P11686 · PSPC_HUMAN
- ProteinSurfactant protein C
- GeneSFTPC
- StatusUniProtKB reviewed (Swiss-Prot)
- Organism
- Amino acids197 (go to sequence)
- Protein existenceEvidence at protein level
- Annotation score5/5
Function
function
Pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces.
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 annotations
all annotations | all molecular function | virus receptor activity | dna binding | rna binding | cytoskeletal motor activity | catalytic activity | gtpase activity | structural molecule activity | transporter activity | cytoskeletal protein binding | lipid binding | cyclase activity | antioxidant activity | oxidoreductase activity | transferase activity | hydrolase activity | lyase activity | isomerase activity | ligase activity | protein tag activity | cargo receptor activity | histone binding | protein folding chaperone | translation regulator activity | nutrient reservoir activity | receptor ligand activity | molecular transducer activity | molecular adaptor activity | toxin activity | cell adhesion mediator activity | molecular function regulator activity | virus coreceptor activity | catalytic activity, acting on a protein | catalytic activity, acting on dna | catalytic activity, acting on rna | molecular carrier activity | transcription regulator activity | general transcription initiation factor activity | molecular sensor activity | molecular sequestering activity | atp-dependent activity | other molecular function | all biological process | mitotic cell cycle | cytokinesis | cytoplasmic translation | immune system process | muscle system process | circulatory system process | renal system process | respiratory system process | carbohydrate metabolic process | generation of precursor metabolites and energy | dna replication | dna repair | dna recombination | chromatin organization | dna-templated transcription | regulation of dna-templated transcription | trna metabolic process | protein folding | protein glycosylation | amino acid metabolic process | modified amino acid metabolic process | lipid metabolic process | vitamin metabolic process | sulfur compound metabolic process | intracellular protein transport | nucleocytoplasmic transport | autophagy | inflammatory response | mitochondrion organization | cytoskeleton organization | microtubule-based movement | peroxisome organization | lysosome organization | chromosome segregation | cell adhesion | establishment or maintenance of cell polarity | programmed cell death | photosynthesis | mrna metabolic process | snrna metabolic process | vesicle-mediated transport | reproductive process | digestive system process | signaling | cell differentiation | protein catabolic process | extracellular matrix organization | regulatory ncrna-mediated gene silencing | telomere organization | cell junction organization | wound healing | ribosome biogenesis | cilium organization | anatomical structure development | cell motility | nervous system process | endocrine process | protein maturation | transmembrane transport | nucleobase-containing small molecule metabolic process | hepaticobiliary system process | membrane organization | protein-containing complex assembly | cell wall organization or biogenesis | nitrogen cycle metabolic process | protein localization to plasma membrane | defense response to other organism | detoxification | meiotic nuclear division | mitotic nuclear division | mitochondrial gene expression | carbohydrate derivative metabolic process | other biological process | all cellular component | nuclear chromosome | extracellular region | extracellular space | cell wall | nucleus | nuclear envelope | nucleoplasm | chromosome | nucleolus | mitochondrion | lysosome | endosome | vacuole | peroxisome | endoplasmic reticulum | golgi apparatus | lipid droplet | microtubule organizing center | cytosol | ribosome | cytoskeleton | plasma membrane | cilium | plastid | thylakoid | external encapsulating structure | extracellular matrix | cytoplasmic vesicle | organelle | other cellular component | |||
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Aspect | Term | |
---|---|---|
Cellular Component | alveolar lamellar body | |
Cellular Component | clathrin-coated endocytic vesicle | |
Cellular Component | endoplasmic reticulum membrane | |
Cellular Component | extracellular region | |
Cellular Component | extracellular space | |
Cellular Component | lamellar body | |
Cellular Component | multivesicular body lumen | |
Molecular Function | identical protein binding | |
Biological Process | respiratory gaseous exchange by respiratory system |
Keywords
- Biological process
Enzyme and pathway databases
Names & Taxonomy
Protein names
- Recommended nameSurfactant protein C
- Short namesSP-C
- Alternative names
Gene names
Organism names
- Organism
- Taxonomic lineageEukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo
Accessions
- Primary accessionP11686
- Secondary accessions
Proteomes
Organism-specific databases
Subcellular Location
UniProt Annotation
GO Annotation
Keywords
- Cellular component
Disease & Variants
Involvement in disease
Pulmonary surfactant metabolism dysfunction 2 (SMDP2)
- Note
- DescriptionA rare disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course, 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.
- See alsoMIM:610913
Natural variants in SMDP2
Variant ID | Position(s) | Change | Description | |
---|---|---|---|---|
VAR_036855 | 66 | E>K | in SMDP2; targeted abnormally to early endosomes and likely to result in a toxic gain of function; dbSNP:rs121917836 | |
VAR_026753 | 73 | I>T | in SMDP2; abnormal trafficking and accumulation of aberrantly processed proSPC within alveoli; dbSNP:rs121917834 | |
VAR_026754 | 116 | A>D | in SMDP2; dbSNP:rs121918559 | |
VAR_026755 | 167 | R>Q | in SMDP2; dbSNP:rs34957318 | |
VAR_026756 | 188 | L>Q | in SMDP2; dbSNP:rs121917835 |
Features
Showing features for natural variant.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Natural variant | VAR_036855 | 66 | in SMDP2; targeted abnormally to early endosomes and likely to result in a toxic gain of function; dbSNP:rs121917836 | |||
Sequence: E → K | ||||||
Natural variant | VAR_026753 | 73 | in SMDP2; abnormal trafficking and accumulation of aberrantly processed proSPC within alveoli; dbSNP:rs121917834 | |||
Sequence: I → T | ||||||
Natural variant | VAR_026754 | 116 | in SMDP2; dbSNP:rs121918559 | |||
Sequence: A → D | ||||||
Natural variant | VAR_007453 | 138 | in dbSNP:rs4715 | |||
Sequence: T → N | ||||||
Natural variant | VAR_026755 | 167 | in SMDP2; dbSNP:rs34957318 | |||
Sequence: R → Q | ||||||
Natural variant | VAR_016175 | 186 | in dbSNP:rs1124 | |||
Sequence: S → N | ||||||
Natural variant | VAR_026756 | 188 | in SMDP2; dbSNP:rs121917835 | |||
Sequence: L → Q |
Variants
We now provide the "Disease & Variants" viewer in its own tab.
The viewer provides 232 variants from UniProt as well as other sources including ClinVar and dbSNP.
Keywords
- Disease
Organism-specific databases
Miscellaneous
Genetic variation databases
PTM/Processing
Features
Showing features for propeptide, chain, lipidation, disulfide bond.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Propeptide | PRO_0000033477 | 1-23 | ||||
Sequence: MDVGSKEVLMESPPDYSAAPRGR | ||||||
Chain | PRO_0000033478 | 24-58 | Surfactant protein C | |||
Sequence: FGIPCCPVHLKRLLIVVVVVVLIVVVIVGALLMGL | ||||||
Lipidation | 28 | S-palmitoyl cysteine | ||||
Sequence: C | ||||||
Lipidation | 29 | S-palmitoyl cysteine | ||||
Sequence: C | ||||||
Propeptide | PRO_0000033479 | 59-197 | ||||
Sequence: HMSQKHTEMVLEMSIGAPEAQQRLALSEHLVTTATFSIGSTGLVVYDYQQLLIAYKPAPGTCCYIMKIAPESIPSLEALTRKVHNFQMECSLQAKPAVPTSKLGQAEGRDAGSAPSGGDPAFLGMAVSTLCGEVPLYYI | ||||||
Disulfide bond | 120↔148 | |||||
Sequence: CCYIMKIAPESIPSLEALTRKVHNFQMEC | ||||||
Disulfide bond | 121↔189 | |||||
Sequence: CYIMKIAPESIPSLEALTRKVHNFQMECSLQAKPAVPTSKLGQAEGRDAGSAPSGGDPAFLGMAVSTLC |
Keywords
- PTM
Proteomic databases
PTM databases
Expression
Interaction
Binary interactions
Protein-protein interaction databases
Miscellaneous
Structure
Family & Domains
Features
Showing features for domain.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Domain | 94-197 | BRICHOS | ||||
Sequence: FSIGSTGLVVYDYQQLLIAYKPAPGTCCYIMKIAPESIPSLEALTRKVHNFQMECSLQAKPAVPTSKLGQAEGRDAGSAPSGGDPAFLGMAVSTLCGEVPLYYI |
Phylogenomic databases
Family and domain databases
Sequence & Isoform
- Sequence statusComplete
- Sequence processingThe displayed sequence is further processed into a mature form.
This entry describes 2 isoforms produced by Alternative splicing.
P11686-1
This isoform has been chosen as the canonical sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
- Name1
- Length197
- Mass (Da)21,013
- Last updated2024-01-24 v3
- ChecksumC26A21E30F60B062
P11686-2
- Name2
- SynonymsC1
- Differences from canonical
- 146-151: Missing
Computationally mapped potential isoform sequences
There are 5 potential isoforms mapped to this entry
Features
Showing features for sequence conflict, alternative sequence.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Sequence conflict | 14 | in Ref. 4; AAB60332 | ||||
Sequence: P → PPCQ | ||||||
Sequence conflict | 45 | in Ref. 4; AAB60332 | ||||
Sequence: L → S | ||||||
Sequence conflict | 65-67 | in Ref. 4; AAB60332 | ||||
Sequence: TEM → FPQ | ||||||
Alternative sequence | VSP_006311 | 146-151 | in isoform 2 | |||
Sequence: Missing |
Keywords
- Coding sequence diversity
- Technical term
Sequence databases
Nucleotide Sequence | Protein Sequence | Molecule Type | Status | |
---|---|---|---|---|
J03553 EMBL· GenBank· DDBJ | AAA36631.1 EMBL· GenBank· DDBJ | mRNA | ||
J03517 EMBL· GenBank· DDBJ | AAA36634.1 EMBL· GenBank· DDBJ | mRNA | ||
J03890 EMBL· GenBank· DDBJ | AAC32022.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
J03890 EMBL· GenBank· DDBJ | AAC32023.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
U02948 EMBL· GenBank· DDBJ | AAB60332.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
AY357924 EMBL· GenBank· DDBJ | AAQ67734.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
AK315742 EMBL· GenBank· DDBJ | BAG38097.1 EMBL· GenBank· DDBJ | mRNA | ||
DQ884411 EMBL· GenBank· DDBJ | ABI63378.1 EMBL· GenBank· DDBJ | mRNA | ||
KU178330 EMBL· GenBank· DDBJ | ALQ33788.1 EMBL· GenBank· DDBJ | mRNA | ||
AY337315 EMBL· GenBank· DDBJ | AAP88034.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
AC105206 EMBL· GenBank· DDBJ | - | Genomic DNA | No translation available. | |
CH471080 EMBL· GenBank· DDBJ | EAW63707.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
BC005913 EMBL· GenBank· DDBJ | AAH05913.1 EMBL· GenBank· DDBJ | mRNA |