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FLASH GENE
Symbol SFTPA1 contributors: mct - updated : 07-05-2019
HGNC name surfactant, pulmonary-associated protein A1
HGNC id 10798
PROTEIN
PHYSICAL PROPERTIES Hydrophilic
STRUCTURE
motifs/domains
  • N terminal segment required for the association of trimeric subunits into higher oligomers, phospholipid aggregation and specific inhibition of surfactant secretion, collagen like lectin
  • secondary structure SPA2 and SPA1
    conjugated GlycoP
    mono polymer homomer , trimer
    HOMOLOGY
    interspecies homolog to murine Sftpa
    Homologene
    FAMILY collectin family
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    basic FUNCTION
  • playing an important role in innate immunity
  • involved in host defense and inflammatory processes of the lung
  • also involved in the surface tension lowering ability of pulmonary surfactant
  • plays an important role in the lung during bacterial infection regulating protease and antiprotease activity
  • stimulates the release of neutrophil chemotactic factor by alveolar type II cells, and this effect is mediated by the receptor for SFTPA1 specifically expressed by these cells
  • most abundant pulmonary soluble collectin, that modulates innate and adaptive immunity of the lung, partially via its direct effects on alveolar macrophages (AM)
  • novel role for SFTPA1 in modulating endolysosomal trafficking via RAB7A in primary alveolar macrophages (AM)
  • SFTPA1, SFTPA2 plays a key role in innate lung host defense, in surfactant-related functions, and in parturition
  • plays critical roles in the innate immune system and surfactant homeostasis of the lung
  • SFTPA1 levels may be important to maintain surfactant function in the presence of high cholesterol within surfactant
  • fetal lungs produce signals to initiate labor when mature and NCOA1/NCOA2-dependent production of SFTPA1B and platelet-activating factor (PAF) is crucial for this process
  • associated with host defense in the lung, and contributes to the pathogenesis of chronic obstructive pulmonary disease (COPD)
  • innate host defense molecules, surfactant protein A1 (SFTPA1), and SFTPA2 differentially affect the function and proteome of the alveolar macrophages
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS inflammation
    PATHWAY
    metabolism
    signaling
    a component
  • component of a complex with SFTPA2 and SFTPD
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • SFTPB
  • in human fetal lung epithelial cells, SUV39H1 and SUV39H2 mRNA levels declined with cAMP induction of SFTPA1
  • SFTPA1 protects lung epithelium from tissue injury caused by DEF103B
  • LEP promotes fetal lung maturity and upregulates SFPTA1 expression in pulmonary alveoli type-II epithelial cells involving TTF1 activation
  • cell & other surfactant-associated glycoprotein
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    in idiopathic pulmonary fibrosis (IPF)
    Susceptibility
  • to idiopathic pulmonary fibrosis
  • to tuberculosis
  • idiopathic interstitial pneumonia (IIP)
  • Variant & Polymorphism SNP
  • 307A and 776T, increasing the risk of tuberculosis, in Ethiopia
  • heterozygous single nucleotide variant Val178Met in the SFTPA1 gene is associated with IIP
  • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Sftpa1 knock-in (Sftpa1-KI) mice that harbored the same mutation as patients spontaneously developed pulmonary fibrosis that was accelerated by influenza virus infection