Bronchiectasis Research Paper

Many people are familiar with the term protein, in fact if you were to converse with a random individual about the function of proteins it is highly likely they would state its importance in building muscle. However, the class of biomolecules known as proteins have functions more diverse than simply increasing the dimensions of skeletal muscles. Of interest are transport proteins, particularly the CFTR which assists in transporting ions across cellular membranes. Failure of the CFTR to function properly affects the ability of ions to be transmitted across epithelial membranes, resulting in abnormal transmembrane potentials as well as respiratory problems which typically shorten the lifespans of those affected. Impairment of CFTR transport

When enlargement of structures such as the bronchi occurs there is an inefficient clearing of mucus and other secretions from the lungs. With the increased presence of mucus in the respiratory apparatus forced expiratory volume of individuals with CF tend to be lower than normal values. Since the mutated CFTR proteins cannot effectively transport ions across cell membranes the consistency of the mucus is thick and is dehydrated. This mucus encourages the proliferation of bacteria and multiple infections of the lung are common. Fibrosis or the formation of scar tissue is another symptom of CF, this tissue replaces normal tissue and is not useful in the respiratory process as such there is difficulty in meeting the body’s oxygen requirements. Ho et al. (1997) state that mutations in CFTR proteins are numerous and certain variations are known to cause organs such as the pancreas to malfunction. Simmons et al. (2011) Considers CF sufferers who are older than 40 to be long term survivors which highlights how abbreviated their life expectancy is. Bronchiectasis and Fibrosis affect the lungs of affected individuals to such an extent that death is normally as a result of failure of the respiratory