Property Summary

NCBI Gene PubMed Count 99
Grant Count 213
R01 Count 137
Funding $49,748,109.47
PubMed Score 487.81
PubTator Score 442.30

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (13)

Disease log2 FC p
osteosarcoma 1.458 0.000
glioblastoma -1.100 0.000
primary pancreatic ductal adenocarcinoma -1.252 0.007
non-small cell lung cancer -6.581 0.000
lung cancer -9.100 0.000
lung adenocarcinoma -6.100 0.000
adult high grade glioma -1.300 0.001
non primary Sjogren syndrome sicca 1.100 0.014
lung carcinoma -4.900 0.000
ovarian cancer -1.400 0.000
pituitary cancer -1.400 0.000
pancreatic cancer -1.100 0.009
psoriasis -1.700 0.000

Synonym

Accession P11686 A6XNE4 B2RE00 E9PGX3 P11687 Q12793 Q7Z5D0 SP-C
Symbols SP-C
PSP-C
SFTP2
SMDP2
BRICD6

Gene

PDB

2YAD  

Gene RIF (83)

PMID Text
26375473 In interstitial lung disease, abnormal proSP-C was seen in small and dense lamellar bodies in type II alveolar epithelial cells. A549 cells expressing proSP-C(L55F) had abnormal organelles. It partly colocalized in CD63-positive cytoplasmic vesicles .
26041777 support a chaperone function of the BRICHOS domain, possibly together with the linker region, during pro-SP-C biosynthesis in the endoplasmic reticulum
25891900 cleavage of BRICHOS in a loop region that is cleaved during proSP-C biosynthesis results in increased capacity to delay Abeta(42) fibril formation.
25782673 We sequenced SFTPC and analyzed morphology, ultrastructure and SP expression in lung tissue when available. We identified eight subjects who were heterozygous for SP-C mutations.
25657025 analysis of clinical patterns in patients with SFTPC mutations
24099305 Structural modeling of transmembrane (BRICHOS) domains of SFTPC precursor and BRI2/ITM2B (integral membrane protein 2B) identifies conserved region structurally complementary to beta-sheet-/amyloid-prone regions in BRICHOS domain-containing proteins.
23406594 elevated expression in nasal mucosa in chronic rhinosinusitis with polyps
22458263 The A116D mutation leads to impaired processing of proSP-C in alveolar epithelial cells, alters cell viability and lipid composition, and also activates cells of the immune system.
21828032 Mutation increases endoplasmic reticulum stress and induces apoptotic cell death compared with wild-type SP-C in alveolar type II cells, supporting the significance of this mutation in the pathogenesis of pulmonary fibrosis.
21707890 Data show that, in contrast to its wild-type counterpart, SP-C(I73T) is misdirected to the plasma membrane and subsequently internalized to the endocytic pathway via early endosomes, leading to the accumulation of abnormally processed proSP-C isoforms.
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AA Sequence

MDVGSKEVLMESPPDYSAAPRGRFGIPCCPVHLKRLLIVVVVVVLIVVVIVGALLMGLHMSQKHTEMVLE      1 - 70
MSIGAPEAQQRLALSEHLVTTATFSIGSTGLVVYDYQQLLIAYKPAPGTCCYIMKIAPESIPSLEALNRK     71 - 140
VHNFQMECSLQAKPAVPTSKLGQAEGRDAGSAPSGGDPAFLGMAVNTLCGEVPLYYI                 141 - 197
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Text Mined References (104)

PMID Year Title
26871637 2016 Widespread Expansion of Protein Interaction Capabilities by Alternative Splicing.
26375473 2016 A novel surfactant protein C L55F mutation associated with interstitial lung disease alters subcellular localization of proSP-C in A549 cells.
26041777 2015 Folding and Intramembraneous BRICHOS Binding of the Prosurfactant Protein C Transmembrane Segment.
25891900 2015 Dissociation of a BRICHOS trimer into monomers leads to increased inhibitory effect on A?42 fibril formation.
25782673 2015 Clinical and ultrastructural spectrum of diffuse lung disease associated with surfactant protein C mutations.
25657025 2015 Genotype alone does not predict the clinical course of SFTPC deficiency in paediatric patients.
25416956 2014 A proteome-scale map of the human interactome network.
24099305 2013 The BRICHOS domain, amyloid fibril formation, and their relationship.
23505323 2013 Genomic study in Mexicans identifies a new locus for triglycerides and refines European lipid loci.
23406594 2013 Detection of surfactant proteins A, B, C, and D in human nasal mucosa and their regulation in chronic rhinosinusitis with polyps.
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