Cellular imbalance in proximal and distal lung of CFTR^-/- sheep in utero and at birth.

ABSTRACT:

Background

The Lung is the major focus of therapeutic approaches for the inherited disorder cystic fibrosis (CF) as without treatment lung disease is life-limiting. However, the initiating events that predispose the CF lung to cycles of infection, inflammation and resultant tissue damage are still unclear. Inflammation may occur in the CF lung prior to birth in human and several large animal models suggesting an in utero origin for the disease and encouraging further studies prior to birth.

Methods

Here we used the sheep model of CF (CFTR^-/-) and age-matched wild-type (WT) sheep of the same breed to investigate the single cell transcriptomes of proximal and distal lung tissue at 80 days and 120 days of gestation and at term (147 days). Single cell RNA-seq was perf

SUBMITTER: Leir SH 

PROVIDER: S-EPMC12153128 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature