Grant: Operating Grant - PA: Regional Partnership Program - Newfoundland and Labrador

Lung surfactant is a complex mixture of biological molecules that lines the air-sacs in the lungs. In order for us to breathe, it is essential the lung surfactant is present and functioning properly. Premature infants often have difficulty breathing because they are born before their bodies have produced enough lung surfactant, a condition called Respiratory Distress Syndrome (RDS). In order to survive, these infants need to be treated with lung surfactant from another source (e.g. from animals), until their bodies start making enough of their own lung surfactant. People of any age with severe illness or injury often develop a condition called Acute Respiratory Distress Syndrome (ARDS) which is frequently fatal. Unfortunately, surfactant replacement treatment has not been shown to save the lives of those with ARDS. To produce an effective treatment for ARDS and other diseases that result from lung surfactant deficiencies and improve the treatment for infants, we need to better understand how lung surfactant works. One of the most important components of lung surfactant, a protein called Surfactant Protein-B (SP-B), is also one of its least well understood components. For a long time, we and others have sought to understand SP-B better by trying to determine its structure, i.e. the special shape it folds into that allows it to function properly. However, this has been very difficult since no one has been able to produce a sample of SP-B that would allow the structure of this protein, as it is found in the lung, to be observed in the laboratory. In collaboration with colleagues in Spain, we have recently succeeded in establishing methods to produce SP-B for structural studies. Now we are using this method to find the shape of SP-B. This will tell us which of its structural features are essential in order for it to carry out its function and enable the design of improved treatments for respiratory distress disorders.