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FLASH GENE
Symbol SFTPB contributors: mct/npt - updated : 19-05-2008
HGNC name surfactant protein B
HGNC id 10801
Corresponding disease
SMDP1 surfactant metabolism dysfunction, pulmonary 1
Location 2p11.2      Physical location : 85.884.440 - 85.895.864
Synonym name
  • surfactant, pulmonary-associated protein B
  • 6 kDa protein
  • 18 kDa pulmonary-surfactant protein
  • Synonym symbol(s) SFTP3, SP-B, SPLP, SFTB3, PSP-B, SMDP1
    DNA
    TYPE functioning gene
    STRUCTURE 10.93 kb     11 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Map pter - D2S394 - D2S2371 - SFTPB - D2S2232 - D23S234E - cen
    Physical map
    FLJ37357 2p11.2 hypothetical protein FLJ37357 LOC200383 2p11.2 similar to Dynein heavy chain at 16F FLJ23529 2p11.2 hypothetical protein FLJ23529 LOC129293 2p11.2 hypothetical protein LOC129293 TMSB10 2p11.2 thymosin, beta 10 KCMF1 2p11.2 potassium channel modulatory factor 1 LOC391388 2 hypothetical gene supported by AJ238095; NM_014463 TCF7L1 2p11.2 transcription factor 7-like 1 (T-cell specific, HMG-box) LOC388966 2 similar to hypothetical protein FLJ20296 TGOLN2 2p11.2 trans-golgi network protein 2 LOC388967 2 LOC388967 LOC388968 2 similar to Phosphatidylethanolamine-binding protein (PEBP) (Prostatic binding protein) (HCNPpp) (Neuropolypeptide h3) (Raf kinase inhibitor protein) (RKIP) FLJ20296 2p11.2 hypothetical protein FLJ20296 FLJ21977 2p11.2 hypothetical protein FLJ21977 CAPG 2cen-q24 capping protein (actin filament), gelsolin-like LOC284948 2p11.2 similar to B-cell linker; B cell linker protein MAT2A 2p11.2 methionine adenosyltransferase II, alpha GGCX 2p12 gamma-glutamyl carboxylase VAMP8 2p11.2-p11.1 vesicle-associated membrane protein 8 (endobrevin) VAMP5 2p11.2 vesicle-associated membrane protein 5 (myobrevin) LOC51255 2p11.2 hypothetical protein LOC51255 FLJ90024 2p11.2 fasting-inducible integral membrane protein TM6P1 LOC388969 2 LOC388969 USP39 2p11.2 ubiquitin specific protease 39 SFTPB 2p12-p11.2 surfactant, pulmonary-associated protein B GNLY 2p12-q11 granulysin HATH6 SIAT9 2p11.2 sialyltransferase 9 (CMP-NeuAc:lactosylceramide alpha-2,3-sialyltransferase; GM3 synthase) POLR1A 2p11.2 polymerase (RNA) 1 polypeptide A, 194kDa FLJ20758 2p11.2 FLJ20758 protein IMMT 2p11.2 inner membrane protein, mitochondrial (mitofilin) MRPL35 2p11.2 mitochondrial ribosomal protein L35 FLJ13110 2p11.2 hypothetical protein FLJ13110 JMJD1 2p11.2 jumonji domain containing 1 NEDF 2p24.3-p24.1 neuroendocrine differentiation factor RNF103 2p11.2 ring finger protein 103 FLJ13910 2p11.2 hypothetical protein FLJ13910
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    11 - 2724 42 381 - Pilot-Matias (1989)
    11 - 2026 42 381 - Jacobs (1987)
    EXPRESSION
    Type
    constitutive of
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Respiratoryrespiratory tractbronchus   
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Epithelialbarrier/lining   
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Respiratoryalveolar cell type 1
    Respiratoryalveolar cell type II
    Respiratoryepithelial cell
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES Hydrophobic
    STRUCTURE
    motifs/domains
  • N and C terminal peptides flanking the mature factor
  • an alternatively spliced in normal lung, saposin-like
  • N-terminal segment comprising residues 1-9, specifically required for surface activity, and prolines 2, 4, and 6 as well as tryptophan 9, may constitute essential structural motifs for protein function (Serrano 2006)
  • conjugated GlycoP
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY immunity/defense
    SUBCELLULAR LOCALIZATION extracellular
    basic FUNCTION
  • structural integrity of pneumocytes and normal processing of surfactant complex
  • play an important role in surfactant metabolism and host defense mechanisms in the lung (Kim 2007)
  • absolutely required for the surface activity of pulmonary surfactant and postnatal lung function (Serrano 2006)
  • 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
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • 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
  • surfactant-associated glycoprotein
  • REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) SMDP1
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in papillary thyroid carcinoma
    Susceptibility
  • to respiratory distress in premature infant associated with SFTPA polymorphism(s) and restricted to patients with the Y131Y SFTPB genotype
  • to idiopathic pulmonary fibrosis in smokers
  • Variant & Polymorphism SNP
  • T131I, B1580 C
  • VNTR in intron 4 is associated with an increased 60 day mortality in acute respiratory distress syndrome (Currier 2008)
  • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS