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Entry version 190 (17 Jun 2020)
Sequence version 2 (01 Feb 1994)
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Protein

Properdin

Gene

CFP

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

A positive regulator of the alternate pathway (AP) of complement (PubMed:20382442, PubMed:28264884). It binds to and stabilizes the C3- and C5-convertase enzyme complexes (PubMed:20382442, PubMed:28264884). Inhibits CFI-CFH mediated degradation of Complement C3 beta chain (C3b) (PubMed:31507604).3 Publications

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Biological processComplement alternate pathway, Immunity, Innate immunity

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-173736 Alternative complement activation
R-HSA-174577 Activation of C3 and C5
R-HSA-5083635 Defective B3GALTL causes Peters-plus syndrome (PpS)
R-HSA-5173214 O-glycosylation of TSR domain-containing proteins
R-HSA-6798695 Neutrophil degranulation
R-HSA-977606 Regulation of Complement cascade

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Properdin
Alternative name(s):
Complement factor P
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:CFP
Synonyms:PFC
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome X

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000126759.12

Human Gene Nomenclature Database

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HGNCi
HGNC:8864 CFP

Online Mendelian Inheritance in Man (OMIM)

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MIMi
300383 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P27918

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Properdin deficiency (PFD)5 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionResults in higher susceptibility to bacterial infections; especially to meningococcal infections. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_08303832C → Y in PFD; type II; inhibits secretion and oligomerization. 1 Publication1
Natural variantiVAR_002002100R → W in PFD; type II. 1 PublicationCorresponds to variant dbSNP:rs132630259EnsemblClinVar.1
Natural variantiVAR_083039244E → K in PFD; type II; decreases expression, inhibits oligomerization and fails to stimulate bacteriolysis; does not affect binding to Complement C3 beta chain. 1 Publication1
Natural variantiVAR_013139298G → V in PFD; type I. 1 PublicationCorresponds to variant dbSNP:rs28935480EnsemblClinVar.1
Natural variantiVAR_002003343Q → R in PFD; type II; significantly decreases Complement C3 beta chain binding. 2 Publications1
Natural variantiVAR_002004414Y → D in PFD; type III; significantly decreases Complement C3 beta chain binding. 2 PublicationsCorresponds to variant dbSNP:rs132630261EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi47L → A: Inhibits oligomerization; when associated with A-58 and A-275. 1 Publication1
Mutagenesisi58L → A: Inhibits oligomerization; when associated with A-47 and A-275. 1 Publication1
Mutagenesisi244E → R: Inhibits oligomerization. 1 Publication1
Mutagenesisi275L → A: Inhibits oligomerization; when associated with A-47 and A-58. 1 Publication1
Mutagenesisi329R → A: Significantly decreases Complement C3 beta chain binding. 1 Publication1
Mutagenesisi330R → A: Slightly decreases Complement C3 beta chain binding. 1 Publication1
Mutagenesisi351R → A: Decreases Complement C3 beta chain binding. 1 Publication1
Mutagenesisi353R → A: Significantly decreases Complement C3 beta chain binding. 1 Publication1
Mutagenesisi359R → A: Significantly decreases Complement C3 beta chain binding. 1 Publication1
Mutagenesisi364 – 365QQ → AA: Decreases Complement C3 beta chain binding. 1 Publication2
Mutagenesisi456L → V: Inhibits oligomerization; when associated with A-47 and A-58. 1 Publication1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNET

More...
DisGeNETi
5199

MalaCards human disease database

More...
MalaCardsi
CFP
MIMi312060 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000126759

Orphanet; a database dedicated to information on rare diseases and orphan drugs

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Orphaneti
2966 Properdin deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA33206

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

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Pharosi
P27918 Tbio

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
CFP

Domain mapping of disease mutations (DMDM)

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DMDMi
464473

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section denotes the presence of an N-terminal signal peptide.<p><a href='/help/signal' target='_top'>More...</a></p>Signal peptidei1 – 27Add BLAST27
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000003586328 – 469ProperdinAdd BLAST442

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the PTM / Processing":/help/ptm_processing_section section describes the positions of cysteine residues participating in disulfide bonds.<p><a href='/help/disulfid' target='_top'>More...</a></p>Disulfide bondi32 ↔ 56PROSITE-ProRule annotation1 Publication
Disulfide bondi43 ↔ 72PROSITE-ProRule annotation1 Publication
Disulfide bondi57 ↔ 751 Publication
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm%5Fprocessing%5Fsection">PTM / Processing</a> section specifies the position and type of each covalently attached glycan group (mono-, di-, or polysaccharide).<p><a href='/help/carbohyd' target='_top'>More...</a></p>Glycosylationi83C-linked (Man) tryptophan2 Publications1
Glycosylationi86C-linked (Man) tryptophan2 Publications1
Disulfide bondi89 ↔ 127PROSITE-ProRule annotation1 Publication
Glycosylationi92O-linked (Fuc...) threonine2 Publications1
Disulfide bondi93 ↔ 133PROSITE-ProRule annotation1 Publication
Disulfide bondi104 ↔ 111PROSITE-ProRule annotation1 Publication
Disulfide bondi132 ↔ 1701 Publication
Glycosylationi139C-linked (Man) tryptophan2 Publications1
Glycosylationi142C-linked (Man) tryptophan2 Publications1
Glycosylationi145C-linked (Man) tryptophan2 Publications1
Disulfide bondi148 ↔ 184PROSITE-ProRule annotation1 Publication
Glycosylationi151O-linked (Fuc...) threonine2 Publications1
Disulfide bondi152 ↔ 190PROSITE-ProRule annotation1 Publication
Disulfide bondi163 ↔ 174PROSITE-ProRule annotation1 Publication
Glycosylationi196C-linked (Man) tryptophan2 Publications1
Glycosylationi199C-linked (Man) tryptophan2 Publications1
Glycosylationi202C-linked (Man) tryptophan1 Publication1
Disulfide bondi205 ↔ 248PROSITE-ProRule annotation1 Publication
Glycosylationi208O-linked (Fuc...) serine2 Publications1
Disulfide bondi209 ↔ 254PROSITE-ProRule annotation1 Publication
Disulfide bondi224 ↔ 238PROSITE-ProRule annotation1 Publication
Glycosylationi260C-linked (Man) tryptophan2 Publications1
Glycosylationi263C-linked (Man) tryptophan2 Publications1
Disulfide bondi269 ↔ 306PROSITE-ProRule annotation1 Publication
Glycosylationi272O-linked (Fuc...) threonine2 Publications1
Disulfide bondi273 ↔ 312PROSITE-ProRule annotation1 Publication
Disulfide bondi284 ↔ 296PROSITE-ProRule annotation1 Publication
Glycosylationi321C-linked (Man) tryptophan2 Publications1
Glycosylationi324C-linked (Man) tryptophan2 Publications1
Disulfide bondi327 ↔ 370PROSITE-ProRule annotation1 Publication
Disulfide bondi337 ↔ 376PROSITE-ProRule annotation1 Publication
Disulfide bondi350 ↔ 360PROSITE-ProRule annotation1 Publication
Glycosylationi382C-linked (Man) tryptophan2 Publications1
Glycosylationi385C-linked (Man) tryptophan2 Publications1
Glycosylationi388C-linked (Man) tryptophan2 Publications1
Disulfide bondi391 ↔ 455PROSITE-ProRule annotation1 Publication
Disulfide bondi395 ↔ 461PROSITE-ProRule annotation1 Publication
Disulfide bondi407 ↔ 439PROSITE-ProRule annotation1 Publication
Glycosylationi428N-linked (GlcNAc...) (complex) asparagine2 Publications1

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
P27918

MassIVE - Mass Spectrometry Interactive Virtual Environment

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MassIVEi
P27918

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
P27918

PeptideAtlas

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PeptideAtlasi
P27918

PRoteomics IDEntifications database

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PRIDEi
P27918

ProteomicsDB: a multi-organism proteome resource

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ProteomicsDBi
54426

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
P27918

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...
PhosphoSitePlusi
P27918

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000126759 Expressed in leukocyte and 114 other tissues

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P27918 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P27918 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
ENSG00000126759 Tissue enhanced (blood, bone marrow, lymphoid tissue)

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

In plasma, properdin exists as dimers, trimers or tetramers in the relative proportions of 26:54:20 (PubMed:20382442, PubMed:15491616, PubMed:28264884, PubMed:31507604).

Interacts with the pro-C3-convertase enzyme complex (C3b-Bb) comprised of Complement C3 beta chain (C3b) and the Complement factor B Bb fragment (Bb), where it binds (via its TSP type-1 5 domain) with C3b and Bb (PubMed:28264884, PubMed:31507604). This interaction stabilizes the complex and allows it to become the active C3-convertase enzyme complex (C3b-Bb-FP) (PubMed:28264884, PubMed:31507604).

Interacts with C3b (PubMed:28264884, PubMed:31507604).

Interacts with CFB (PubMed:31507604).

4 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Hide details
P27918
With#Exp.IntAct
ARMS2 [P0C7Q2]8EBI-9038570,EBI-21986906
BANF2 - isoform 2 [Q9H503-2]3EBI-9038570,EBI-11977289
CFAP206 [Q8IYR0]3EBI-9038570,EBI-749051
CFHR4 - isoform 1 [Q92496-1]2EBI-9038570,EBI-22033617
CFHR4 - isoform 3 [Q92496-3]2EBI-9038570,EBI-22033638
C-reactive protein (PRO_0000023526)2EBI-9038570,EBI-22033103
CYSRT1 [A8MQ03]3EBI-9038570,EBI-3867333
FHL5 [Q5TD97]3EBI-9038570,EBI-750641
FKBP6 [O75344]4EBI-9038570,EBI-744771
GEMIN4 [P57678]3EBI-9038570,EBI-356700
GNE - isoform 2 [Q9Y223-2]3EBI-9038570,EBI-11975289
HEXIM2 [Q96MH2]3EBI-9038570,EBI-5460660
HOXA1 [P49639]3EBI-9038570,EBI-740785
KCTD9 [Q7L273]3EBI-9038570,EBI-4397613
KLHL38 [Q2WGJ6]3EBI-9038570,EBI-6426443
KRTAP11-1 [Q8IUC1]3EBI-9038570,EBI-1052037
KRTAP12-2 [P59991]3EBI-9038570,EBI-10176379
KRTAP3-1 [Q9BYR8]3EBI-9038570,EBI-9996449
KRTAP6-2 [Q3LI66]5EBI-9038570,EBI-11962084
LCE1A [Q5T7P2]3EBI-9038570,EBI-11962058
LONRF1 [Q17RB8]3EBI-9038570,EBI-2341787
MID2 - isoform 2 [Q9UJV3-2]3EBI-9038570,EBI-10172526
MPO [P05164]4EBI-9038570,EBI-2556173
NEK6 - isoform 4 [Q9HC98-4]3EBI-9038570,EBI-11750983
NOXA1 - isoform 2 [Q86UR1-2]3EBI-9038570,EBI-12025760
PRKAB2 [O43741]3EBI-9038570,EBI-1053424
RECK [Q6P9E2]3EBI-9038570,EBI-10253121
SMARCC1 [Q92922]3EBI-9038570,EBI-355653
SPAG8 - isoform 2 [Q99932-2]4EBI-9038570,EBI-11959123
STK16 [O75716]3EBI-9038570,EBI-749295
VENTX [O95231]5EBI-9038570,EBI-10191303
ZNF330 [Q9Y3S2]3EBI-9038570,EBI-373456
ZNF414 [Q96IQ9]3EBI-9038570,EBI-744257
Ncr1 [Q8C567] from Mus musculus.2EBI-9038570,EBI-11707971
Properdin (PRO_0000035863)
With#Exp.IntAct
NCR1 [O76036]5EBI-15183949,EBI-13915737
Ncr1 [Q8C567] from Mus musculus.3EBI-15183949,EBI-11707971

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

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BioGRIDi
111222, 10 interactors

ComplexPortal: manually curated resource of macromolecular complexes

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ComplexPortali
CPX-5602 Alternative pathway pathogen cell-bound C3 convertase complex C3bBbP
CPX-5605 Alternative pathway pathogen cell-bound C5 convertase complex C3bBbC3bP

Protein interaction database and analysis system

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IntActi
P27918, 49 interactors

STRING: functional protein association networks

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STRINGi
9606.ENSP00000247153

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...
RNActi
P27918 protein

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1469
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P27918

Database of comparative protein structure models

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ModBasei
Search...

Protein Data Bank in Europe - Knowledge Base

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PDBe-KBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...
EvolutionaryTracei
P27918

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family%5Fand%5Fdomains%5Fsection">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini28 – 76TSP type-1 0PROSITE-ProRule annotationAdd BLAST49
Domaini77 – 134TSP type-1 1PROSITE-ProRule annotationAdd BLAST58
Domaini136 – 191TSP type-1 2PROSITE-ProRule annotationAdd BLAST56
Domaini193 – 255TSP type-1 3PROSITE-ProRule annotationAdd BLAST63
Domaini257 – 313TSP type-1 4PROSITE-ProRule annotationAdd BLAST57
Domaini315 – 377TSP type-1 5PROSITE-ProRule annotationAdd BLAST63
Domaini379 – 462TSP type-1 6PROSITE-ProRule annotationAdd BLAST84

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni351 – 359Interaction with Compliment C3 beta chain2 Publications9

<p>This subsection of the 'Family and domains' section provides general information on the biological role of a domain. The term 'domain' is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

TSP type-1 domain 0 binds to TSP type-1 domain 4, and TSP type-1 domain 1 binds to TSP type-1 domain 6 (PubMed:15491616, PubMed:28264884, PubMed:31507604). These interactions mediate multimerization (PubMed:15491616, PubMed:28264884, PubMed:31507604).3 Publications

Keywords - Domaini

Repeat, Signal

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
ENOG410IUGK Eukaryota
ENOG410ZHSM LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000161209

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P27918

KEGG Orthology (KO)

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KOi
K15412

Identification of Orthologs from Complete Genome Data

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OMAi
KPCPGLA

Database of Orthologous Groups

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OrthoDBi
304371at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
P27918

TreeFam database of animal gene trees

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TreeFami
TF315491

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
2.20.100.10, 6 hits

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR000884 TSP1_rpt
IPR036383 TSP1_rpt_sf

Pfam protein domain database

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Pfami
View protein in Pfam
PF00090 TSP_1, 6 hits

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00209 TSP1, 6 hits

Superfamily database of structural and functional annotation

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SUPFAMi
SSF82895 SSF82895, 6 hits

PROSITE; a protein domain and family database

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PROSITEi
View protein in PROSITE
PS50092 TSP1, 6 hits

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All

P27918-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MITEGAQAPR LLLPPLLLLL TLPATGSDPV LCFTQYEESS GKCKGLLGGG
60 70 80 90 100
VSVEDCCLNT AFAYQKRSGG LCQPCRSPRW SLWSTWAPCS VTCSEGSQLR
110 120 130 140 150
YRRCVGWNGQ CSGKVAPGTL EWQLQACEDQ QCCPEMGGWS GWGPWEPCSV
160 170 180 190 200
TCSKGTRTRR RACNHPAPKC GGHCPGQAQE SEACDTQQVC PTHGAWATWG
210 220 230 240 250
PWTPCSASCH GGPHEPKETR SRKCSAPEPS QKPPGKPCPG LAYEQRRCTG
260 270 280 290 300
LPPCPVAGGW GPWGPVSPCP VTCGLGQTME QRTCNHPVPQ HGGPFCAGDA
310 320 330 340 350
TRTHICNTAV PCPVDGEWDS WGEWSPCIRR NMKSISCQEI PGQQSRGRTC
360 370 380 390 400
RGRKFDGHRC AGQQQDIRHC YSIQHCPLKG SWSEWSTWGL CMPPCGPNPT
410 420 430 440 450
RARQRLCTPL LPKYPPTVSM VEGQGEKNVT FWGRPLPRCE ELQGQKLVVE
460
EKRPCLHVPA CKDPEEEEL
Length:469
Mass (Da):51,276
Last modified:February 1, 1994 - v2
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i5EB42B63F0283917
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E9PAQ1E9PAQ1_HUMAN
Properdin
CFP
415Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9J7V5C9J7V5_HUMAN
Properdin
CFP
167Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0358133T → I in a breast cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_08303832C → Y in PFD; type II; inhibits secretion and oligomerization. 1 Publication1
Natural variantiVAR_02039553V → M1 PublicationCorresponds to variant dbSNP:rs8177068Ensembl.1
Natural variantiVAR_002002100R → W in PFD; type II. 1 PublicationCorresponds to variant dbSNP:rs132630259EnsemblClinVar.1
Natural variantiVAR_020396204P → L1 PublicationCorresponds to variant dbSNP:rs8177076Ensembl.1
Natural variantiVAR_083039244E → K in PFD; type II; decreases expression, inhibits oligomerization and fails to stimulate bacteriolysis; does not affect binding to Complement C3 beta chain. 1 Publication1
Natural variantiVAR_020397250G → S1 PublicationCorresponds to variant dbSNP:rs8177077Ensembl.1
Natural variantiVAR_013139298G → V in PFD; type I. 1 PublicationCorresponds to variant dbSNP:rs28935480EnsemblClinVar.1
Natural variantiVAR_002003343Q → R in PFD; type II; significantly decreases Complement C3 beta chain binding. 2 Publications1
Natural variantiVAR_002004414Y → D in PFD; type III; significantly decreases Complement C3 beta chain binding. 2 PublicationsCorresponds to variant dbSNP:rs132630261EnsemblClinVar.1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...
EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
X57748 mRNA Translation: CAA40914.1
X70872 Genomic DNA Translation: CAA50220.1
M83652 mRNA Translation: AAA36489.1
AF005664 Genomic DNA Translation: AAB63279.1
AF005665 Genomic DNA Translation: AAB63280.1
AF005666 Genomic DNA Translation: AAC51626.1
AF005668, AF005667 Genomic DNA Translation: AAB62886.1
AY297813 Genomic DNA Translation: AAP43692.1
AL009172 Genomic DNA No translation available.
BC015756 mRNA Translation: AAH15756.1

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS14282.1

Protein sequence database of the Protein Information Resource

More...
PIRi
S29126

NCBI Reference Sequences

More...
RefSeqi
NP_001138724.1, NM_001145252.1
NP_002612.1, NM_002621.2

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000247153; ENSP00000247153; ENSG00000126759
ENST00000396992; ENSP00000380189; ENSG00000126759

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
5199

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:5199

UCSC genome browser

More...
UCSCi
uc004dig.5 human

Keywords - Coding sequence diversityi

Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross%5Freferences%5Fsection">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

CFPbase

CFP mutation db

SeattleSNPs
Wikipedia

Properdin entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X57748 mRNA Translation: CAA40914.1
X70872 Genomic DNA Translation: CAA50220.1
M83652 mRNA Translation: AAA36489.1
AF005664 Genomic DNA Translation: AAB63279.1
AF005665 Genomic DNA Translation: AAB63280.1
AF005666 Genomic DNA Translation: AAC51626.1
AF005668, AF005667 Genomic DNA Translation: AAB62886.1
AY297813 Genomic DNA Translation: AAP43692.1
AL009172 Genomic DNA No translation available.
BC015756 mRNA Translation: AAH15756.1
CCDSiCCDS14282.1
PIRiS29126
RefSeqiNP_001138724.1, NM_001145252.1
NP_002612.1, NM_002621.2

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...
PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1W0RX-ray-A/B28-469[»]
1W0SX-ray-A/B/C28-469[»]
6RURX-ray6.0A28-255[»]
B256-469[»]
6RUSX-ray2.8A28-255[»]
B256-469[»]
6RUVX-ray6.15U/X28-191[»]
V/Y256-469[»]
6RV6X-ray3.51A28-191[»]
B256-469[»]
6S08X-ray2.03A256-469[»]
B26-132[»]
6S0AX-ray2.52A256-469[»]
B26-191[»]
6S0BX-ray2.31A256-469[»]
B26-132[»]
6SEJX-ray3.50A28-191[»]
B256-469[»]
SMRiP27918
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi111222, 10 interactors
ComplexPortaliCPX-5602 Alternative pathway pathogen cell-bound C3 convertase complex C3bBbP
CPX-5605 Alternative pathway pathogen cell-bound C5 convertase complex C3bBbC3bP
IntActiP27918, 49 interactors
STRINGi9606.ENSP00000247153

PTM databases

iPTMnetiP27918
PhosphoSitePlusiP27918

Polymorphism and mutation databases

BioMutaiCFP
DMDMi464473

Proteomic databases

jPOSTiP27918
MassIVEiP27918
PaxDbiP27918
PeptideAtlasiP27918
PRIDEiP27918
ProteomicsDBi54426

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...
Antibodypediai
11427 507 antibodies

Genome annotation databases

EnsembliENST00000247153; ENSP00000247153; ENSG00000126759
ENST00000396992; ENSP00000380189; ENSG00000126759
GeneIDi5199
KEGGihsa:5199
UCSCiuc004dig.5 human

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
5199
DisGeNETi5199
EuPathDBiHostDB:ENSG00000126759.12

GeneCards: human genes, protein and diseases

More...
GeneCardsi
CFP
HGNCiHGNC:8864 CFP
HPAiENSG00000126759 Tissue enhanced (blood, bone marrow, lymphoid tissue)
MalaCardsiCFP
MIMi300383 gene
312060 phenotype
neXtProtiNX_P27918
OpenTargetsiENSG00000126759
Orphaneti2966 Properdin deficiency
PharmGKBiPA33206

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiENOG410IUGK Eukaryota
ENOG410ZHSM LUCA
GeneTreeiENSGT00940000161209
InParanoidiP27918
KOiK15412
OMAiKPCPGLA
OrthoDBi304371at2759
PhylomeDBiP27918
TreeFamiTF315491

Enzyme and pathway databases

ReactomeiR-HSA-173736 Alternative complement activation
R-HSA-174577 Activation of C3 and C5
R-HSA-5083635 Defective B3GALTL causes Peters-plus syndrome (PpS)
R-HSA-5173214 O-glycosylation of TSR domain-containing proteins
R-HSA-6798695 Neutrophil degranulation
R-HSA-977606 Regulation of Complement cascade

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...
BioGRID-ORCSi
5199 1 hit in 412 CRISPR screens

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
CFP human
EvolutionaryTraceiP27918

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
5199
PharosiP27918 Tbio

Protein Ontology

More...
PROi
PR:P27918
RNActiP27918 protein

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000126759 Expressed in leukocyte and 114 other tissues
ExpressionAtlasiP27918 baseline and differential
GenevisibleiP27918 HS

Family and domain databases

Gene3Di2.20.100.10, 6 hits
InterProiView protein in InterPro
IPR000884 TSP1_rpt
IPR036383 TSP1_rpt_sf
PfamiView protein in Pfam
PF00090 TSP_1, 6 hits
SMARTiView protein in SMART
SM00209 TSP1, 6 hits
SUPFAMiSSF82895 SSF82895, 6 hits
PROSITEiView protein in PROSITE
PS50092 TSP1, 6 hits

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiPROP_HUMAN
<p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P27918
Secondary accession number(s): O15134
, O15135, O15136, O75826
<p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1992
Last sequence update: February 1, 1994
Last modified: June 17, 2020
This is version 190 of the entry and version 2 of the sequence. See complete history.
<p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Reference proteome

Documents

  1. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  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. Human chromosome X
    Human chromosome X: entries, gene names and cross-references to MIM
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