Research
Determined to Defeat CF
Ineke Braakman, Utrecht, February 28, 2018
Our Quest for Helping Hands for CFTR We consume proteins because we need the building blocks of those proteins (amino acids) to continually create new proteins.

This occurs in all cells of our body, where proteins perform almost all the work. They can only perform this work when they have attained the correct shape, and this is impossible without assistance.
Cystic Fibrosis is caused by a defect in the large and complex protein CFTR. In our project, we are searching for the helper proteins that assist CFTR in attaining its active form.
Proteins are like strings of beads that fold into a specific 3D shape. Only when CFTR has the correct shape (as shown in the picture) can it perform its function, which is transporting salt out of the cell.
CFTR is a chain of 1480 beads. It's almost unimaginable that this protein machine consists of just a single chain, but it does. The beginning and end of the chain are indicated.
It helps that CFTR initially folds into 5 components. Then, it becomes a chain of 5 interconnected puzzle pieces (T1, N1, R, T2, N2, as shown in the drawing) that need to fit together. But even then, this process isn't achievable without assistance, and CFTR is just one of approximately 20,000 different proteins in our body that require folding.
