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pulmonary surfactant-associated protein C; SP-C; pulmonary surfactant-associated proteolipid SPL(Val); SP5 (SFTPC, SFTP2)
Function:
- pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces
Structure: contains 1 BRICHOS domain
Compartment: -secreted, extracellular space, surface film
Alternative splicing: named isoforms=2
Pathology:
- defects in SFTPC are the cause of pulmonary surfactant metabolism dysfunction type 2 (pulmonary alveolar proteinosis due to SFTPC deficiency)
- genetic variations in SFTPC are a cause of susceptibility to neonatal respiratory distress syndrome
General
glycoprotein
phosphoprotein
proteolipid
secreted protein
Properties
SIZE: entity length = 197 aa
MW = 21 kD
COMPARTMENT: extracellular compartment
MOTIF: Tyr phosphorylation site {Y16}
cysteine residue {C28}
MODIFICATION: palmitate
COMPARTMENT: membrane
cysteine residue {C29}
MODIFICATION: palmitate
COMPARTMENT: membrane
BRICHOS {94-197}
Database Correlations
OMIM correlations
UniProt P11686
PFAM correlations
Entrez Gene 6440
Kegg hsa:6440
References
- UniProt :accession P11686
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/SFTPC
- SeattleSNPs
http://pga.gs.washington.edu/data/sftpc/