AI Article Synopsis
- The synthesis of pulmonary surfactant is crucial for proper lung function at birth, as it reduces surface tension and prevents lung collapse.
- The study found that the gene EMC3 is essential for the production of surfactant in mouse embryonic lung cells; when deleted, it disrupts lipid and protein synthesis.
- EMC3 plays a key role in processing important surfactant proteins and coordinating their assembly with lipids in lung cells, which is vital for respiratory adaptation at birth.
Article Abstract
Adaptation to respiration at birth depends upon the synthesis of pulmonary surfactant, a lipid-protein complex that reduces surface tension at the air-liquid interface in the alveoli and prevents lung collapse during the ventilatory cycle. Herein, we demonstrated that the gene encoding a subunit of the endoplasmic reticulum membrane complex, EMC3, also known as TMEM111 (Emc3/Tmem111), was required for murine pulmonary surfactant synthesis and lung function at birth. Conditional deletion of Emc3 in murine embryonic lung epithelial cells disrupted the synthesis and packaging of surfactant lipids and proteins, impaired the formation of lamellar bodies, and induced the unfolded protein response in alveolar type 2 (AT2) cells. EMC3 was essential for the processing and routing of surfactant proteins, SP-B and SP-C, and the biogenesis of the phospholipid transport protein ABCA3. Transcriptomic, lipidomic, and proteomic analyses demonstrated that EMC3 coordinates the assembly of lipids and proteins in AT2 cells that is necessary for surfactant synthesis and function at birth.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5707157 | PMC |
| http://dx.doi.org/10.1172/JCI94152 | DOI Listing |
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