SFTPC
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesSFTPC, BRICD6, PSP-C, SFTP2, SMDP2, SP-C, surfactant protein C, SP5
External IDsOMIM: 178620 MGI: 109517 HomoloGene: 2271 GeneCards: SFTPC
Orthologs
SpeciesHumanMouse
Entrez

6440

20389

Ensembl

ENSG00000168484

ENSMUSG00000022097

UniProt

P11686

P21841
Q6P8P8

RefSeq (mRNA)

NM_011359

RefSeq (protein)

NP_035489

Location (UCSC)Chr 8: 22.16 – 22.16 MbChr 14: 70.76 – 70.76 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Surfactant protein C (SP-C), is one of the pulmonary surfactant proteins. In humans this is encoded by the SFTPC gene.[5][6][7]

It is a membrane protein.

Structure

SFTPC is a 197-residue protein made up of two halves: a unique N-terminal propeptide domain and a C-terminal BRICHOS domain. The around 100-aa long propeptide domain actually contains not only the cleaved part, but also the mature peptide. It can be further broken down into a 23-aa helical transmembrane propeptide proper, the mature secreted SP-C (24-58), and a linker (59-89) that connects to the BRICHOS domain.[8]

The propeptide of pulmonary surfactant C has an N-terminal alpha-helical segment whose suggested function was stabilization of the protein structure, since the mature peptide 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.[9]

The structure of the BRICHOS domain has been solved. Mutations in this domain also lead to amyloid fibrils made up of the mature peptide, suggesting a chaperone activity.[8]

Clinical significance

Mutations are associated with surfactant metabolism dysfunction type 2.

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

Recombinant SP-C is used in Venticute, an artificial lung surfactant.

A process to mass-produce an analogue called rSP-C33Le by fusion with spidroin has been described.[10]

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000168484 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000022097 - Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. Keller A, Eistetter HR, Voss T, Schäfer KP (July 1991). "The pulmonary surfactant protein C (SP-C) precursor is a type II transmembrane protein". The Biochemical Journal. 277 ( Pt 2) (Pt 2): 493–9. doi:10.1042/bj2770493. PMC 1151261. PMID 1859376.
  6. Johansson H, Nordling K, Weaver TE, Johansson J (July 2006). "The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment". The Journal of Biological Chemistry. 281 (30): 21032–9. doi:10.1074/jbc.M603001200. PMID 16709565.
  7. "Entrez Gene: SFTPC surfactant, pulmonary-associated protein C".
  8. 1 2 Willander H, Askarieh G, Landreh M, Westermark P, Nordling K, Keränen H, et al. (February 2012). "High-resolution structure of a BRICHOS domain and its implications for anti-amyloid chaperone activity on lung surfactant protein C". Proceedings of the National Academy of Sciences of the United States of America. 109 (7): 2325–9. Bibcode:2012PNAS..109.2325W. doi:10.1073/pnas.1114740109. PMC 3289314. PMID 22308375.
  9. 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". The FEBS Journal. 273 (5): 926–35. doi:10.1111/j.1742-4658.2006.05124.x. PMID 16478467. S2CID 1231483.
  10. Kronqvist N, Sarr M, Lindqvist A, Nordling K, Otikovs M, Venturi L, et al. (May 2017). "Efficient protein production inspired by how spiders make silk". Nature Communications. 8 (1): 15504. Bibcode:2017NatCo...815504K. doi:10.1038/ncomms15504. PMC 5457526. PMID 28534479.

Further reading

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