Pulmonary surfactant-associated protein C

SFTPC

Available structures

Ortholog search: PDBe RCSB

List of PDB id codes
2YAD

Identifiers

Aliases

SFTPC, BRICD6, PSP-C, SFTP2, SMDP2, SP-C, surfactant protein C

External IDs

MGI: 109517 HomoloGene: 2271 GeneCards: SFTPC

Gene ontology
Molecular function	• protein binding
Cellular component	• extracellular fluid • extracellular region • clathrin-coated endocytic vesicle • lamellar body • endoplasmic reticulum membrane • multivesicular body lumen
Biological process	• cellular protein metabolic process • respiratory gaseous exchange
Sources:Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

Entrez


6440


20389

Ensembl


ENSG00000168484


ENSMUSG00000022097

UniProt


P11686


P21841

RefSeq (mRNA)

NM_001172357 NM_001172410 NM_003018 NM_001317778 NM_001317779
NM_001317780


NM_011359

RefSeq (protein)

NP_001165828.1 NP_001165881.1 NP_003009.2 NP_001304707.1 NP_001304708.1
NP_001304709.1


n/a

Location (UCSC)

Chr 8: 22.16 – 22.16 Mb

Chr 14: 70.52 – 70.52 Mb

PubMed search

^[1]

^[2]

Wikidata

View/Edit Human

View/Edit Mouse

Pulmonary surfactant-associated protein C is a protein that in humans is encoded by the SFTPC gene.^[3]^[4]^[5]

It is a membrane protein which manufactures surfactant.

Clinical significance

It is associated with Surfactant metabolism dysfunction type 2.

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

References

↑ "Human PubMed Reference:".
↑ "Mouse PubMed Reference:".
↑ 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 1151261. PMID 1859376.
↑ 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.
↑ "Entrez Gene: SFTPC surfactant, pulmonary-associated protein C".

Pérez-Gil J (2002). "Lipid-protein interactions of hydrophobic proteins SP-B and SP-C in lung surfactant assembly and dynamics". Pediatric pathology & molecular medicine. 20 (6): 445–69. doi:10.1080/15227950152625783. PMID 11699574.
Solarin KO, Wang WJ, Beers MF (2002). "Synthesis and post-translational processing of surfactant protein C". Pediatric pathology & molecular medicine. 20 (6): 471–500. doi:10.1080/15227950152625792. PMID 11699575.
Johansson J, Curstedt T, Robertson B (2002). "Artificial surfactants based on analogues of SP-B and SP-C". Pediatric pathology & molecular medicine. 20 (6): 501–18. doi:10.1080/15227950152625800. PMID 11699576.
Nogee LM (2004). "Alterations in SP-B and SP-C expression in neonatal lung disease". Annu. Rev. Physiol. 66: 601–23. doi:10.1146/annurev.physiol.66.032102.134711. PMID 14977415.
Brasch F, Griese M, Tredano M, et al. (2005). "Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene". Eur. Respir. J. 24 (1): 30–9. doi:10.1183/09031936.04.00000104. PMID 15293602.
Curstedt T, Johansson J, Persson P, et al. (1990). "Hydrophobic surfactant-associated polypeptides: SP-C is a lipopeptide with two palmitoylated cysteine residues, whereas SP-B lacks covalently linked fatty acyl groups". Proc. Natl. Acad. Sci. U.S.A. 87 (8): 2985–9. doi:10.1073/pnas.87.8.2985. PMC 53818. PMID 2326260.
Simatos GA, Forward KB, Morrow MR, Keough KM (1990). "Interaction between perdeuterated dimyristoylphosphatidylcholine and low molecular weight pulmonary surfactant protein SP-C". Biochemistry. 29 (24): 5807–14. doi:10.1021/bi00476a023. PMID 2383558.
Glasser SW, Korfhagen TR, Perme CM, et al. (1988). "Two SP-C genes encoding human pulmonary surfactant proteolipid". J. Biol. Chem. 263 (21): 10326–31. PMID 2839484.
Glasser SW, Korfhagen TR, Weaver TE, et al. (1988). "cDNA, deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid, SPL(pVal)". J. Biol. Chem. 263 (1): 9–12. PMID 3335510.
Johansson J, Jörnvall H, Eklund A, et al. (1988). "Hydrophobic 3.7 kDa surfactant polypeptide: structural characterization of the human and bovine forms". FEBS Lett. 232 (1): 61–4. doi:10.1016/0014-5793(88)80386-7. PMID 3366248.
Warr RG, Hawgood S, Buckley DI, et al. (1987). "Low molecular weight human pulmonary surfactant protein (SP5): isolation, characterization, and cDNA and amino acid sequences". Proc. Natl. Acad. Sci. U.S.A. 84 (22): 7915–9. doi:10.1073/pnas.84.22.7915. PMC 299446. PMID 3479771.
Young WA (1966). "Familial fibrocystic pulmonary dysplasia: a new case in a known affected family". Canadian Medical Association journal. 94 (20): 1059–61. PMC 1935417. PMID 5942662.
Wood S, Yaremko ML, Schertzer M, et al. (1995). "Mapping of the pulmonary surfactant SP5 (SFTP2) locus to 8p21 and characterization of a microsatellite repeat marker that shows frequent loss of heterozygosity in human carcinomas". Genomics. 24 (3): 597–600. doi:10.1006/geno.1994.1673. PMID 7713515.
Hatzis D, Deiter G, deMello DE, Floros J (1994). "Human surfactant protein-C: genetic homogeneity and expression in RDS; comparison with other species". Exp. Lung Res. 20 (1): 57–72. doi:10.3109/01902149409064373. PMID 8181452.
Nogee LM, Dunbar AE, Wert SE, et al. (2001). "A mutation in the surfactant protein C gene associated with familial interstitial lung disease". N. Engl. J. Med. 344 (8): 573–9. doi:10.1056/NEJM200102223440805. PMID 11207353.
Glasser SW, Burhans MS, Korfhagen TR, et al. (2001). "Altered stability of pulmonary surfactant in SP-C-deficient mice". Proc. Natl. Acad. Sci. U.S.A. 98 (11): 6366–71. doi:10.1073/pnas.101500298. PMC 33474. PMID 11344267.

PDB gallery

1spf: THE NMR STRUCTURE OF THE PULMONARY SURFACTANT-ASSOCIATED POLYPEPTIDE SP-C IN AN APOLAR SOLVENT CONTAINS A VALYL-RICH ALPHA-HELIX

External links

GeneReviews/NIH/NCBI/UW entry on Pulmonary Fibrosis, Familial
Pulmonary Surfactant-Associated Protein B at the US National Library of Medicine Medical Subject Headings (MeSH)

Protein: cell membrane proteins (other than Cell surface receptor, enzymes, and cytoskeleton)

Arrestin	SAG ARRB1 ARRB2 ARR3

Membrane-spanning 4A	MS4A1 MS4A2 MS4A3 MS4A4A MS4A4E MS4A5 MS4A6A MS4A6E MS4A7 MS4A8B MS4A9 MS4A10 MS4A12 MS4A13 MS4A14 MS4A15 MS4A18

Myelin	Myelin basic protein PMP2 Myelin proteolipid protein PLP1 Myelin oligodendrocyte glycoprotein Myelin-associated glycoprotein Myelin protein zero

Pulmonary surfactant	Pulmonary surfactant-associated protein B Pulmonary surfactant-associated protein C

Tetraspanin	TSPAN1 TSPAN2 TSPAN3 TSPAN4 TSPAN5 TSPAN6 TSPAN7 TSPAN8 TSPAN9 TSPAN10 TSPAN11 TSPAN12 TSPAN13 TSPAN14 TSPAN15 TSPAN16 TSPAN17 TSPAN18 TSPAN19 TSPAN20 TSPAN21 TSPAN22 TSPAN23 TSPAN24 TSPAN25 TSPAN26 TSPAN27 TSPAN28 TSPAN29 TSPAN30 TSPAN31 TSPAN32 TSPAN33 TSPAN34

Other/ungrouped	Calnexin LDL-receptor-related protein-associated protein Neurofibromin 2 Presenilin PSEN1 PSEN2 HFE Phospholipid transfer proteins Dysferlin STRC OTOF

see also other cell membrane protein disorders

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Pulmonary surfactant-associated protein C

Clinical significance

See also

References

Further reading

External links