Pulmonary surfactant-associated protein C

Surfactant protein C
PDB rendering based on 1spf.
Available structures PDB 1spf
Identifiers
Symbols	SFTPC; PSP-C; SFTP2; SMDP2; SP-C
External IDs	OMIM: 178620 MGI: 109517 HomoloGene: 2271 GeneCards: SFTPC Gene
Gene Ontology Molecular function • protein homodimerization activity Cellular component • extracellular region • extracellular space • cytoplasm • multivesicular body • lamellar body Biological process • respiratory gaseous exchange • circadian rhythm • response to hormone stimulus • response to glucose stimulus • response to lipopolysaccharide • response to retinoic acid • protein homooligomerization • response to glucocorticoid stimulus • response to cAMP • response to hyperoxia • response to interleukin-6 • response to growth factor stimulus • cellular response to mechanical stimulus • cellular response to nitric oxide Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	6440	20389
Ensembl	ENSG00000168484	ENSMUSG00000022097
UniProt	P11686	Q6P8P8
RefSeq (mRNA)	NM_001172357.1	NM_011359.2
RefSeq (protein)	NP_001165828.1	NP_035489.2
Location (UCSC)	Chr 8: 22.01 – 22.02 Mb	Chr 14: 70.92 – 70.92 Mb
PubMed search	[1]	[2]

Pulmonary surfactant-associated protein C is a protein that in humans is encoded by the SFTPC gene.

Pulmonary surfactant-associated protein C 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.

See also

• Pulmonary_surfactant#Proteins

Further reading

• 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. 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. 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. 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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=53818. 
• 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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=299446. 
• 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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1935417. 
• 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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=33474. 

External links

• GeneReviews/NIH/NCBI/UW entry on Pulmonary Fibrosis, Familial
• MeSH Pulmonary+Surfactant-Associated+Protein+B