Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Basket 0
(max 400 entries)x

Your basket is currently empty.

Select item(s) and click on "Add to basket" to create your own collection here
(400 entries max)

P07988

- PSPB_HUMAN

UniProt

P07988 - PSPB_HUMAN

Protein

Pulmonary surfactant-associated protein B

Gene

SFTPB

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
    • BLAST
    • Align
    • Format
    • Add to basket
    • History
      Entry version 169 (01 Oct 2014)
      Sequence version 3 (01 May 1992)
      Previous versions | rss
    • Help video
    • Feedback
    • Comment

    Functioni

    Pulmonary surfactant-associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces. SP-B increases the collapse pressure of palmitic acid to nearly 70 millinewtons per meter.

    GO - Biological processi

    1. organ morphogenesis Source: ProtInc
    2. respiratory gaseous exchange Source: ProtInc
    3. sphingolipid metabolic process Source: InterPro

    Keywords - Biological processi

    Gaseous exchange

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Pulmonary surfactant-associated protein B
    Short name:
    SP-B
    Alternative name(s):
    18 kDa pulmonary-surfactant protein
    6 kDa protein
    Pulmonary surfactant-associated proteolipid SPL(Phe)
    Gene namesi
    Name:SFTPB
    Synonyms:SFTP3
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 2

    Organism-specific databases

    HGNCiHGNC:10801. SFTPB.

    Subcellular locationi

    GO - Cellular componenti

    1. extracellular space Source: UniProtKB-SubCell
    2. lysosome Source: InterPro

    Keywords - Cellular componenti

    Secreted, Surface film

    Pathology & Biotechi

    Involvement in diseasei

    Pulmonary surfactant metabolism dysfunction 1 (SMDP1) [MIM:265120]: A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.1 Publication
    Note: The disease is caused by mutations affecting the gene represented in this entry.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti236 – 2361R → C in SMDP1. 1 Publication
    VAR_036856
    Respiratory distress syndrome in premature infants (RDS) [MIM:267450]: A lung disease affecting usually premature newborn infants. It is characterized by deficient gas exchange, diffuse atelectasis, high-permeability lung edema and fibrin-rich alveolar deposits called 'hyaline membranes'.1 Publication
    Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. A variation Ile to Thr at position 131 influences the association between specific alleles of SFTPA1 and respiratory distress syndrome in premature infants.

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MIMi265120. phenotype.
    267450. phenotype.
    Orphaneti264675. Congenital pulmonary alveolar proteinosis.
    70587. Infant acute respiratory distress syndrome.
    217563. Neonatal acute respiratory distress with surfactant metabolism deficiency.
    PharmGKBiPA35713.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Signal peptidei1 – 2424Sequence AnalysisAdd
    BLAST
    Propeptidei25 – 200176PRO_0000031647Add
    BLAST
    Chaini201 – 27979Pulmonary surfactant-associated protein BPRO_0000031648Add
    BLAST
    Propeptidei280 – 381102PRO_0000031649Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Disulfide bondi69 ↔ 143PROSITE-ProRule annotation
    Disulfide bondi72 ↔ 137PROSITE-ProRule annotation
    Disulfide bondi100 ↔ 112PROSITE-ProRule annotation
    Glycosylationi129 – 1291N-linked (GlcNAc...)PROSITE-ProRule annotation
    Disulfide bondi208 ↔ 2771 PublicationPROSITE-ProRule annotation
    Disulfide bondi211 ↔ 2711 PublicationPROSITE-ProRule annotation
    Disulfide bondi235 ↔ 2461 PublicationPROSITE-ProRule annotation
    Disulfide bondi248 – 248Interchain1 PublicationPROSITE-ProRule annotation
    Disulfide bondi299 ↔ 366PROSITE-ProRule annotation
    Disulfide bondi302 ↔ 360PROSITE-ProRule annotation
    Glycosylationi311 – 3111N-linked (GlcNAc...)PROSITE-ProRule annotation
    Disulfide bondi325 ↔ 335PROSITE-ProRule annotation

    Keywords - PTMi

    Disulfide bond, Glycoprotein

    Proteomic databases

    PaxDbiP07988.
    PRIDEiP07988.

    Miscellaneous databases

    PMAP-CutDBP07988.

    Expressioni

    Gene expression databases

    ArrayExpressiP07988.
    BgeeiP07988.
    CleanExiHS_SFTPB.
    GenevestigatoriP07988.

    Organism-specific databases

    HPAiCAB002440.
    HPA034820.

    Interactioni

    Subunit structurei

    Homodimer; disulfide-linked.1 Publication

    Protein-protein interaction databases

    STRINGi9606.ENSP00000345161.

    Structurei

    Secondary structure

    1
    381
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Helixi213 – 2219
    Helixi260 – 2634
    Beta strandi265 – 2684
    Helixi269 – 2768

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    1DFWinfrared-A201-225[»]
    1KMRNMR-A211-225[»]
    1RG3NMR-A263-278[»]
    1RG4NMR-A263-278[»]
    1SSZinfrared-A208-278[»]
    2DWFNMR-A208-278[»]
    2JOUNMR-A208-278[»]
    2M0HNMR-A259-280[»]
    2M1TNMR-A259-278[»]
    ProteinModelPortaliP07988.
    SMRiP07988. Positions 69-145.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiP07988.

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini25 – 6541Saposin A-typePROSITE-ProRule annotationAdd
    BLAST
    Domaini65 – 14783Saposin B-type 1PROSITE-ProRule annotationAdd
    BLAST
    Domaini204 – 28178Saposin B-type 2PROSITE-ProRule annotationAdd
    BLAST
    Domaini295 – 37076Saposin B-type 3PROSITE-ProRule annotationAdd
    BLAST

    Sequence similaritiesi

    Contains 1 saposin A-type domain.PROSITE-ProRule annotation
    Contains 3 saposin B-type domains.PROSITE-ProRule annotation

    Keywords - Domaini

    Repeat, Signal

    Phylogenomic databases

    eggNOGiNOG286450.
    HOGENOMiHOG000115745.
    HOVERGENiHBG006905.
    InParanoidiP07988.
    OrthoDBiEOG7F24TB.
    PhylomeDBiP07988.

    Family and domain databases

    Gene3Di1.10.225.10. 3 hits.
    InterProiIPR003119. SapA.
    IPR007856. SapB_1.
    IPR008138. SapB_2.
    IPR008373. Saposin.
    IPR011001. Saposin-like.
    IPR008139. SaposinB.
    [Graphical view]
    PfamiPF02199. SapA. 1 hit.
    PF05184. SapB_1. 1 hit.
    PF03489. SapB_2. 2 hits.
    [Graphical view]
    PRINTSiPR01797. SAPOSIN.
    SMARTiSM00162. SAPA. 1 hit.
    SM00741. SapB. 3 hits.
    [Graphical view]
    SUPFAMiSSF47862. SSF47862. 3 hits.
    PROSITEiPS51110. SAP_A. 1 hit.
    PS50015. SAP_B. 3 hits.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    P07988-1 [UniParc]FASTAAdd to Basket

    « Hide

    MAESHLLQWL LLLLPTLCGP GTAAWTTSSL ACAQGPEFWC QSLEQALQCR    50
    ALGHCLQEVW GHVGADDLCQ ECEDIVHILN KMAKEAIFQD TMRKFLEQEC 100
    NVLPLKLLMP QCNQVLDDYF PLVIDYFQNQ TDSNGICMHL GLCKSRQPEP 150
    EQEPGMSDPL PKPLRDPLPD PLLDKLVLPV LPGALQARPG PHTQDLSEQQ 200
    FPIPLPYCWL CRALIKRIQA MIPKGALAVA VAQVCRVVPL VAGGICQCLA 250
    ERYSVILLDT LLGRMLPQLV CRLVLRCSMD DSAGPRSPTG EWLPRDSECH 300
    LCMSVTTQAG NSSEQAIPQA MLQACVGSWL DREKCKQFVE QHTPQLLTLV 350
    PRGWDAHTTC QALGVCGTMS SPLQCIHSPD L 381
    Length:381
    Mass (Da):42,117
    Last modified:May 1, 1992 - v3
    Checksum:i9FD7F66678A35153
    GO

    Sequence cautioni

    The sequence AAA88099.1 differs from that shown. Reason: Erroneous initiation.

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti178 – 1781L → V in AAA36628. (PubMed:3343343)Curated
    Sequence conflicti318 – 3181P → L in AAA88099. (PubMed:3035561)Curated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti131 – 1311T → I.2 Publications
    Corresponds to variant rs1130866 [ dbSNP | Ensembl ].
    VAR_006948
    Natural varianti176 – 1761L → F.1 Publication
    Corresponds to variant rs3024801 [ dbSNP | Ensembl ].
    VAR_013099
    Natural varianti228 – 2281A → I Requires 2 nucleotide substitutions. 1 Publication
    VAR_006950
    Natural varianti228 – 2281A → R Requires 2 nucleotide substitutions. 2 Publications
    VAR_006949
    Natural varianti236 – 2361R → C in SMDP1. 1 Publication
    VAR_036856
    Natural varianti272 – 2721R → H.1 Publication
    Corresponds to variant rs3024809 [ dbSNP | Ensembl ].
    VAR_013100

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    J02761 mRNA. Translation: AAA60212.1.
    M24461 Genomic DNA. Translation: AAB59541.1.
    AF400074 Genomic DNA. Translation: AAK77913.1.
    BC032785 mRNA. Translation: AAH32785.1.
    M16764 mRNA. Translation: AAA88099.1. Different initiation.
    M19097 mRNA. Translation: AAA36628.1.
    PIRiA31361. LNHUB.
    RefSeqiNP_000533.3. NM_000542.3.
    NP_942140.2. NM_198843.2.
    UniGeneiHs.512690.

    Genome annotation databases

    EnsembliENST00000519937; ENSP00000428719; ENSG00000168878.
    GeneIDi6439.
    KEGGihsa:6439.

    Polymorphism databases

    DMDMi131418.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Web resourcesi

    SeattleSNPs

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    J02761 mRNA. Translation: AAA60212.1 .
    M24461 Genomic DNA. Translation: AAB59541.1 .
    AF400074 Genomic DNA. Translation: AAK77913.1 .
    BC032785 mRNA. Translation: AAH32785.1 .
    M16764 mRNA. Translation: AAA88099.1 . Different initiation.
    M19097 mRNA. Translation: AAA36628.1 .
    PIRi A31361. LNHUB.
    RefSeqi NP_000533.3. NM_000542.3.
    NP_942140.2. NM_198843.2.
    UniGenei Hs.512690.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    1DFW infrared - A 201-225 [» ]
    1KMR NMR - A 211-225 [» ]
    1RG3 NMR - A 263-278 [» ]
    1RG4 NMR - A 263-278 [» ]
    1SSZ infrared - A 208-278 [» ]
    2DWF NMR - A 208-278 [» ]
    2JOU NMR - A 208-278 [» ]
    2M0H NMR - A 259-280 [» ]
    2M1T NMR - A 259-278 [» ]
    ProteinModelPortali P07988.
    SMRi P07988. Positions 69-145.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    STRINGi 9606.ENSP00000345161.

    Polymorphism databases

    DMDMi 131418.

    Proteomic databases

    PaxDbi P07988.
    PRIDEi P07988.

    Protocols and materials databases

    DNASUi 6439.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000519937 ; ENSP00000428719 ; ENSG00000168878 .
    GeneIDi 6439.
    KEGGi hsa:6439.

    Organism-specific databases

    CTDi 6439.
    GeneCardsi GC02M085884.
    HGNCi HGNC:10801. SFTPB.
    HPAi CAB002440.
    HPA034820.
    MIMi 178640. gene.
    265120. phenotype.
    267450. phenotype.
    neXtProti NX_P07988.
    Orphaneti 264675. Congenital pulmonary alveolar proteinosis.
    70587. Infant acute respiratory distress syndrome.
    217563. Neonatal acute respiratory distress with surfactant metabolism deficiency.
    PharmGKBi PA35713.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi NOG286450.
    HOGENOMi HOG000115745.
    HOVERGENi HBG006905.
    InParanoidi P07988.
    OrthoDBi EOG7F24TB.
    PhylomeDBi P07988.

    Miscellaneous databases

    ChiTaRSi SFTPB. human.
    EvolutionaryTracei P07988.
    GeneWikii Pulmonary_surfactant-associated_protein_B.
    GenomeRNAii 6439.
    NextBioi 25021.
    PMAP-CutDB P07988.
    PROi P07988.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P07988.
    Bgeei P07988.
    CleanExi HS_SFTPB.
    Genevestigatori P07988.

    Family and domain databases

    Gene3Di 1.10.225.10. 3 hits.
    InterProi IPR003119. SapA.
    IPR007856. SapB_1.
    IPR008138. SapB_2.
    IPR008373. Saposin.
    IPR011001. Saposin-like.
    IPR008139. SaposinB.
    [Graphical view ]
    Pfami PF02199. SapA. 1 hit.
    PF05184. SapB_1. 1 hit.
    PF03489. SapB_2. 2 hits.
    [Graphical view ]
    PRINTSi PR01797. SAPOSIN.
    SMARTi SM00162. SAPA. 1 hit.
    SM00741. SapB. 3 hits.
    [Graphical view ]
    SUPFAMi SSF47862. SSF47862. 3 hits.
    PROSITEi PS51110. SAP_A. 1 hit.
    PS50015. SAP_B. 3 hits.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Isolation of a cDNA clone encoding a high molecular weight precursor to a 6-kDa pulmonary surfactant-associated protein."
      Jacobs K.A., Phelps D.S., Steinbrink R., Fisch J., Kriz R., Mitsock L., Dougherty J.P., Taeusch H.W., Floros J.
      J. Biol. Chem. 262:9808-9811(1987) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], PROTEIN SEQUENCE OF 201-214.
      Tissue: Lung.
    2. "Structure and organization of the gene encoding human pulmonary surfactant proteolipid SP-B."
      Pilot-Matias T.J., Kister S.E., Fox J.L., Kropp K., Glasser S.W., Whitsett J.A.
      DNA 8:75-86(1989) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT ARG-228.
    3. SeattleSNPs variation discovery resource
      Submitted (JUL-2001) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS ILE-131; PHE-176 AND HIS-272.
    4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Brain.
    5. "cDNA and deduced amino acid sequence of human pulmonary surfactant-associated proteolipid SPL(Phe)."
      Glasser S.W., Korfhagen T.R., Weaver T., Pilot-Matias T., Fox J.L., Whitsett J.A.
      Proc. Natl. Acad. Sci. U.S.A. 84:4007-4011(1987) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 99-381, VARIANT ARG-228.
    6. "Use of human surfactant low molecular weight apoproteins in the reconstitution of surfactant biologic activity."
      Revak S.D., Merritt T.A., Degryse E., Stefani L., Courtney M., Hallman M., Cochrane C.G.
      J. Clin. Invest. 81:826-833(1988) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 139-381.
    7. "Human surfactant polypeptide SP-B. Disulfide bridges, C-terminal end, and peptide analysis of the airway form."
      Johansson J., Joernvall H., Curstedt T.
      FEBS Lett. 301:165-167(1992) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 201-279, DISULFIDE BONDS, VARIANT ILE-228.
    8. "Conformational mapping of the N-terminal segment of surfactant protein B in lipid using 13C-enhanced Fourier transform infrared spectroscopy."
      Gordon L.M., Lee K.Y., Lipp M.M., Zasadzinski J.A., Walther F.J., Sherman M.A., Waring A.J.
      J. Pept. Res. 55:330-347(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: STRUCTURE BY FTIR OF 201-225.
    9. "Structure of mini-B, a functional fragment of surfactant protein B, in detergent micelles."
      Sarker M., Waring A.J., Walther F.J., Keough K.M., Booth V.
      Biochemistry 46:11047-11056(2007) [PubMed] [Europe PMC] [Abstract]
      Cited for: STRUCTURE BY NMR OF 208-278.
    10. "Partial deficiency of surfactant protein B in an infant with chronic lung disease."
      Ballard P.L., Nogee L.M., Beers M.F., Ballard R.A., Planer B.C., Polk L., deMello D.E., Moxley M.A., Longmore W.J.
      Pediatrics 96:1046-1052(1995) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SMDP1 CYS-236.
    11. "Polymorphisms of human SP-A, SP-B, and SP-D genes: association of SP-B Thr131Ile with ARDS."
      Lin Z., Pearson C., Chinchilli V., Pietschmann S.M., Luo J., Pison U., Floros J.
      Clin. Genet. 58:181-191(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT ILE-131.
    12. "Surfactant proteins A and B as interactive genetic determinants of neonatal respiratory distress syndrome."
      Haataja R., Raemet M., Marttila R., Hallman M.
      Hum. Mol. Genet. 9:2751-2760(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN SUSCEPTIBILITY TO RDS.

    Entry informationi

    Entry nameiPSPB_HUMAN
    AccessioniPrimary (citable) accession number: P07988
    Secondary accession number(s): Q96R04
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: August 1, 1988
    Last sequence update: May 1, 1992
    Last modified: October 1, 2014
    This is version 169 of the entry and version 3 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    Miscellaneousi

    Miscellaneous

    Pulmonary surfactant consists of 90% lipid and 10% protein. There are 4 surfactant-associated proteins: 2 collagenous, carbohydrate-binding glycoproteins (SP-A and SP-D) and 2 small hydrophobic proteins (SP-B and SP-C).

    Keywords - Technical termi

    3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. Human chromosome 2
      Human chromosome 2: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    6. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3