Background of Poractant alfa
Pulmonary surfactants are complex mixtures of proteins and lipids that help in reducing surface tension in the lungs, thereby preventing alveolar collapse. One such surfactant of importance in medical science is poractant alfa.
Poractant alfa, commercially known as Curosurf, is a pulmonary surfactant. It was discovered in the 1980s by a group of clinicians and researchers who were investigating different types of surfactants for the treatment of neonatal Respiratory Distress Syndrome (RDS). It is derived from the lung lavage of a pig, encompassing distinct proportions of phospholipids, neutral lipids, fatty acids, and surfactant-associated proteins.
Regarding its gene locus and protein structure, pulmonary surfactants, including poractant alfa, are synthesized and secreted by alveolar type II cells in the lung. The surfactant-associated proteins essential for surfactant function and metabolism are encoded by the SFTPA1, SFTPB, SFTPC, and SFTPD genes.
Functions of Poractant alfa
Understanding the function of poractant alfa involves understanding the role of pulmonary surfactants. A central function of poractant alfa, or any pulmonary surfactant, is to importantly decrease surface tension at the air-liquid interface of the alveoli, mostly during exhalation, thereby stabilizing the alveoli and preventing their collapse. Other notable functions are in the alveolar size regulation, providing immunity to the lung, and acting as a carrier for other molecules.
Poractant alfa related diseases
The pathway related to the surfactant protein has a crucial role in lung function and health. Surfactant protein B (SP-B) and Surfactant protein C (SP-C) are vital for lowering surface tension in the lungs. Genetic mutations in the genes encoding these proteins, SFTPB for SP-B and SFTPC for SP-C, lead to disorders of surfactant metabolism. Variants in these genes may lead to conditions like pulmonary fibrosis and respiratory distress syndrome in newborns.
Pulmonary Surfactant-related diseases are often a result of surfactant deficiency or dysfunction. Neonatal Respiratory Distress Syndrome (RDS), also known as hyaline membrane disease, is a crucial example where there is a deficiency of surfactant in the lungs of preterm infants. Additionally, surfactant dysfunction can result from mutations in genes encoding surfactant proteins and lead to Interstitial Lung Diseases (ILDs).
The application of poractant alfa in medicine is mainly in the treatment of RDS in prematurely born infants. By replacing the deficient surfactant, poractant alfa helps restore the surface tension reducing properties in their lungs. Its use has shown to significantly improve lung function and decrease morbidity and mortality rates among preterm infants.
List of drug candidates related to Poractant alfa
Potential candidates for drug development related to poractant alfa include drugs that can enhance surfactant production or function, drugs that can mimic the mode of action of surfactant proteins or lipids, gene therapy for families known to have genetic forms of surfactant dysfunction, and novel methods of delivering surfactants.
In conclusion, poractant alfa is a life-saving drug used to manage respiratory distress syndrome in preterm infants. As a natural pulmonary surfactant, understanding its genetic locus, protein structure, and associated pathways gives us a comprehensive outlook of its unique functions and potential in managing pulmonary surfactant-related diseases. While it is already an instrumental inclusion in neonatology, further research holds promising potential in advancing its applications in research and medical science.
