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P11686

- PSPC_HUMAN

UniProt

P11686 - PSPC_HUMAN

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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

Functioni

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

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
Complete GO annotation...

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: Ensembl
  3. multivesicular body Source: Ensembl
Complete GO annotation...

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

BgeeiP11686.
CleanExiHS_SFTPC.
ExpressionAtlasiP11686. baseline and differential.
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 – 966Combined sources
Turni97 – 993Combined sources
Beta strandi100 – 1056Combined sources
Turni106 – 1094Combined sources
Beta strandi110 – 1167Combined sources
Beta strandi121 – 1255Combined sources
Helixi134 – 14310Combined sources
Helixi183 – 1897Combined sources
Beta strandi194 – 1974Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
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.
GeneTreeiENSGT00390000017162.
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

        10         20         30         40         50
MDVGSKEVLM ESPPDYSAAP RGRFGIPCCP VHLKRLLIVV VVVVLIVVVI
60 70 80 90 100
VGALLMGLHM SQKHTEMVLE MSIGAPEAQQ RLALSEHLVT TATFSIGSTG
110 120 130 140 150
LVVYDYQQLL IAYKPAPGTC CYIMKIAPES IPSLEALNRK VHNFQMECSL
160 170 180 190
QAKPAVPTSK LGQAEGRDAG SAPSGGDPAF LGMAVNTLCG EVPLYYI
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:
EMBLi
GenBanki
DDBJi
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:
EMBLi
GenBanki
DDBJi
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:
PDBei
RCSB PDBi
PDBji
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.
GeneTreei ENSGT00390000017162.
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

Bgeei P11686.
CleanExi HS_SFTPC.
ExpressionAtlasi P11686. baseline and differential.
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

« Hide 'large scale' publications
  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: November 26, 2014
This is version 158 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