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P07988

- PSPB_HUMAN

UniProt

P07988 - PSPB_HUMAN

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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

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
Complete GO annotation...

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 region Source: UniProtKB-KW
  2. lysosome Source: InterPro
Complete GO annotation...

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

BgeeiP07988.
CleanExiHS_SFTPB.
ExpressionAtlasiP07988. baseline and differential.
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.
GeneTreeiENSGT00530000063434.
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

        10         20         30         40         50
MAESHLLQWL LLLLPTLCGP GTAAWTTSSL ACAQGPEFWC QSLEQALQCR
60 70 80 90 100
ALGHCLQEVW GHVGADDLCQ ECEDIVHILN KMAKEAIFQD TMRKFLEQEC
110 120 130 140 150
NVLPLKLLMP QCNQVLDDYF PLVIDYFQNQ TDSNGICMHL GLCKSRQPEP
160 170 180 190 200
EQEPGMSDPL PKPLRDPLPD PLLDKLVLPV LPGALQARPG PHTQDLSEQQ
210 220 230 240 250
FPIPLPYCWL CRALIKRIQA MIPKGALAVA VAQVCRVVPL VAGGICQCLA
260 270 280 290 300
ERYSVILLDT LLGRMLPQLV CRLVLRCSMD DSAGPRSPTG EWLPRDSECH
310 320 330 340 350
LCMSVTTQAG NSSEQAIPQA MLQACVGSWL DREKCKQFVE QHTPQLLTLV
360 370 380
PRGWDAHTTC QALGVCGTMS SPLQCIHSPD L
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

EnsembliENST00000393822; ENSP00000377409; ENSG00000168878.
ENST00000519937; 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 ENST00000393822 ; ENSP00000377409 ; ENSG00000168878 .
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.
GeneTreei ENSGT00530000063434.
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

Bgeei P07988.
CleanExi HS_SFTPB.
ExpressionAtlasi P07988. baseline and differential.
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

« Hide 'large scale' publications
  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 29, 2014
This is version 170 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