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Association of Serum TGF-β1 Levels with Different Clinical Phenotypes of Cystic Fibrosis Exacerbation

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Abstract

Purpose

Cystic Fibrosis (CF) is a multi-organ genetic disorder and Transforming Growth Factor (TGF-β1) is a modifier gene which modulates lung pathology in CF. There is great phenotypic variability among CF patients who even have similar genotype. The aim of the present study was to associate the serum levels of TGF-β1 with several clinical phenotypes of CF.

Methods

The diagnosed cases of CF were recruited and the blood sample was withdrawn at different time points: during exacerbation (n = 26), non-exacerbation (n = 9) and after antibiotic therapy (n = 11). The concentration of the total TGF-β1 in serum was measured with commercial ELISA kit. The ΔF508 mutation was assessed by the Amplification Refractory Mutation System (ARMS-PCR).

Results

The levels of TGF-β1 were increased in exacerbation phase (119.89 ± 29.64 ng/mL), infection with P. aeruginosa (121.8 ± 28.83 ng/mL) and in subjects with ΔF508 mutation (139.2 ± 19.59 ng/mL). The levels of TGF-β1 in CF patients with Allergic Bronchopulmonary Aspergillosis (ABPA) (109.97 ± 27.71 ng/mL) were decreased as compared to CF patients without ABPA (123.55 ± 30.20 ng/mL). It was observed that the serum levels of TGF-β1 were decreased significantly after antibiotic therapy (p < 0.05).

Conclusions

The present study has determined that the serum levels of TGF-β1 vary with the type of infections, ΔF508 CFTR mutation, presence of ABPA and response to therapy.

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Acknowledgement

Indian Council of Medical Research, New Delhi.

Funding

The study was supported by Indian Council of Medical Research, New Delhi through grant no: ICMR JRF No. 3/1/3/JRF-2012/HRD-08 (10835).

Author information

SS was involved in conceptualizing the research proposal, performed all experimental procedures and wrote the manuscript. AC was involved in genetic analysis of CFTR mutation and wrote the manuscript. JK and RP provided the laboratory facilities and helped in analysing the data. MS (Meenu Singh) was responsible for patient recruitment, obtaining informed consent and data analysis. MS (Manvi Singh) was involved in patient recruitment.

Correspondence to Meenu Singh.

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The authors declare that they have no conflict of interest.

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Sagwal, S., Chauhan, A., Kaur, J. et al. Association of Serum TGF-β1 Levels with Different Clinical Phenotypes of Cystic Fibrosis Exacerbation. Lung (2020) doi:10.1007/s00408-020-00320-x

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Keywords

  • Cystic fibrosis
  • TGF-β
  • Exacerbation
  • ABPA
  • ΔF508 CFTR mutation