutation

The desert of interstitial lung disease (ILD) research is a vast and complex landscape, with researchers constantly seeking to unravel the mysteries of this debilitating condition. This study focuses on the effects of methylprednisolone pulse treatment in twins carrying a mutation in the surfactant protein C (SP-C) gene (SFTPC). The researchers aimed to investigate the mechanisms behind the successful treatment with glucocorticosteroids in these twins, exploring the potential role of methylprednisolone in improving proSP-C trafficking and overall lung function.

Methylprednisolone Pulse Treatment: A Potential Therapy for SFTPC Mutations

This study, like a caravan navigating a treacherous path through the desert, reveals a potential mechanism by which methylprednisolone pulse treatment might benefit twins with SFTPC mutations. The researchers found that methylprednisolone pulse treatment improved proSP-C trafficking in these twins, potentially contributing to improved lung function. These findings offer a glimmer of hope for individuals with SFTPC mutations and highlight the potential benefits of glucocorticosteroid therapy in this context.

Navigating the Desert of SFTPC Mutations

This research, like a beacon of light in the desert, emphasizes the importance of understanding the molecular mechanisms underlying SFTPC mutations and the potential therapeutic interventions that can improve proSP-C trafficking and lung function. The study's findings provide valuable insights into the potential role of methylprednisolone pulse treatment in managing SFTPC mutations, offering a promising path towards developing effective therapies for this challenging condition.

Dr. Camel's Conclusion

This study, like a camel caravan seeking an oasis of knowledge, provides a valuable glimpse into the potential of methylprednisolone pulse treatment in improving proSP-C trafficking in twins with SFTPC mutations. The study's findings offer a promising avenue for further research and development of effective therapies for this debilitating condition.

Date :
  1. Date Completed 2021-09-20
  2. Date Revised 2022-05-31
Further Info :

Pubmed ID

33179299

DOI: Digital Object Identifier

10.1111/bcp.14645

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Article Analysis
・Effective treatment of twins
SNS
PICO Info
in preparation
Languages

English

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