SFTPC
Function
Pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces.
Involvement in disease
Pulmonary surfactant metabolism dysfunction 2
SMDP2
A rare disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course, due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.
None
The disease is caused by variants affecting the gene represented in this entry.
Cellular localization
- Secreted
- Extracellular space
- Surface film
Alternative names
SFTP2, SFTPC, Surfactant protein C, SP-C, Pulmonary surfactant-associated protein C, Pulmonary surfactant-associated proteolipid SPL(Val), SP5