SFTPC Antibody – Rabbit Polyclonal

SFTPC: Pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air- liquid interface in the peripheral air spaces. Defects in SFTPC are the cause of pulmonary surfactant metabolism dysfunction type 2 (SMDP2); also called pulmonary alveolar proteinosis due to surfactant protein C deficiency. 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. Genetic variations in SFTPC are a cause of susceptibility to respiratory distress syndrome in premature infants (RDS); also known as RDS in prematurity. RDS is a lung disease affecting usually premature newborn infants. It is characterized by deficient gas exchange, diffuse atelectasis, high-permeability lung edema and fibrin-rich alveolar deposits called 'hyaline membranes'. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Lipid-binding

Chromosomal Location of Human Ortholog: 8p21

Cellular Component: multivesicular body; extracellular space

Molecular Function: protein binding; protein homodimerization activity

Biological Process: circadian rhythm; response to cAMP; response to hyperoxia; response to retinoic acid; response to glucocorticoid stimulus; response to glucose stimulus; response to lipopolysaccharide; respiratory gaseous exchange; protein homooligomerization; response to vitamin A

Disease: Pulmonary Fibrosis, Idiopathic; Surfactant Metabolism Dysfunction, Pulmonary, 2