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P11686

- PSPC_HUMAN

UniProt

P11686 - PSPC_HUMAN

Protein

Pulmonary surfactant-associated protein C

Gene

SFTPC

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 156 (01 Oct 2014)
      Sequence version 2 (01 Apr 1990)
      Previous versions | rss
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    Functioni

    Pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces.

    GO - Molecular functioni

    1. protein binding Source: UniProtKB

    GO - Biological processi

    1. cellular response to mechanical stimulus Source: Ensembl
    2. cellular response to nitric oxide Source: Ensembl
    3. circadian rhythm Source: Ensembl
    4. protein homooligomerization Source: Ensembl
    5. respiratory gaseous exchange Source: UniProtKB-KW
    6. response to cAMP Source: Ensembl
    7. response to glucocorticoid Source: Ensembl
    8. response to glucose Source: Ensembl
    9. response to growth factor Source: Ensembl
    10. response to hyperoxia Source: Ensembl
    11. response to interleukin-6 Source: Ensembl
    12. response to lipopolysaccharide Source: Ensembl
    13. response to retinoic acid Source: Ensembl
    14. response to vitamin A Source: Ensembl

    Keywords - Biological processi

    Gaseous exchange

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Pulmonary surfactant-associated protein C
    Short name:
    SP-C
    Alternative name(s):
    Pulmonary surfactant-associated proteolipid SPL(Val)
    SP5
    Gene namesi
    Name:SFTPC
    Synonyms:SFTP2
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 8

    Organism-specific databases

    HGNCiHGNC:10802. SFTPC.

    Subcellular locationi

    GO - Cellular componenti

    1. alveolar lamellar body Source: Ensembl
    2. extracellular space Source: UniProtKB-SubCell
    3. multivesicular body Source: Ensembl

    Keywords - Cellular componenti

    Secreted, Surface film

    Pathology & Biotechi

    Involvement in diseasei

    Pulmonary surfactant metabolism dysfunction 2 (SMDP2) [MIM:610913]: A 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.5 Publications
    Note: The disease is caused by mutations affecting the gene represented in this entry.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti66 – 661E → K in SMDP2; targeted abnormally to early endosomes and likely to result in a toxic gain of function. 1 Publication
    VAR_036855
    Natural varianti73 – 731I → T in SMDP2; abnormal trafficking and accumulation of aberrantly processed proSPC within alveoli. 3 Publications
    VAR_026753
    Natural varianti116 – 1161A → D in SMDP2. 1 Publication
    VAR_026754
    Natural varianti167 – 1671R → Q in SMDP2. 1 Publication
    Corresponds to variant rs34957318 [ dbSNP | Ensembl ].
    VAR_026755
    Natural varianti188 – 1881L → Q in SMDP2. 1 Publication
    VAR_026756
    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.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti138 – 1381N → T Influences susceptibility to RDS in premature infants. 8 Publications
    Corresponds to variant rs4715 [ dbSNP | Ensembl ].
    VAR_007453
    Natural varianti186 – 1861N → S Influences susceptibility to RDS in premature infants. 7 Publications
    Corresponds to variant rs1124 [ dbSNP | Ensembl ].
    VAR_016175

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MIMi267450. phenotype.
    610913. phenotype.
    Orphaneti217566. Chronic respiratory distress with surfactant metabolism deficiency.
    264675. Congenital pulmonary alveolar proteinosis.
    98852. Desquamative interstitial pneumonia.
    2032. Idiopathic pulmonary fibrosis.
    70587. Infant acute respiratory distress syndrome.
    PharmGKBiPA35714.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Propeptidei1 – 23231 PublicationPRO_0000033477Add
    BLAST
    Chaini24 – 5835Pulmonary surfactant-associated protein CPRO_0000033478Add
    BLAST
    Propeptidei59 – 197139PRO_0000033479Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Lipidationi28 – 281S-palmitoyl cysteine1 Publication
    Lipidationi29 – 291S-palmitoyl cysteine1 Publication
    Disulfide bondi120 ↔ 1481 Publication
    Disulfide bondi121 ↔ 1891 Publication

    Keywords - PTMi

    Disulfide bond, Lipoprotein, Palmitate

    Proteomic databases

    PaxDbiP11686.
    PRIDEiP11686.

    PTM databases

    PhosphoSiteiP11686.

    Expressioni

    Gene expression databases

    ArrayExpressiP11686.
    BgeeiP11686.
    CleanExiHS_SFTPC.
    GenevestigatoriP11686.

    Organism-specific databases

    HPAiCAB009313.
    HPA010928.

    Interactioni

    Protein-protein interaction databases

    BioGridi112338. 9 interactions.
    STRINGi9606.ENSP00000316152.

    Structurei

    Secondary structure

    1
    197
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Beta strandi91 – 966
    Turni97 – 993
    Beta strandi100 – 1056
    Turni106 – 1094
    Beta strandi110 – 1167
    Beta strandi121 – 1255
    Helixi134 – 14310
    Helixi183 – 1897
    Beta strandi194 – 1974

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    2YADX-ray2.20A/B/C/D/E/F59-197[»]
    ProteinModelPortaliP11686.
    SMRiP11686. Positions 87-197.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini94 – 197104BRICHOSPROSITE-ProRule annotationAdd
    BLAST

    Sequence similaritiesi

    Contains 1 BRICHOS domain.PROSITE-ProRule annotation

    Phylogenomic databases

    eggNOGiNOG47102.
    HOGENOMiHOG000115746.
    HOVERGENiHBG000160.
    InParanoidiP11686.
    OrthoDBiEOG7QVM46.
    PhylomeDBiP11686.
    TreeFamiTF337317.

    Family and domain databases

    InterProiIPR007084. BRICHOS_dom.
    IPR001729. Pulm_surfact_AP.
    IPR018051. SP-C_palmitoylation_site.
    IPR015091. Surfactant_protein_propep.
    [Graphical view]
    PfamiPF04089. BRICHOS. 1 hit.
    PF08999. SP_C-Propep. 1 hit.
    [Graphical view]
    SMARTiSM01039. BRICHOS. 1 hit.
    SM00019. SF_P. 1 hit.
    [Graphical view]
    PROSITEiPS50869. BRICHOS. 1 hit.
    PS00341. SURFACT_PALMITOYL. 1 hit.
    [Graphical view]

    Sequences (2)i

    Sequence statusi: Complete.

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

    This entry describes 2 isoformsi produced by alternative splicing. Align

    Isoform 1 (identifier: P11686-1) [UniParc]FASTAAdd to Basket

    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.

    « Hide

    MDVGSKEVLM ESPPDYSAAP RGRFGIPCCP VHLKRLLIVV VVVVLIVVVI    50
    VGALLMGLHM SQKHTEMVLE MSIGAPEAQQ RLALSEHLVT TATFSIGSTG 100
    LVVYDYQQLL IAYKPAPGTC CYIMKIAPES IPSLEALNRK VHNFQMECSL 150
    QAKPAVPTSK LGQAEGRDAG SAPSGGDPAF LGMAVNTLCG EVPLYYI 197
    Length:197
    Mass (Da):21,053
    Last modified:April 1, 1990 - v2
    Checksum:iC26A21E33C60AA78
    GO
    Isoform 2 (identifier: P11686-2) [UniParc]FASTAAdd to Basket

    Also known as: C1

    The sequence of this isoform differs from the canonical sequence as follows:
         146-151: Missing.

    Show »
    Length:191
    Mass (Da):20,361
    Checksum:i4C45300448DE03FA
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti14 – 141P → PPCQ in AAB60332. (PubMed:8181452)Curated
    Sequence conflicti45 – 451L → S in AAB60332. (PubMed:8181452)Curated
    Sequence conflicti65 – 673TEM → FPQ in AAB60332. (PubMed:8181452)Curated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti66 – 661E → K in SMDP2; targeted abnormally to early endosomes and likely to result in a toxic gain of function. 1 Publication
    VAR_036855
    Natural varianti73 – 731I → T in SMDP2; abnormal trafficking and accumulation of aberrantly processed proSPC within alveoli. 3 Publications
    VAR_026753
    Natural varianti116 – 1161A → D in SMDP2. 1 Publication
    VAR_026754
    Natural varianti138 – 1381N → T Influences susceptibility to RDS in premature infants. 8 Publications
    Corresponds to variant rs4715 [ dbSNP | Ensembl ].
    VAR_007453
    Natural varianti167 – 1671R → Q in SMDP2. 1 Publication
    Corresponds to variant rs34957318 [ dbSNP | Ensembl ].
    VAR_026755
    Natural varianti186 – 1861N → S Influences susceptibility to RDS in premature infants. 7 Publications
    Corresponds to variant rs1124 [ dbSNP | Ensembl ].
    VAR_016175
    Natural varianti188 – 1881L → Q in SMDP2. 1 Publication
    VAR_026756

    Alternative sequence

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Alternative sequencei146 – 1516Missing in isoform 2. 1 PublicationVSP_006311

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    J03553 mRNA. Translation: AAA36631.1.
    J03517 mRNA. Translation: AAA36634.1.
    J03890 Genomic DNA. Translation: AAC32022.1.
    J03890 Genomic DNA. Translation: AAC32023.1.
    U02948 Genomic DNA. Translation: AAB60332.1.
    AY357924 Genomic DNA. Translation: AAQ67734.1.
    AK315742 mRNA. Translation: BAG38097.1.
    DQ884411 mRNA. Translation: ABI63378.1.
    AY337315 Genomic DNA. Translation: AAP88034.1.
    AC105206 Genomic DNA. No translation available.
    CH471080 Genomic DNA. Translation: EAW63707.1.
    BC005913 mRNA. Translation: AAH05913.1.
    CCDSiCCDS43722.1. [P11686-1]
    CCDS55209.1. [P11686-2]
    PIRiA28801. LNHUC.
    RefSeqiNP_001165828.1. NM_001172357.1.
    NP_001165881.1. NM_001172410.1.
    NP_003009.2. NM_003018.3.
    UniGeneiHs.1074.

    Genome annotation databases

    EnsembliENST00000318561; ENSP00000316152; ENSG00000168484.
    GeneIDi6440.
    KEGGihsa:6440.
    UCSCiuc003xax.4. human. [P11686-1]

    Polymorphism databases

    DMDMi131425.

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    Cross-referencesi

    Web resourcesi

    SeattleSNPs

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    J03553 mRNA. Translation: AAA36631.1 .
    J03517 mRNA. Translation: AAA36634.1 .
    J03890 Genomic DNA. Translation: AAC32022.1 .
    J03890 Genomic DNA. Translation: AAC32023.1 .
    U02948 Genomic DNA. Translation: AAB60332.1 .
    AY357924 Genomic DNA. Translation: AAQ67734.1 .
    AK315742 mRNA. Translation: BAG38097.1 .
    DQ884411 mRNA. Translation: ABI63378.1 .
    AY337315 Genomic DNA. Translation: AAP88034.1 .
    AC105206 Genomic DNA. No translation available.
    CH471080 Genomic DNA. Translation: EAW63707.1 .
    BC005913 mRNA. Translation: AAH05913.1 .
    CCDSi CCDS43722.1. [P11686-1 ]
    CCDS55209.1. [P11686-2 ]
    PIRi A28801. LNHUC.
    RefSeqi NP_001165828.1. NM_001172357.1.
    NP_001165881.1. NM_001172410.1.
    NP_003009.2. NM_003018.3.
    UniGenei Hs.1074.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    2YAD X-ray 2.20 A/B/C/D/E/F 59-197 [» ]
    ProteinModelPortali P11686.
    SMRi P11686. Positions 87-197.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 112338. 9 interactions.
    STRINGi 9606.ENSP00000316152.

    PTM databases

    PhosphoSitei P11686.

    Polymorphism databases

    DMDMi 131425.

    Proteomic databases

    PaxDbi P11686.
    PRIDEi P11686.

    Protocols and materials databases

    DNASUi 6440.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000318561 ; ENSP00000316152 ; ENSG00000168484 .
    GeneIDi 6440.
    KEGGi hsa:6440.
    UCSCi uc003xax.4. human. [P11686-1 ]

    Organism-specific databases

    CTDi 6440.
    GeneCardsi GC08P022019.
    GeneReviewsi SFTPC.
    H-InvDB HIX0007365.
    HGNCi HGNC:10802. SFTPC.
    HPAi CAB009313.
    HPA010928.
    MIMi 178620. gene.
    267450. phenotype.
    610913. phenotype.
    neXtProti NX_P11686.
    Orphaneti 217566. Chronic respiratory distress with surfactant metabolism deficiency.
    264675. Congenital pulmonary alveolar proteinosis.
    98852. Desquamative interstitial pneumonia.
    2032. Idiopathic pulmonary fibrosis.
    70587. Infant acute respiratory distress syndrome.
    PharmGKBi PA35714.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi NOG47102.
    HOGENOMi HOG000115746.
    HOVERGENi HBG000160.
    InParanoidi P11686.
    OrthoDBi EOG7QVM46.
    PhylomeDBi P11686.
    TreeFami TF337317.

    Miscellaneous databases

    ChiTaRSi SFTPC. human.
    GeneWikii Pulmonary_surfactant-associated_protein_C.
    GenomeRNAii 6440.
    NextBioi 25027.
    PROi P11686.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P11686.
    Bgeei P11686.
    CleanExi HS_SFTPC.
    Genevestigatori P11686.

    Family and domain databases

    InterProi IPR007084. BRICHOS_dom.
    IPR001729. Pulm_surfact_AP.
    IPR018051. SP-C_palmitoylation_site.
    IPR015091. Surfactant_protein_propep.
    [Graphical view ]
    Pfami PF04089. BRICHOS. 1 hit.
    PF08999. SP_C-Propep. 1 hit.
    [Graphical view ]
    SMARTi SM01039. BRICHOS. 1 hit.
    SM00019. SF_P. 1 hit.
    [Graphical view ]
    PROSITEi PS50869. BRICHOS. 1 hit.
    PS00341. SURFACT_PALMITOYL. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Low molecular weight human pulmonary surfactant protein (SP5): isolation, characterization, and cDNA and amino acid sequences."
      Warr R.G., Hawgood S., Buckley D.I., Crisp T.M., Schilling J., Benson B.J., Ballard P.L., Clements J.A., White R.T.
      Proc. Natl. Acad. Sci. U.S.A. 84:7915-7919(1987) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    2. "cDNA, deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid, SPL(pVal)."
      Glasser S.W., Korfhagen T.R., Weaver T.E., Clark J.C., Pilot-Matias T., Meuth J., Fox J.L., Whitsett J.A.
      J. Biol. Chem. 263:9-12(1988) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), PROTEIN SEQUENCE OF 26-42.
    3. "Two SP-C genes encoding human pulmonary surfactant proteolipid."
      Glasser S.W., Korfhagen T.R., Perme C.M., Pilot-Matias T.J., Kister S.E., Whitsett J.A.
      J. Biol. Chem. 263:10326-10331(1988) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], ALTERNATIVE SPLICING, VARIANT THR-138.
    4. "Human surfactant protein-C: genetic homogeneity and expression in RDS; comparison with other species."
      Hatzis D., Deiter G., deMello D.E., Floros J.
      Exp. Lung Res. 20:57-72(1994) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORM 1), VARIANTS THR-138 AND SER-186.
    5. Frerking I., Stevens P., Pison U., Witt H.
      Submitted (AUG-2003) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORM 1), VARIANTS THR-138 AND SER-186.
    6. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
      , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
      Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), VARIANTS THR-138 AND SER-186.
      Tissue: Lung.
    7. "A computer system platform used to predict novel genes."
      Yu Z., Zheng Z., Tang T., Fu Y.
      Submitted (JUL-2006) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
    8. SeattleSNPs variation discovery resource
      Submitted (JUL-2003) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS THR-138 AND SER-186.
    9. "DNA sequence and analysis of human chromosome 8."
      Nusbaum C., Mikkelsen T.S., Zody M.C., Asakawa S., Taudien S., Garber M., Kodira C.D., Schueler M.G., Shimizu A., Whittaker C.A., Chang J.L., Cuomo C.A., Dewar K., FitzGerald M.G., Yang X., Allen N.R., Anderson S., Asakawa T.
      , Blechschmidt K., Bloom T., Borowsky M.L., Butler J., Cook A., Corum B., DeArellano K., DeCaprio D., Dooley K.T., Dorris L. III, Engels R., Gloeckner G., Hafez N., Hagopian D.S., Hall J.L., Ishikawa S.K., Jaffe D.B., Kamat A., Kudoh J., Lehmann R., Lokitsang T., Macdonald P., Major J.E., Matthews C.D., Mauceli E., Menzel U., Mihalev A.H., Minoshima S., Murayama Y., Naylor J.W., Nicol R., Nguyen C., O'Leary S.B., O'Neill K., Parker S.C.J., Polley A., Raymond C.K., Reichwald K., Rodriguez J., Sasaki T., Schilhabel M., Siddiqui R., Smith C.L., Sneddon T.P., Talamas J.A., Tenzin P., Topham K., Venkataraman V., Wen G., Yamazaki S., Young S.K., Zeng Q., Zimmer A.R., Rosenthal A., Birren B.W., Platzer M., Shimizu N., Lander E.S.
      Nature 439:331-335(2006) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANTS THR-138 AND SER-186.
    10. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANTS THR-138 AND SER-186.
    11. "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] (ISOFORM 1).
      Tissue: Lung.
    12. "Hydrophobic 3.7 kDa surfactant polypeptide: structural characterization of the human and bovine forms."
      Johansson J., Joernvall H., Eklund A., Christensen N., Robertson B., Curstedt T.
      FEBS Lett. 232:61-64(1988) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 24-58.
    13. "Hydrophobic surfactant-associated polypeptides: SP-C is a lipopeptide with two palmitoylated cysteine residues, whereas SP-B lacks covalently linked fatty acyl groups."
      Curstedt T., Johansson J., Persson P., Eklund A., Robertson B., Loewenadler B., Joernvall H.
      Proc. Natl. Acad. Sci. U.S.A. 87:2985-2989(1990) [PubMed] [Europe PMC] [Abstract]
      Cited for: PALMITOYLATION AT CYS-28 AND CYS-29.
    14. "High-resolution structure of a BRICHOS domain and its implications for anti-amyloid chaperone activity on lung surfactant protein C."
      Willander H., Askarieh G., Landreh M., Westermark P., Nordling K., Keranen H., Hermansson E., Hamvas A., Nogee L.M., Bergman T., Saenz A., Casals C., Aqvistg J., Jornvall H., Berglund H., Presto J., Knight S.D., Johansson J.
      Proc. Natl. Acad. Sci. U.S.A. 109:2325-2329(2012) [PubMed] [Europe PMC] [Abstract]
      Cited for: X-RAY CRYSTALLOGRAPHY (2.2 ANGSTROMS) OF 59-197, DISULFIDE BONDS.
    15. "Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred."
      Thomas A.Q., Lane K., Phillips J.A. III, Prince M., Markin C., Speer M., Schwartz D.A., Gaddipati R., Marney A., Johnson J., Roberts R., Haines J., Stahlman M., Loyd J.E.
      Am. J. Respir. Crit. Care Med. 165:1322-1328(2002) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SMDP2 GLN-188.
    16. "Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease."
      Tredano M., Griese M., Brasch F., Schumacher S., de Blic J., Marque S., Houdayer C., Elion J., Couderc R., Bahuau M.
      Am. J. Med. Genet. A 126:18-26(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANTS SMDP2 THR-73 AND GLN-167.
    17. "Surfactant protein C gene variation in the Finnish population -association with perinatal respiratory disease."
      Lahti M., Marttila R., Hallman M.
      Eur. J. Hum. Genet. 12:312-320(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANTS THR-138 AND SER-186, INVOLVEMENT IN RDS.
    18. "Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene."
      Brasch F., Griese M., Tredano M., Johnen G., Ochs M., Rieger C., Mulugeta S., Mueller K.M., Bahuau M., Beers M.F.
      Eur. Respir. J. 24:30-39(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SMDP2 THR-73, CHARACTERIZATION OF VARIANT SMDP2 THR-73.
    19. "Variable phenotype associated with SP-C gene mutations: fatal case with the I73T mutation."
      Percopo S., Cameron H.S., Nogee L.M., Pettinato G., Montella S., Santamaria F.
      Eur. Respir. J. 24:1072-1073(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SMDP2 THR-73.
    20. "Hydroxychloroquine and surfactant protein C deficiency."
      Rosen D.M., Waltz D.A.
      N. Engl. J. Med. 352:207-208(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SURFACTANT PROTEIN C DEFICIENCY ASP-116.
    21. "Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene."
      Stevens P.A., Pettenazzo A., Brasch F., Mulugeta S., Baritussio A., Ochs M., Morrison L., Russo S.J., Beers M.F.
      Pediatr. Res. 57:89-98(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SMDP2 LYS-66, CHARACTERIZATION OF VARIANT SMDP2 LYS-66.

    Entry informationi

    Entry nameiPSPC_HUMAN
    AccessioniPrimary (citable) accession number: P11686
    Secondary accession number(s): A6XNE4
    , B2RE00, E9PGX3, P11687, Q12793, Q7Z5D0
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: October 1, 1989
    Last sequence update: April 1, 1990
    Last modified: October 1, 2014
    This is version 156 of the entry and version 2 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 8
      Human chromosome 8: 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