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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
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CyFi-MAP: an interactive pathway-based resource for cystic fibrosis.
Catarina Pereira1,2, Alexander Mazein3,4, Carlos M Farinha1
1Faculty of Sciences, BioISI-Biosystems Integrative Sciences Institute, University of Lisboa, Campo Grande, 1749-016, Lisbon, Portugal.
Scientific Reports
|November 16, 2021
Summary
Cystic fibrosis (CF) is a genetic disease caused by CFTR gene mutations. We created CyFi-MAP, a systems biology tool to visualize CFTR pathways and aid in discovering new therapeutic targets for CF.
Area of Science:
- Systems biology
- Genetics
- Molecular biology
Background:
- Cystic fibrosis (CF) is a severe autosomal recessive disease.
- Over 2100 mutations in the CF transmembrane conductance regulator (CFTR) gene cause CF.
- Disease severity varies even with identical CFTR genotypes.
Purpose of the Study:
- To develop a systems biology approach for CF data analysis.
- To create a comprehensive disease map of CFTR molecular mechanisms and pathways.
- To identify novel therapeutic targets for cystic fibrosis.
Main Methods:
- Developed CyFi-MAP, a disease map repository for CFTR.
- Represented wild-type (wt-CFTR) and F508del-CFTR pathways in separate submaps.
- Included pathways for protein biosynthesis, ER processing, channel function, and degradation.
Main Results:
- CyFi-MAP visualizes wt-CFTR and F508del-CFTR pathways.
- The map details protein processing, trafficking, and channel activity.
- It serves as a curated, continuously updated resource.
Conclusions:
- CyFi-MAP enhances understanding of CFTR molecular mechanisms.
- This open-access resource supports global CF research.
- It facilitates the identification of new therapeutic strategies for cystic fibrosis.
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