UniProtKB - P07988 (PSPB_HUMAN)
Protein
Pulmonary surfactant-associated protein B
Gene
SFTPB
Organism
Homo sapiens (Human)
Status
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.
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).
GO - Biological processi
- animal organ morphogenesis Source: ProtInc
- cellular protein metabolic process Source: Reactome
- respiratory gaseous exchange by respiratory system Source: ProtInc
- sphingolipid metabolic process Source: InterPro
Keywordsi
Biological process | Gaseous exchange |
Enzyme and pathway databases
PathwayCommonsi | P07988 |
Reactomei | R-HSA-5683826, Surfactant metabolism R-HSA-5688031, Defective pro-SFTPB causes pulmonary surfactant metabolism dysfunction 1 (SMDP1) and respiratory distress syndrome (RDS) R-HSA-5688849, Defective CSF2RB causes pulmonary surfactant metabolism dysfunction 5 (SMDP5) R-HSA-5688890, Defective CSF2RA causes pulmonary surfactant metabolism dysfunction 4 (SMDP4) |
SIGNORi | P07988 |
Names & Taxonomyi
Protein namesi | Recommended name: Pulmonary surfactant-associated protein BShort 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 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
EuPathDBi | HostDB:ENSG00000168878.16 |
HGNCi | HGNC:10801, SFTPB |
MIMi | 178640, gene |
neXtProti | NX_P07988 |
Subcellular locationi
Extracellular region or secreted
Endoplasmic reticulum
- endoplasmic reticulum membrane Source: Reactome
Endosome
- multivesicular body Source: GO_Central
- multivesicular body lumen Source: Reactome
Extracellular region or secreted
- extracellular region Source: Reactome
- extracellular space Source: GO_Central
Lysosome
- lysosome Source: InterPro
Nucleus
- nucleoplasm Source: Reactome
Other locations
- alveolar lamellar body Source: GO_Central
- clathrin-coated endocytic vesicle Source: Reactome
- lamellar body Source: Reactome
Keywords - Cellular componenti
Secreted, Surface filmPathology & Biotechi
Involvement in diseasei
Pulmonary surfactant metabolism dysfunction 1 (SMDP1)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA 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.
Related information in OMIMFeature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_036856 | 236 | R → C in SMDP1. 1 PublicationCorresponds to variant dbSNP:rs137853202EnsemblClinVar. | 1 |
Respiratory distress syndrome in premature infants (RDS)1 Publication
Disease susceptibility may be 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.
Disease descriptionA 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'.
Related information in OMIMKeywords - Diseasei
Disease mutationOrganism-specific databases
DisGeNETi | 6439 |
MalaCardsi | SFTPB |
MIMi | 265120, phenotype 267450, phenotype |
OpenTargetsi | ENSG00000168878 |
Orphaneti | 70587, Infant acute respiratory distress syndrome 217563, Neonatal acute respiratory distress due to SP-B deficiency |
PharmGKBi | PA35713 |
Miscellaneous databases
Pharosi | P07988, Tbio |
Polymorphism and mutation databases
BioMutai | SFTPB |
DMDMi | 131418 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 24 | Sequence analysisAdd BLAST | 24 | |
PropeptideiPRO_0000031647 | 25 – 200 | Add BLAST | 176 | |
ChainiPRO_0000031648 | 201 – 279 | Pulmonary surfactant-associated protein BAdd BLAST | 79 | |
PropeptideiPRO_0000031649 | 280 – 381 | Add BLAST | 102 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Disulfide bondi | 69 ↔ 143 | PROSITE-ProRule annotation | ||
Disulfide bondi | 72 ↔ 137 | PROSITE-ProRule annotation | ||
Disulfide bondi | 100 ↔ 112 | PROSITE-ProRule annotation | ||
Glycosylationi | 129 | N-linked (GlcNAc...) asparaginePROSITE-ProRule annotation | 1 | |
Disulfide bondi | 208 ↔ 277 | PROSITE-ProRule annotation1 Publication | ||
Disulfide bondi | 211 ↔ 271 | PROSITE-ProRule annotation1 Publication | ||
Disulfide bondi | 235 ↔ 246 | PROSITE-ProRule annotation1 Publication | ||
Disulfide bondi | 248 | InterchainPROSITE-ProRule annotation1 Publication | ||
Disulfide bondi | 299 ↔ 366 | PROSITE-ProRule annotation | ||
Disulfide bondi | 302 ↔ 360 | PROSITE-ProRule annotation | ||
Glycosylationi | 311 | N-linked (GlcNAc...) asparaginePROSITE-ProRule annotation | 1 | |
Disulfide bondi | 325 ↔ 335 | PROSITE-ProRule annotation |
Keywords - PTMi
Disulfide bond, GlycoproteinProteomic databases
MassIVEi | P07988 |
PaxDbi | P07988 |
PeptideAtlasi | P07988 |
PRIDEi | P07988 |
ProteomicsDBi | 52054 |
PTM databases
GlyGeni | P07988, 2 sites |
Expressioni
Gene expression databases
Bgeei | ENSG00000168878, Expressed in visceral pleura and 217 other tissues |
ExpressionAtlasi | P07988, baseline and differential |
Genevisiblei | P07988, HS |
Organism-specific databases
HPAi | ENSG00000168878, Tissue enriched (lung) |
Interactioni
Subunit structurei
Homodimer; disulfide-linked.
1 PublicationBinary interactionsi
P07988
With | #Exp. | IntAct |
---|---|---|
MEOX2 [Q6FHY5] | 3 | EBI-14955352,EBI-16439278 |
Protein-protein interaction databases
BioGRIDi | 112337, 1 interactor |
IntActi | P07988, 1 interactor |
STRINGi | 9606.ENSP00000386346 |
Miscellaneous databases
RNActi | P07988, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
BMRBi | P07988 |
PCDDBi | P07988 |
SMRi | P07988 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P07988 |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 25 – 65 | Saposin A-typePROSITE-ProRule annotationAdd BLAST | 41 | |
Domaini | 65 – 147 | Saposin B-type 1PROSITE-ProRule annotationAdd BLAST | 83 | |
Domaini | 204 – 281 | Saposin B-type 2PROSITE-ProRule annotationAdd BLAST | 78 | |
Domaini | 295 – 370 | Saposin B-type 3PROSITE-ProRule annotationAdd BLAST | 76 |
Keywords - Domaini
Repeat, SignalPhylogenomic databases
eggNOGi | KOG1340, Eukaryota |
GeneTreei | ENSGT00940000161711 |
InParanoidi | P07988 |
OrthoDBi | 865505at2759 |
PhylomeDBi | P07988 |
Family and domain databases
InterProi | View protein in InterPro IPR003119, SAP_A IPR007856, SapB_1 IPR008138, SapB_2 IPR008373, Saposin IPR011001, Saposin-like IPR008139, SaposinB_dom |
Pfami | View protein in Pfam PF02199, SapA, 1 hit PF05184, SapB_1, 1 hit PF03489, SapB_2, 2 hits |
PRINTSi | PR01797, SAPOSIN |
SMARTi | View protein in SMART SM00162, SAPA, 1 hit SM00741, SapB, 3 hits |
SUPFAMi | SSF47862, SSF47862, 3 hits |
PROSITEi | View protein in PROSITE PS51110, SAP_A, 1 hit PS50015, SAP_B, 3 hits |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All
P07988-1 [UniParc]FASTAAdd to basket
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
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketH0Y7V6 | H0Y7V6_HUMAN | Pulmonary surfactant-associated pro... | SFTPB | 374 | Annotation score: | ||
U3KQT2 | U3KQT2_HUMAN | Pulmonary surfactant-associated pro... | SFTPB | 92 | Annotation score: |
Sequence cautioni
The sequence AAA88099 differs from that shown. Reason: Erroneous initiation.Curated
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 178 | L → V in AAA36628 (PubMed:3343343).Curated | 1 | |
Sequence conflicti | 318 | P → L in AAA88099 (PubMed:3035561).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_006948 | 131 | T → I2 PublicationsCorresponds to variant dbSNP:rs1130866EnsemblClinVar. | 1 | |
Natural variantiVAR_013099 | 176 | L → F1 PublicationCorresponds to variant dbSNP:rs3024801EnsemblClinVar. | 1 | |
Natural variantiVAR_006950 | 228 | A → I Requires 2 nucleotide substitutions. 1 Publication | 1 | |
Natural variantiVAR_006949 | 228 | A → R Requires 2 nucleotide substitutions. 2 Publications | 1 | |
Natural variantiVAR_036856 | 236 | R → C in SMDP1. 1 PublicationCorresponds to variant dbSNP:rs137853202EnsemblClinVar. | 1 | |
Natural variantiVAR_013100 | 272 | R → H1 PublicationCorresponds to variant dbSNP:rs3024809EnsemblClinVar. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi 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 |
CCDSi | CCDS1983.2 |
PIRi | A31361, LNHUB |
RefSeqi | NP_000533.3, NM_000542.3 NP_942140.2, NM_198843.2 |
Genome annotation databases
Ensembli | ENST00000393822; ENSP00000377409; ENSG00000168878 ENST00000409383; ENSP00000386346; ENSG00000168878 ENST00000519937; ENSP00000428719; ENSG00000168878 |
GeneIDi | 6439 |
KEGGi | hsa:6439 |
UCSCi | uc061lja.1, human |
Keywords - Coding sequence diversityi
PolymorphismSimilar proteinsi
Cross-referencesi
Web resourcesi
SeattleSNPs |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi 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 |
CCDSi | CCDS1983.2 |
PIRi | A31361, LNHUB |
RefSeqi | NP_000533.3, NM_000542.3 NP_942140.2, NM_198843.2 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB 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 | [»] | |
BMRBi | P07988 | |||||
PCDDBi | P07988 | |||||
SMRi | P07988 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 112337, 1 interactor |
IntActi | P07988, 1 interactor |
STRINGi | 9606.ENSP00000386346 |
PTM databases
GlyGeni | P07988, 2 sites |
Polymorphism and mutation databases
BioMutai | SFTPB |
DMDMi | 131418 |
Proteomic databases
MassIVEi | P07988 |
PaxDbi | P07988 |
PeptideAtlasi | P07988 |
PRIDEi | P07988 |
ProteomicsDBi | 52054 |
Protocols and materials databases
Antibodypediai | 31946, 348 antibodies |
DNASUi | 6439 |
Genome annotation databases
Ensembli | ENST00000393822; ENSP00000377409; ENSG00000168878 ENST00000409383; ENSP00000386346; ENSG00000168878 ENST00000519937; ENSP00000428719; ENSG00000168878 |
GeneIDi | 6439 |
KEGGi | hsa:6439 |
UCSCi | uc061lja.1, human |
Organism-specific databases
CTDi | 6439 |
DisGeNETi | 6439 |
EuPathDBi | HostDB:ENSG00000168878.16 |
GeneCardsi | SFTPB |
HGNCi | HGNC:10801, SFTPB |
HPAi | ENSG00000168878, Tissue enriched (lung) |
MalaCardsi | SFTPB |
MIMi | 178640, gene 265120, phenotype 267450, phenotype |
neXtProti | NX_P07988 |
OpenTargetsi | ENSG00000168878 |
Orphaneti | 70587, Infant acute respiratory distress syndrome 217563, Neonatal acute respiratory distress due to SP-B deficiency |
PharmGKBi | PA35713 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG1340, Eukaryota |
GeneTreei | ENSGT00940000161711 |
InParanoidi | P07988 |
OrthoDBi | 865505at2759 |
PhylomeDBi | P07988 |
Enzyme and pathway databases
PathwayCommonsi | P07988 |
Reactomei | R-HSA-5683826, Surfactant metabolism R-HSA-5688031, Defective pro-SFTPB causes pulmonary surfactant metabolism dysfunction 1 (SMDP1) and respiratory distress syndrome (RDS) R-HSA-5688849, Defective CSF2RB causes pulmonary surfactant metabolism dysfunction 5 (SMDP5) R-HSA-5688890, Defective CSF2RA causes pulmonary surfactant metabolism dysfunction 4 (SMDP4) |
SIGNORi | P07988 |
Miscellaneous databases
BioGRID-ORCSi | 6439, 6 hits in 841 CRISPR screens |
ChiTaRSi | SFTPB, human |
EvolutionaryTracei | P07988 |
GeneWikii | Pulmonary_surfactant-associated_protein_B |
GenomeRNAii | 6439 |
Pharosi | P07988, Tbio |
PROi | PR:P07988 |
RNActi | P07988, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000168878, Expressed in visceral pleura and 217 other tissues |
ExpressionAtlasi | P07988, baseline and differential |
Genevisiblei | P07988, HS |
Family and domain databases
InterProi | View protein in InterPro IPR003119, SAP_A IPR007856, SapB_1 IPR008138, SapB_2 IPR008373, Saposin IPR011001, Saposin-like IPR008139, SaposinB_dom |
Pfami | View protein in Pfam PF02199, SapA, 1 hit PF05184, SapB_1, 1 hit PF03489, SapB_2, 2 hits |
PRINTSi | PR01797, SAPOSIN |
SMARTi | View protein in SMART SM00162, SAPA, 1 hit SM00741, SapB, 3 hits |
SUPFAMi | SSF47862, SSF47862, 3 hits |
PROSITEi | View protein in PROSITE PS51110, SAP_A, 1 hit PS50015, SAP_B, 3 hits |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | PSPB_HUMAN | |
Accessioni | P07988Primary (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: | December 2, 2020 | |
This is version 211 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Keywords - Technical termi
3D-structure, Direct protein sequencing, Reference proteomeDocuments
- Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - Human chromosome 2
Human chromosome 2: entries, gene names and cross-references to MIM - Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations