SFTPAs translocate from ER membrane to extracellular region

Stable Identifier
Reaction [uncertain]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Orthologous Events
Cite Us!