Surfactant protein C

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Surfactant protein C
Protein SFTPC PDB 1spf.png
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesSFTPC, BRICD6, PSP-C, SFTP2, SMDP2, SP-C, surfactant protein C
External IDsMGI: 109517 HomoloGene: 2271 GeneCards: SFTPC
Gene location (Human)
Chromosome 8 (human)
Chr.Chromosome 8 (human)[1]
Chromosome 8 (human)
Genomic location for Surfactant protein C
Genomic location for Surfactant protein C
Band8p21.3Start22,156,913 bp[1]
End22,164,479 bp[1]
RNA expression pattern
PBB GE SFTPC 205982 x at fs.png

PBB GE SFTPC 38691 s at fs.png

PBB GE SFTPC 211735 x at fs.png
More reference expression data
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_011359

RefSeq (protein)

NP_035489

Location (UCSC)Chr 8: 22.16 – 22.16 MbChr 14: 70.52 – 70.52 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[edit]

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[edit]

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[edit]

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000168484 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000022097 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:".
  4. ^ "Mouse PubMed Reference:".
  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. ^ a b 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. doi:10.1073/pnas.1114740109. 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.
  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. doi:10.1038/ncomms15504. PMC 5457526. PMID 28534479.

Further reading[edit]

External links[edit]

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