SFTPAs translocate from ER membrane to extracellular region

Stable Identifier
R-HSA-5685649
Type
Reaction [uncertain]
Species
Homo sapiens
Compartment
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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

Wang, Y, Kuan, PJ, Xing, C, Cronkhite, JT, Torres, F, Rosenblatt, RL, DiMaio, JM, Kinch, LN, Grishin, NV, Garcia, CK

Am. J. Hum. Genet. 2009
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