Reactome | SFTPAs translocate from ER membrane to extracellular region

SFTPAs translocate from ER membrane to extracellular region

Stable Identifier

R-HSA-5685649

Type

Reaction [uncertain]

Species

Homo sapiens

Compartment

endoplasmic reticulum membrane, extracellular region

ReviewStatus

5/5

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SFTPAs translocate from ER membrane to extracellular region

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The pulmonary collectins, surfactant proteins A1, A2, A3 and D (SFTPAs, D), play important roles in innate host defense by binding and clearing invading microbes from the lung. They also influence surfactant homeostasis, contributing to the physical structures of lipids in the alveoli and to the regulation of surfactant function and metabolism. They are directly secreted from alveolar type II cells into the airway to function as part of the surfactant. The mechanism of secretion is unknown. Mutations in SFTPA2 disrupt protein structure and the defective protein is retained in the ER membrane (thus not secreted). Lack of SFTPA2 in surfactant contributes towards idiopathic pulmonary fibrosis (IPF; MIM:178500) (Wang et al. 2009). The mechanism of pathophysiology is unknown.

Literature References

PubMed ID	Title	Journal	Year
19100526	Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer Garcia, CK, Torres, F, Kuan, PJ, Rosenblatt, RL, Kinch, LN, Wang, Y, Cronkhite, JT, Grishin, NV, Xing, C, DiMaio, JM	Am. J. Hum. Genet.	2009