Surfactant protein C

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Surfactant protein C, N terminal propeptide
1spf opm.png
Pulmonary surfactant-associated polypeptide SP-C Protein
Identifiers
Symbol SP_C-Propep
Pfam PF08999
InterPro IPR001729
PROSITE PDOC00298
OPM superfamily 75
OPM protein 1spf
surfactant, pulmonary-associated protein C
Identifiers
Symbol SFTPC
Alt. symbols SFTP2
Entrez 6440
HUGO 10802
OMIM 178620
RefSeq NM_003018
UniProt P11686
Other data
Locus Chr. 8 p21

Surfactant protein C (SP-C), is one of the pulmonary surfactant proteins. In humans this is encoded by the SFTPC gene.[1][2][3]

It is a membrane protein which manufactures surfactant.

The propeptide of pulmonary surfactant C has an N-terminal alpha-helical segment whose suggested function was stabilization of the protein structure, since the latter can irreversibly transform from its native alpha-helical structure to beta-sheet aggregates and form amyloid fibrils. The correct intracellular trafficking of proSP-C has also been reported to depend on the propeptide.[4]

Clinical significance[edit]

It is associated with surfactant metabolism dysfunction type 2.

Humans and animals born lacking SP-C tend to develop progressive interstitial lung disease.


References[edit]

  1. ^ Keller A, Eistetter HR, Voss T, Schafer KP (Aug 1991). "The pulmonary surfactant protein C (SP-C) precursor is a type II transmembrane protein". Biochem J. 277 (Pt 2): 493–9. PMC 1151261Freely accessible. PMID 1859376. 
  2. ^ Johansson H, Nordling K, Weaver TE, Johansson J (Jul 2006). "The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment". J Biol Chem. 281 (30): 21032–9. doi:10.1074/jbc.M603001200. PMID 16709565. 
  3. ^ "Entrez Gene: SFTPC surfactant, pulmonary-associated protein C". 
  4. ^ Li J, Liepinsh E, Almlén A, Thyberg J, Curstedt T, Jörnvall H, Johansson J (March 2006). "Structure and influence on stability and activity of the N-terminal propeptide part of lung surfactant protein C". FEBS J. 273 (5): 926–35. doi:10.1111/j.1742-4658.2006.05124.x. PMID 16478467. 

External links[edit]

Further reading[edit]


This article incorporates text from the public domain Pfam and InterPro IPR015091