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New insights on congenital pulmonary airways malformations revealed by proteomic analyses.

C Barazzone-ArgiroffoJ Lascano MaillardI VidalM L Bochaton-PiallatS BlaskovicY DonatiB E WildhaberA-L RougemontC DelacourtIsabelle Ruchonnet-Métrailler
Published in: Orphanet journal of rare diseases (2019)
This study provides new insights in CPAM etiology, showing clear distinction between CPAM types 1 and 2, by immunohistochemistry and proteomics. This suggests that CPAM 1 and CPAM 2 might occur at different stages of lung branching. Finally, the comparison between fetal lung structures and CPAMs shows clearly different protein profiles, thereby arguing against a developmental arrest in a localized part of the lung.
Keyphrases
  • pulmonary hypertension
  • cystic fibrosis
  • label free
  • small molecule