Alveolar surfactant

Alveolar surfactant mainly refers to a protein, whose main component is disalucarbyl phosphatidylcholine, which has a good effect on reducing the tension on the alveolar surface and increasing the compliance of the lungs. It is also very effective in preventing pulmonary edema and atelectasis. The active substances on the alveoli have a certain relationship with some genetic diseases, such as chromosomal recessive genetic diseases, etc.

Alveolar surfactant

Alveolar surfactant is a lipoprotein secreted by alveolar type II cells. Its main component is dipalmitoyl phosphatidylcholine (also called disalbuminyl phosphatidylcholine), which is distributed on the surface of the alveolar fluid molecular layer, that is, between the liquid-gas interface.

Physiological significance

(1) Reduce alveolar surface tension; (2) Increase lung compliance; (3) Maintain relative stability of alveolar volume; (4) Prevent atelectasis; (5) Prevent pulmonary edema;

Appear

The surface tension of the alveoli increases, large alveoli rupture, small alveoli atrophy, and neonatal respiratory distress syndrome and other diseases.

Synthetic secretion

Alveolar surfactant is secreted by type II alveolar epithelial cells. Its main component is dipalmitoyl phosphatidylcholine, which is arranged vertically at the alveolar fluid-air interface in the form of a single layer of molecules, and has the effect of reducing alveolar surface tension. Its physiological significance is: 1. Maintaining the stability of large and small alveolar volumes. ② Prevent alveolar collapse and facilitate lung expansion. ③ Prevent the formation of tissue fluid in the alveoli to facilitate lung ventilation.

Inherited ABCA3 Disorders

Hereditary ABCA3 disease is an autosomal recessive genetic disease. More than 150 different mutations in the ABCA3 gene have been found, including missense mutations, nonsense mutations, frameshift mutations, splice site mutations, as well as insertions and deletions. The infant mortality rate of ABCA3 gene mutations is 100%. Because the clinical manifestations, mortality, and histopathology of ABCA3 mutations are similar to those of patients with inherited SP-B deficiency, lung transplantation has been proposed as a treatment for severe, early-onset inherited ABCA3 disease. The study found that mutations that cause fatal ABCA3 disease are associated with ABCA3 transport defects, while mutations that cause non-severe lung disease (such as the E292V mutation) are associated with phospholipid transport defects.