Testing organ-specific responses to therapies in tissues differentiated from Cystic Fibrosis patient derived iPSCs.

Cystic Fibrosis (CF) is a life-shortening disease that is caused by mutations in the CFTR gene, a gene that is expressed in multiple organs. There are several primary tissue models of CF disease, including nasal epithelial cultures and rectal organoids, that are effective in reporting the potential efficacy of mutation-targeted therapies called CFTR modulators. However, there is the well-documented variation in tissue dependent, therapeutic response amongst CF patients, even those with the same CF-causing mutation.

Truth and Reconcilition Commissions and Health Care System Responses for Indigenous Peoples: A Scoping Review.

Grounded in human rights approaches, truth and reconciliation commissions (TRCs) explore an event or process that did widespread and systematic intentional harm to a group of people. Health as a fundamental right is an important component addressed by TRCs. Yet despite TRCs often having recommendations for health care systems, it is unknown how well these recommendations are being translated within health care settings.

Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells.

Background: Cystic Fibrosis causing mutations in the gene , reduce the activity of the CFTR channel protein, and leads to mucus aggregation, airway obstruction and poor lung function. A role for CFTR in the pathogenesis of other muco-obstructive airway diseases such as Chronic Obstructive Pulmonary Disease (COPD) has been well established. The CFTR modulatory compound, Ivacaftor (VX-770), potentiates channel activity of CFTR and certain CF-causing mutations and has been shown to ameliorate mucus obstruction and improve lung function in people harbouring these CF-causing mutations.