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FLASH GENE
Symbol SFTPC contributors: mct - updated : 28-09-2013
HGNC name surfactant, pulmonary-associated protein C
HGNC id 10802
PROTEIN
PHYSICAL PROPERTIES Hydrophobic
STRUCTURE
motifs/domains
  • N-terminus, situated in the cytosol
  • BRICHOS domain, of approximately 100 amino acids , suggested to act as a chaperone that targets the SFTPC region of proSFTPC and prevents its aggregation while assisting its safe membrane insertion as a TM helix
    • conjugated GlycoP
    isoforms Precursor
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY immunity/defense
    SUBCELLULAR LOCALIZATION extracellular
        plasma membrane
        intracellular
    intracellular,nucleus
    text integral membrane protein anchored in the membrane by the hydrophobic core of mature SFTPC
    basic FUNCTION
  • enhancer the surface tension-lowering properties of surfactant lipides
  • SFTPB, and SFTPC proteins promote the formation of proteolipid channels in which lipid molecules are functionally involved
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS inflammation
    PATHWAY
    metabolism
    signaling
    a component component of surfactant
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • DNAJB9, DNAJC10 promote turnover of misfolded SFTPC and this activity is dependent on their ability to stimulate HSPA5 ATPase activity
  • ER lumenal domain of MKS3 interacted with a complex that included mutant SFTPC and associated chaperones, whereas the region predicted to encode the transmembrane domains of MKS3 interacted with cytosolic VCP
  • role of JNK signaling in mediating SFTPC BRICHOS-induced cytokine release, and provide a link between SFTPC BRICHOS mutants and proinflammatory cytokine signaling
  • functional cooperation between surfactant proteins SFTPB and SFTPC may be required to sustain a proper compression-expansion dynamics in the presence of physiological proportions of cholesterol
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) FILD , SMDP2
    Susceptibility to perinatal respiratory disease and bronchopulmonary dysplasia in premature infant
    Variant & Polymorphism allele 138 ASN increasing the risk of bronchopulmonary dysplasia in premature infant
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS