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Protein

Coagulation factor XI

Gene

F11

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Factor XI triggers the middle phase of the intrinsic pathway of blood coagulation by activating factor IX.

Catalytic activityi

Selective cleavage of Arg-|-Ala and Arg-|-Val bonds in factor IX to form factor IXa.

Enzyme regulationi

Inhibited by SERPINA5.1 Publication

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Active sitei431 – 4311Charge relay system
Active sitei480 – 4801Charge relay system
Active sitei575 – 5751Charge relay system

GO - Molecular functioni

  • heparin binding Source: UniProtKB-KW
  • serine-type aminopeptidase activity Source: Ensembl
  • serine-type endopeptidase activity Source: UniProtKB

GO - Biological processi

  • blood coagulation Source: UniProtKB
  • blood coagulation, intrinsic pathway Source: Reactome
  • plasminogen activation Source: BHF-UCL
  • positive regulation of fibrinolysis Source: BHF-UCL
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Protease, Serine protease

Keywords - Biological processi

Blood coagulation, Hemostasis

Keywords - Ligandi

Heparin-binding

Enzyme and pathway databases

BRENDAi3.4.21.27. 2681.
ReactomeiR-HSA-140837. Intrinsic Pathway of Fibrin Clot Formation.
SABIO-RKP03951.

Protein family/group databases

MEROPSiS01.213.

Names & Taxonomyi

Protein namesi
Recommended name:
Coagulation factor XI (EC:3.4.21.27)
Short name:
FXI
Alternative name(s):
Plasma thromboplastin antecedent
Short name:
PTA
Cleaved into the following 2 chains:
Gene namesi
Name:F11
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:3529. F11.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • extracellular region Source: UniProtKB
  • extracellular space Source: BHF-UCL
  • membrane Source: UniProtKB
  • plasma membrane Source: Reactome
Complete GO annotation...

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Involvement in diseasei

Factor XI deficiency (FA11D)21 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA hemorrhagic disease characterized by reduced levels and activity of factor XI resulting in moderate bleeding symptoms, usually occurring after trauma or surgery. Patients usually do not present spontaneous bleeding but women can present with menorrhagia. Hemorrhages are usually moderate.
See also OMIM:612416
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti30 – 301F → S in FA11D. 1 Publication
VAR_076515
Natural varianti32 – 321G → R in FA11D. 1 Publication
Corresponds to variant rs281875259 [ dbSNP | Ensembl ].
VAR_067929
Natural varianti34 – 341D → H in FA11D. 1 Publication
Corresponds to variant rs281875267 [ dbSNP | Ensembl ].
VAR_012085
Natural varianti43 – 431A → T in FA11D; dominant-negative mutation that results in severely decreased protein secretion. 1 Publication
Corresponds to variant rs281875264 [ dbSNP | Ensembl ].
VAR_067930
Natural varianti46 – 461C → F in FA11D. 1 Publication
Corresponds to variant rs281875271 [ dbSNP | Ensembl ].
VAR_054894
Natural varianti51 – 511T → I in FA11D. 1 Publication
Corresponds to variant rs281875252 [ dbSNP | Ensembl ].
VAR_067931
Natural varianti51 – 511T → P in FA11D. 2 Publications
Corresponds to variant rs281875243 [ dbSNP | Ensembl ].
VAR_067932
Natural varianti53 – 531H → Q in FA11D. 1 Publication
Corresponds to variant rs281875261 [ dbSNP | Ensembl ].
VAR_067933
Natural varianti56 – 561C → R in FA11D; secretion of the mutant protein is impaired. 2 Publications
Corresponds to variant rs121965069 [ dbSNP | Ensembl ].
VAR_054895
Natural varianti63 – 631A → V in FA11D. 1 Publication
Corresponds to variant rs281875244 [ dbSNP | Ensembl ].
VAR_067934
Natural varianti101 – 1011K → R in FA11D. 1 Publication
Corresponds to variant rs281875272 [ dbSNP | Ensembl ].
VAR_054896
Natural varianti109 – 1091A → T in FA11D. 1 Publication
VAR_076516
Natural varianti140 – 1401C → Y in FA11D. 1 Publication
Corresponds to variant rs281875256 [ dbSNP | Ensembl ].
VAR_067935
Natural varianti151 – 1511Y → C in FA11D. 1 Publication
Corresponds to variant rs281875273 [ dbSNP | Ensembl ].
VAR_054897
Natural varianti216 – 2161D → N in FA11D. 1 Publication
VAR_076517
Natural varianti222 – 2221D → Y in FA11D. 1 Publication
Corresponds to variant rs281875245 [ dbSNP | Ensembl ].
VAR_067936
Natural varianti228 – 2281R → Q in FA11D. 1 Publication
Corresponds to variant rs281875246 [ dbSNP | Ensembl ].
VAR_067937
Natural varianti241 – 2411F → L in FA11D; dominant-negative mutation that results in severely decreased protein secretion. 1 Publication
Corresponds to variant rs281875265 [ dbSNP | Ensembl ].
VAR_067938
Natural varianti246 – 2461W → C in FA11D. 1 Publication
Corresponds to variant rs281875279 [ dbSNP | Ensembl ].
VAR_012086
Natural varianti252 – 2521R → T in FA11D. 1 Publication
Corresponds to variant rs281875260 [ dbSNP | Ensembl ].
VAR_067939
Natural varianti255 – 2551C → Y in FA11D; secretion of the mutant protein is impaired. 1 Publication
Corresponds to variant rs281875277 [ dbSNP | Ensembl ].
VAR_054898
Natural varianti263 – 2631G → E in FA11D. 1 Publication
Corresponds to variant rs281875274 [ dbSNP | Ensembl ].
VAR_054899
Natural varianti266 – 2661S → N in FA11D. 1 Publication
Corresponds to variant rs145168351 [ dbSNP | Ensembl ].
VAR_012087
Natural varianti270 – 2701K → I in FA11D; although the mutant protein is synthesized the secretion is reduced. 1 Publication
Corresponds to variant rs121965070 [ dbSNP | Ensembl ].
VAR_054900
Natural varianti276 – 2761S → C in FA11D. 1 Publication
Corresponds to variant rs281875247 [ dbSNP | Ensembl ].
VAR_067940
Natural varianti277 – 2771G → D in FA11D. 1 Publication
Corresponds to variant rs281875248 [ dbSNP | Ensembl ].
VAR_067941
Natural varianti301 – 3011F → L in FA11D. 2 Publications
Corresponds to variant rs121965064 [ dbSNP | Ensembl ].
VAR_006622
Natural varianti315 – 3151E → K in FA11D. 2 Publications
Corresponds to variant rs281875257 [ dbSNP | Ensembl ].
VAR_067942
Natural varianti320 – 3201L → P in FA11D. 1 Publication
Corresponds to variant rs281875268 [ dbSNP | Ensembl ].
VAR_012088
Natural varianti322 – 3221T → I in FA11D. 1 Publication
Corresponds to variant rs281875269 [ dbSNP | Ensembl ].
VAR_012089
Natural varianti326 – 3261R → C in FA11D. 1 Publication
Corresponds to variant rs28934608 [ dbSNP | Ensembl ].
VAR_012090
Natural varianti331 – 3311T → I in FA11D. 1 Publication
Corresponds to variant rs281875253 [ dbSNP | Ensembl ].
VAR_067943
Natural varianti341 – 3411E → K in FA11D. 1 Publication
Corresponds to variant rs281875270 [ dbSNP | Ensembl ].
VAR_012091
Natural varianti360 – 3601L → P in FA11D. 1 Publication
Corresponds to variant rs281875254 [ dbSNP | Ensembl ].
VAR_067944
Natural varianti401 – 4011W → R in FA11D. 1 Publication
Corresponds to variant rs281875262 [ dbSNP | Ensembl ].
VAR_067945
Natural varianti403 – 4031V → M in FA11D; dominant-negative mutation that results in severely decreased protein secretion. 1 Publication
Corresponds to variant rs281875266 [ dbSNP | Ensembl ].
VAR_067946
Natural varianti404 – 4041T → N in FA11D. 1 Publication
Corresponds to variant rs121965067 [ dbSNP | Ensembl ].
VAR_012092
Natural varianti418 – 4181G → V in FA11D; mutant is not secreted by transfected fibroblasts; dominant-negative effect. 1 Publication
Corresponds to variant rs121965071 [ dbSNP | Ensembl ].
VAR_054901
Natural varianti430 – 4301A → V in FA11D. 1 Publication
Corresponds to variant rs121965068 [ dbSNP | Ensembl ].
VAR_012093
Natural varianti454 – 4541I → K in FA11D. 1 Publication
Corresponds to variant rs281875241 [ dbSNP | Ensembl ].
VAR_067947
Natural varianti460 – 4601F → V in FA11D. 1 Publication
Corresponds to variant rs121965065 [ dbSNP | Ensembl ].
VAR_012094
Natural varianti481 – 4811I → S in FA11D. 1 Publication
Corresponds to variant rs281875242 [ dbSNP | Ensembl ].
VAR_067948
Natural varianti493 – 4931T → I in FA11D. 1 Publication
VAR_012095
Natural varianti503 – 5031S → P in FA11D. 1 Publication
Corresponds to variant rs140068026 [ dbSNP | Ensembl ].
VAR_067949
Natural varianti506 – 5061D → G in FA11D; mild phenotype. 1 Publication
Corresponds to variant rs281875258 [ dbSNP | Ensembl ].
VAR_067950
Natural varianti511 – 5111Y → H in FA11D; transfected cells contain reduced amount of mutant protein and display decreased secretion. 1 Publication
Corresponds to variant rs281875278 [ dbSNP | Ensembl ].
VAR_054902
Natural varianti514 – 5141C → F in FA11D. 1 Publication
Corresponds to variant rs281875249 [ dbSNP | Ensembl ].
VAR_067951
Natural varianti526 – 5261D → E in FA11D. 1 Publication
Corresponds to variant rs281875263 [ dbSNP | Ensembl ].
VAR_067952
Natural varianti538 – 5381P → L in FA11D. 1 Publication
Corresponds to variant rs139695003 [ dbSNP | Ensembl ].
VAR_054903
Natural varianti543 – 5431E → K in FA11D. 1 Publication
VAR_076518
Natural varianti552 – 5521H → R in FA11D. 1 Publication
VAR_076519
Natural varianti565 – 5651E → K in FA11D. 1 Publication
Corresponds to variant rs281875275 [ dbSNP | Ensembl ].
VAR_054904
Natural varianti575 – 5751S → L in FA11D. 1 Publication
Corresponds to variant rs281875250 [ dbSNP | Ensembl ].
VAR_067953
Natural varianti587 – 5871W → S in FA11D; mutant is not secreted by transfected fibroblasts; dominant-negative effect. 1 Publication
Corresponds to variant rs121965072 [ dbSNP | Ensembl ].
VAR_054905
Natural varianti594 – 5941S → R in FA11D. 1 Publication
Corresponds to variant rs28934609 [ dbSNP | Ensembl ].
VAR_012096
Natural varianti597 – 5971E → K in FA11D. 2 Publications
Corresponds to variant rs281875251 [ dbSNP | Ensembl ].
VAR_067954
Natural varianti608 – 6081Y → H in FA11D. 1 Publication
Corresponds to variant rs281875255 [ dbSNP | Ensembl ].
VAR_067955
Natural varianti618 – 6181I → S in FA11D. 1 Publication
Corresponds to variant rs281875276 [ dbSNP | Ensembl ].
VAR_054906

Keywords - Diseasei

Disease mutation

Organism-specific databases

MalaCardsiF11.
MIMi612416. phenotype.
Orphaneti329. Congenital factor XI deficiency.
PharmGKBiPA27941.

Chemistry

ChEMBLiCHEMBL2820.
DrugBankiDB00100. Coagulation Factor IX.
GuidetoPHARMACOLOGYi2360.

Polymorphism and mutation databases

BioMutaiF11.
DMDMi119762.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 1818Add
BLAST
Chaini19 – 387369Coagulation factor XIa heavy chainPRO_0000027825Add
BLAST
Chaini388 – 625238Coagulation factor XIa light chainPRO_0000027826Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi20 ↔ 103Sequence analysis
Disulfide bondi29 – 29Interchain
Disulfide bondi46 ↔ 761 Publication
Disulfide bondi50 ↔ 561 Publication
Glycosylationi90 – 901N-linked (GlcNAc...) (complex)1 Publication
Disulfide bondi110 ↔ 1931 Publication
Glycosylationi126 – 1261N-linked (GlcNAc...) (complex)2 Publications
Disulfide bondi136 ↔ 1651 Publication
Disulfide bondi140 ↔ 1461 Publication
Glycosylationi163 – 1631N-linked (GlcNAc...) (complex); atypical1 Publication
Disulfide bondi200 ↔ 283
Disulfide bondi226 ↔ 2551 Publication
Disulfide bondi230 ↔ 2361 Publication
Disulfide bondi291 ↔ 3741 Publication
Disulfide bondi317 ↔ 3461 Publication
Disulfide bondi321 ↔ 3271 Publication
Disulfide bondi339 – 339InterchainSequence analysis
Glycosylationi353 – 3531N-linked (GlcNAc...)
Disulfide bondi380 ↔ 500Interchain (between heavy and light chains)
Disulfide bondi416 ↔ 4321 Publication
Glycosylationi450 – 4501N-linked (GlcNAc...) (complex)3 Publications
Glycosylationi491 – 4911N-linked (GlcNAc...) (complex)4 Publications
Disulfide bondi514 ↔ 581
Disulfide bondi545 ↔ 5601 Publication
Disulfide bondi571 ↔ 599

Post-translational modificationi

N-glycosylated on both chains. N-glycosylated sites mainly consist of nonfucosylated sialylated biantennary (in high abundance) and/or triantennary (in low abundance) complex structures. Glycosylation at Asn-163 uses a rare non-canonical Asn-X-Cys glycosite.1 Publication
Activated by factor XIIa (or XII), which cleaves each polypeptide after Arg-387 into the light chain, which contains the active site, and the heavy chain, which associates with high molecular weight (HMW) kininogen.

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiP03951.
PeptideAtlasiP03951.
PRIDEiP03951.

PTM databases

iPTMnetiP03951.
PhosphoSiteiP03951.

Miscellaneous databases

PMAP-CutDBP03951.

Expressioni

Tissue specificityi

Isoform 2 is produced by platelets and megakaryocytes but absent from other blood cells.

Gene expression databases

BgeeiENSG00000088926.
CleanExiHS_F11.
ExpressionAtlasiP03951. baseline and differential.
GenevisibleiP03951. HS.

Interactioni

Subunit structurei

Homodimer; disulfide-linked. Forms a heterodimer with SERPINA5. After activation the heavy and light chains are also linked by a disulfide bond.2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
ecoP238273EBI-1041019,EBI-1029159From a different organism.

Protein-protein interaction databases

BioGridi108458. 6 interactions.
DIPiDIP-29085N.
IntActiP03951. 1 interaction.
STRINGi9606.ENSP00000384957.

Chemistry

BindingDBiP03951.

Structurei

Secondary structure

1
625
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Beta strandi25 – 306Combined sources
Beta strandi34 – 396Combined sources
Helixi43 – 5210Combined sources
Beta strandi53 – 553Combined sources
Beta strandi58 – 625Combined sources
Helixi70 – 723Combined sources
Beta strandi75 – 795Combined sources
Beta strandi82 – 843Combined sources
Beta strandi88 – 9811Combined sources
Helixi100 – 1023Combined sources
Beta strandi114 – 12916Combined sources
Helixi133 – 14210Combined sources
Beta strandi143 – 1453Combined sources
Beta strandi147 – 1526Combined sources
Turni159 – 1635Combined sources
Beta strandi164 – 1696Combined sources
Beta strandi171 – 1744Combined sources
Beta strandi176 – 18813Combined sources
Helixi190 – 1923Combined sources
Turni193 – 1953Combined sources
Beta strandi205 – 21915Combined sources
Helixi223 – 2319Combined sources
Beta strandi237 – 2426Combined sources
Helixi249 – 2513Combined sources
Beta strandi254 – 2596Combined sources
Beta strandi261 – 2644Combined sources
Beta strandi266 – 27813Combined sources
Helixi281 – 2833Combined sources
Beta strandi294 – 2963Combined sources
Beta strandi299 – 3024Combined sources
Beta strandi304 – 3129Combined sources
Helixi314 – 3229Combined sources
Beta strandi329 – 3335Combined sources
Turni337 – 3393Combined sources
Beta strandi344 – 3507Combined sources
Beta strandi352 – 3554Combined sources
Beta strandi357 – 3648Combined sources
Beta strandi366 – 3694Combined sources
Helixi372 – 3765Combined sources
Helixi379 – 3813Combined sources
Beta strandi390 – 3934Combined sources
Beta strandi402 – 4076Combined sources
Beta strandi409 – 4113Combined sources
Beta strandi413 – 42210Combined sources
Beta strandi425 – 4284Combined sources
Helixi430 – 4334Combined sources
Helixi439 – 4413Combined sources
Beta strandi442 – 4454Combined sources
Helixi451 – 4533Combined sources
Beta strandi456 – 4583Combined sources
Beta strandi461 – 4688Combined sources
Helixi475 – 4773Combined sources
Beta strandi482 – 4887Combined sources
Beta strandi494 – 4963Combined sources
Helixi504 – 5063Combined sources
Beta strandi507 – 5093Combined sources
Beta strandi514 – 5196Combined sources
Beta strandi522 – 5254Combined sources
Beta strandi533 – 5364Combined sources
Helixi542 – 5487Combined sources
Turni549 – 5513Combined sources
Beta strandi558 – 5614Combined sources
Beta strandi578 – 5836Combined sources
Beta strandi586 – 59510Combined sources
Beta strandi597 – 6004Combined sources
Beta strandi606 – 6105Combined sources
Helixi611 – 6144Combined sources
Helixi615 – 6228Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1XX9X-ray2.20A/B388-625[»]
1XXDX-ray2.91A/B388-625[»]
1XXFX-ray2.60A/B388-625[»]
1ZHMX-ray1.96A388-624[»]
1ZHPX-ray2.70A388-625[»]
1ZHRX-ray1.73A388-625[»]
1ZJDX-ray2.60A388-624[»]
1ZLRX-ray2.50A388-624[»]
1ZMJX-ray2.00A388-624[»]
1ZMLX-ray2.25A388-625[»]
1ZMNX-ray2.05A388-625[»]
1ZOMX-ray2.25A388-624[»]
1ZPBX-ray2.10A388-624[»]
1ZPCX-ray2.60A388-624[»]
1ZPZX-ray2.50A388-625[»]
1ZRKX-ray2.30A388-625[»]
1ZSJX-ray1.90A388-625[»]
1ZSKX-ray1.90A388-625[»]
1ZSLX-ray2.05A388-625[»]
1ZTJX-ray2.05A388-624[»]
1ZTKX-ray2.50A388-624[»]
1ZTLX-ray2.60A388-624[»]
2F83X-ray2.87A1-625[»]
2FDAX-ray2.00A388-625[»]
2J8JNMR-A/B290-379[»]
2J8LNMR-A/B290-379[»]
3BG8X-ray1.60A388-625[»]
3SORX-ray1.80A388-625[»]
3SOSX-ray2.58A388-625[»]
4CR5X-ray2.00A388-625[»]
4CR9X-ray1.70A388-625[»]
4CRAX-ray1.80A388-625[»]
4CRBX-ray1.85A388-625[»]
4CRCX-ray1.60A388-625[»]
4CRDX-ray2.10A388-625[»]
4CREX-ray1.73A388-625[»]
4CRFX-ray2.30A388-625[»]
4CRGX-ray1.25A388-625[»]
4D76X-ray1.77A388-625[»]
4D7FX-ray1.62A388-625[»]
4D7GX-ray2.33A388-625[»]
4NA7X-ray2.80A388-625[»]
4NA8X-ray2.30A388-625[»]
4TY6X-ray1.85A388-625[»]
H375-387[»]
4TY7X-ray2.09A388-625[»]
4WXIX-ray2.60A388-625[»]
4X6MX-ray2.40A388-625[»]
4X6NX-ray2.10A388-625[»]
H375-387[»]
4X6OX-ray2.10A388-625[»]
4X6PX-ray1.93A/B388-625[»]
4Y8XX-ray1.90A388-625[»]
4Y8YX-ray2.60A388-625[»]
4Y8ZX-ray2.20A388-625[»]
5E2OX-ray2.08A388-625[»]
5E2PX-ray2.11A388-625[»]
5EODX-ray3.10A20-625[»]
5EOKX-ray2.80A20-625[»]
5EXLX-ray2.30A388-625[»]
5EXMX-ray2.09A388-625[»]
5EXNX-ray1.49A388-625[»]
5I25X-ray2.85A19-625[»]
ProteinModelPortaliP03951.
SMRiP03951. Positions 20-623.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP03951.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini20 – 10384Apple 1PROSITE-ProRule annotationAdd
BLAST
Domaini110 – 19384Apple 2PROSITE-ProRule annotationAdd
BLAST
Domaini200 – 28384Apple 3PROSITE-ProRule annotationAdd
BLAST
Domaini291 – 37484Apple 4PROSITE-ProRule annotationAdd
BLAST
Domaini388 – 623236Peptidase S1PROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni547 – 5504Heparin-binding

Sequence similaritiesi

Belongs to the peptidase S1 family. Plasma kallikrein subfamily.PROSITE-ProRule annotation
Contains 4 apple domains.PROSITE-ProRule annotation
Contains 1 peptidase S1 domain.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Signal

Phylogenomic databases

eggNOGiKOG3627. Eukaryota.
COG5640. LUCA.
GeneTreeiENSGT00760000118962.
HOGENOMiHOG000112467.
HOVERGENiHBG000399.
InParanoidiP03951.
KOiK01323.
OMAiGWGYRKL.
OrthoDBiEOG091G0AH5.
PhylomeDBiP03951.
TreeFamiTF343687.

Family and domain databases

CDDicd00190. Tryp_SPc. 1 hit.
InterProiIPR000177. Apple.
IPR003609. Pan_app.
IPR009003. Peptidase_S1_PA.
IPR001314. Peptidase_S1A.
IPR001254. Trypsin_dom.
IPR018114. TRYPSIN_HIS.
IPR033116. TRYPSIN_SER.
[Graphical view]
PfamiPF00024. PAN_1. 4 hits.
PF00089. Trypsin. 1 hit.
[Graphical view]
PRINTSiPR00005. APPLEDOMAIN.
PR00722. CHYMOTRYPSIN.
SMARTiSM00223. APPLE. 4 hits.
SM00020. Tryp_SPc. 1 hit.
[Graphical view]
SUPFAMiSSF50494. SSF50494. 1 hit.
PROSITEiPS00495. APPLE. 4 hits.
PS50948. PAN. 4 hits.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 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. AlignAdd to basket

Isoform 1 (identifier: P03951-1) [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
MIFLYQVVHF ILFTSVSGEC VTQLLKDTCF EGGDITTVFT PSAKYCQVVC
60 70 80 90 100
TYHPRCLLFT FTAESPSEDP TRWFTCVLKD SVTETLPRVN RTAAISGYSF
110 120 130 140 150
KQCSHQISAC NKDIYVDLDM KGINYNSSVA KSAQECQERC TDDVHCHFFT
160 170 180 190 200
YATRQFPSLE HRNICLLKHT QTGTPTRITK LDKVVSGFSL KSCALSNLAC
210 220 230 240 250
IRDIFPNTVF ADSNIDSVMA PDAFVCGRIC THHPGCLFFT FFSQEWPKES
260 270 280 290 300
QRNLCLLKTS ESGLPSTRIK KSKALSGFSL QSCRHSIPVF CHSSFYHDTD
310 320 330 340 350
FLGEELDIVA AKSHEACQKL CTNAVRCQFF TYTPAQASCN EGKGKCYLKL
360 370 380 390 400
SSNGSPTKIL HGRGGISGYT LRLCKMDNEC TTKIKPRIVG GTASVRGEWP
410 420 430 440 450
WQVTLHTTSP TQRHLCGGSI IGNQWILTAA HCFYGVESPK ILRVYSGILN
460 470 480 490 500
QSEIKEDTSF FGVQEIIIHD QYKMAESGYD IALLKLETTV NYTDSQRPIC
510 520 530 540 550
LPSKGDRNVI YTDCWVTGWG YRKLRDKIQN TLQKAKIPLV TNEECQKRYR
560 570 580 590 600
GHKITHKMIC AGYREGGKDA CKGDSGGPLS CKHNEVWHLV GITSWGEGCA
610 620
QRERPGVYTN VVEYVDWILE KTQAV
Length:625
Mass (Da):70,109
Last modified:October 23, 1986 - v1
Checksum:i147AFA94B7709E8F
GO
Isoform 2 (identifier: P03951-2) [UniParc]FASTAAdd to basket
Also known as: Platelet

The sequence of this isoform differs from the canonical sequence as follows:
     109-162: Missing.

Show »
Length:571
Mass (Da):63,840
Checksum:iFB9D65D72151755E
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti226 – 2261C → S in AAA51985 (PubMed:2827746).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti30 – 301F → S in FA11D. 1 Publication
VAR_076515
Natural varianti32 – 321G → R in FA11D. 1 Publication
Corresponds to variant rs281875259 [ dbSNP | Ensembl ].
VAR_067929
Natural varianti34 – 341D → H in FA11D. 1 Publication
Corresponds to variant rs281875267 [ dbSNP | Ensembl ].
VAR_012085
Natural varianti43 – 431A → T in FA11D; dominant-negative mutation that results in severely decreased protein secretion. 1 Publication
Corresponds to variant rs281875264 [ dbSNP | Ensembl ].
VAR_067930
Natural varianti46 – 461C → F in FA11D. 1 Publication
Corresponds to variant rs281875271 [ dbSNP | Ensembl ].
VAR_054894
Natural varianti51 – 511T → I in FA11D. 1 Publication
Corresponds to variant rs281875252 [ dbSNP | Ensembl ].
VAR_067931
Natural varianti51 – 511T → P in FA11D. 2 Publications
Corresponds to variant rs281875243 [ dbSNP | Ensembl ].
VAR_067932
Natural varianti53 – 531H → Q in FA11D. 1 Publication
Corresponds to variant rs281875261 [ dbSNP | Ensembl ].
VAR_067933
Natural varianti56 – 561C → R in FA11D; secretion of the mutant protein is impaired. 2 Publications
Corresponds to variant rs121965069 [ dbSNP | Ensembl ].
VAR_054895
Natural varianti63 – 631A → V in FA11D. 1 Publication
Corresponds to variant rs281875244 [ dbSNP | Ensembl ].
VAR_067934
Natural varianti66 – 661P → L.1 Publication
Corresponds to variant rs5968 [ dbSNP | Ensembl ].
VAR_011774
Natural varianti101 – 1011K → R in FA11D. 1 Publication
Corresponds to variant rs281875272 [ dbSNP | Ensembl ].
VAR_054896
Natural varianti109 – 1091A → T in FA11D. 1 Publication
VAR_076516
Natural varianti140 – 1401C → Y in FA11D. 1 Publication
Corresponds to variant rs281875256 [ dbSNP | Ensembl ].
VAR_067935
Natural varianti151 – 1511Y → C in FA11D. 1 Publication
Corresponds to variant rs281875273 [ dbSNP | Ensembl ].
VAR_054897
Natural varianti216 – 2161D → N in FA11D. 1 Publication
VAR_076517
Natural varianti222 – 2221D → Y in FA11D. 1 Publication
Corresponds to variant rs281875245 [ dbSNP | Ensembl ].
VAR_067936
Natural varianti228 – 2281R → Q in FA11D. 1 Publication
Corresponds to variant rs281875246 [ dbSNP | Ensembl ].
VAR_067937
Natural varianti241 – 2411F → L in FA11D; dominant-negative mutation that results in severely decreased protein secretion. 1 Publication
Corresponds to variant rs281875265 [ dbSNP | Ensembl ].
VAR_067938
Natural varianti244 – 2441Q → R Found in a patient with factor XI deficiency that also carries mutation N-266. 2 Publications
Corresponds to variant rs5969 [ dbSNP | Ensembl ].
VAR_011775
Natural varianti246 – 2461W → C in FA11D. 1 Publication
Corresponds to variant rs281875279 [ dbSNP | Ensembl ].
VAR_012086
Natural varianti252 – 2521R → T in FA11D. 1 Publication
Corresponds to variant rs281875260 [ dbSNP | Ensembl ].
VAR_067939
Natural varianti255 – 2551C → Y in FA11D; secretion of the mutant protein is impaired. 1 Publication
Corresponds to variant rs281875277 [ dbSNP | Ensembl ].
VAR_054898
Natural varianti263 – 2631G → E in FA11D. 1 Publication
Corresponds to variant rs281875274 [ dbSNP | Ensembl ].
VAR_054899
Natural varianti266 – 2661S → N in FA11D. 1 Publication
Corresponds to variant rs145168351 [ dbSNP | Ensembl ].
VAR_012087
Natural varianti270 – 2701K → I in FA11D; although the mutant protein is synthesized the secretion is reduced. 1 Publication
Corresponds to variant rs121965070 [ dbSNP | Ensembl ].
VAR_054900
Natural varianti276 – 2761S → C in FA11D. 1 Publication
Corresponds to variant rs281875247 [ dbSNP | Ensembl ].
VAR_067940
Natural varianti277 – 2771G → D in FA11D. 1 Publication
Corresponds to variant rs281875248 [ dbSNP | Ensembl ].
VAR_067941
Natural varianti301 – 3011F → L in FA11D. 2 Publications
Corresponds to variant rs121965064 [ dbSNP | Ensembl ].
VAR_006622
Natural varianti308 – 3081I → F.1 Publication
Corresponds to variant rs5972 [ dbSNP | Ensembl ].
VAR_011776
Natural varianti315 – 3151E → K in FA11D. 2 Publications
Corresponds to variant rs281875257 [ dbSNP | Ensembl ].
VAR_067942
Natural varianti320 – 3201L → P in FA11D. 1 Publication
Corresponds to variant rs281875268 [ dbSNP | Ensembl ].
VAR_012088
Natural varianti322 – 3221T → I in FA11D. 1 Publication
Corresponds to variant rs281875269 [ dbSNP | Ensembl ].
VAR_012089
Natural varianti326 – 3261R → C in FA11D. 1 Publication
Corresponds to variant rs28934608 [ dbSNP | Ensembl ].
VAR_012090
Natural varianti331 – 3311T → I in FA11D. 1 Publication
Corresponds to variant rs281875253 [ dbSNP | Ensembl ].
VAR_067943
Natural varianti339 – 3391C → F.3 Publications
Corresponds to variant rs5967 [ dbSNP | Ensembl ].
VAR_011777
Natural varianti341 – 3411E → K in FA11D. 1 Publication
Corresponds to variant rs281875270 [ dbSNP | Ensembl ].
VAR_012091
Natural varianti360 – 3601L → P in FA11D. 1 Publication
Corresponds to variant rs281875254 [ dbSNP | Ensembl ].
VAR_067944
Natural varianti399 – 3991W → R.
Corresponds to variant rs1800439 [ dbSNP | Ensembl ].
VAR_011778
Natural varianti401 – 4011W → R in FA11D. 1 Publication
Corresponds to variant rs281875262 [ dbSNP | Ensembl ].
VAR_067945
Natural varianti403 – 4031V → M in FA11D; dominant-negative mutation that results in severely decreased protein secretion. 1 Publication
Corresponds to variant rs281875266 [ dbSNP | Ensembl ].
VAR_067946
Natural varianti404 – 4041T → N in FA11D. 1 Publication
Corresponds to variant rs121965067 [ dbSNP | Ensembl ].
VAR_012092
Natural varianti418 – 4181G → V in FA11D; mutant is not secreted by transfected fibroblasts; dominant-negative effect. 1 Publication
Corresponds to variant rs121965071 [ dbSNP | Ensembl ].
VAR_054901
Natural varianti430 – 4301A → V in FA11D. 1 Publication
Corresponds to variant rs121965068 [ dbSNP | Ensembl ].
VAR_012093
Natural varianti454 – 4541I → K in FA11D. 1 Publication
Corresponds to variant rs281875241 [ dbSNP | Ensembl ].
VAR_067947
Natural varianti460 – 4601F → V in FA11D. 1 Publication
Corresponds to variant rs121965065 [ dbSNP | Ensembl ].
VAR_012094
Natural varianti481 – 4811I → S in FA11D. 1 Publication
Corresponds to variant rs281875242 [ dbSNP | Ensembl ].
VAR_067948
Natural varianti493 – 4931T → I in FA11D. 1 Publication
VAR_012095
Natural varianti503 – 5031S → P in FA11D. 1 Publication
Corresponds to variant rs140068026 [ dbSNP | Ensembl ].
VAR_067949
Natural varianti506 – 5061D → G in FA11D; mild phenotype. 1 Publication
Corresponds to variant rs281875258 [ dbSNP | Ensembl ].
VAR_067950
Natural varianti511 – 5111Y → H in FA11D; transfected cells contain reduced amount of mutant protein and display decreased secretion. 1 Publication
Corresponds to variant rs281875278 [ dbSNP | Ensembl ].
VAR_054902
Natural varianti514 – 5141C → F in FA11D. 1 Publication
Corresponds to variant rs281875249 [ dbSNP | Ensembl ].
VAR_067951
Natural varianti526 – 5261D → E in FA11D. 1 Publication
Corresponds to variant rs281875263 [ dbSNP | Ensembl ].
VAR_067952
Natural varianti538 – 5381P → L in FA11D. 1 Publication
Corresponds to variant rs139695003 [ dbSNP | Ensembl ].
VAR_054903
Natural varianti543 – 5431E → K in FA11D. 1 Publication
VAR_076518
Natural varianti552 – 5521H → R in FA11D. 1 Publication
VAR_076519
Natural varianti565 – 5651E → K in FA11D. 1 Publication
Corresponds to variant rs281875275 [ dbSNP | Ensembl ].
VAR_054904
Natural varianti575 – 5751S → L in FA11D. 1 Publication
Corresponds to variant rs281875250 [ dbSNP | Ensembl ].
VAR_067953
Natural varianti587 – 5871W → S in FA11D; mutant is not secreted by transfected fibroblasts; dominant-negative effect. 1 Publication
Corresponds to variant rs121965072 [ dbSNP | Ensembl ].
VAR_054905
Natural varianti594 – 5941S → R in FA11D. 1 Publication
Corresponds to variant rs28934609 [ dbSNP | Ensembl ].
VAR_012096
Natural varianti597 – 5971E → K in FA11D. 2 Publications
Corresponds to variant rs281875251 [ dbSNP | Ensembl ].
VAR_067954
Natural varianti608 – 6081Y → H in FA11D. 1 Publication
Corresponds to variant rs281875255 [ dbSNP | Ensembl ].
VAR_067955
Natural varianti618 – 6181I → S in FA11D. 1 Publication
Corresponds to variant rs281875276 [ dbSNP | Ensembl ].
VAR_054906

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei109 – 16254Missing in isoform 2. 1 PublicationVSP_005388Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M13142 mRNA. Translation: AAA52487.1.
M20218
, M18296, M21184, M18298, M18299, M18300, M18301, M18302, M18303, M18304, M19417, M20217 Genomic DNA. Translation: AAA51985.1.
AF045649 mRNA. Translation: AAC24506.1.
AY191837 Genomic DNA. Translation: AAN85554.1.
AC110771 Genomic DNA. Translation: AAY40901.1.
CH471056 Genomic DNA. Translation: EAX04621.1.
BC119014 mRNA. Translation: AAI19015.1.
BC122863 mRNA. Translation: AAI22864.1.
CCDSiCCDS3847.1. [P03951-1]
PIRiA27431. KFHU1.
RefSeqiNP_000119.1. NM_000128.3. [P03951-1]
UniGeneiHs.1430.

Genome annotation databases

EnsembliENST00000264692; ENSP00000264692; ENSG00000088926. [P03951-2]
ENST00000403665; ENSP00000384957; ENSG00000088926. [P03951-1]
GeneIDi2160.
KEGGihsa:2160.
UCSCiuc003iza.2. human. [P03951-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Web resourcesi

Wikipedia

Factor XI entry

SeattleSNPs
Mendelian genes Coagulation factor XI (F11)

Leiden Open Variation Database (LOVD)

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M13142 mRNA. Translation: AAA52487.1.
M20218
, M18296, M21184, M18298, M18299, M18300, M18301, M18302, M18303, M18304, M19417, M20217 Genomic DNA. Translation: AAA51985.1.
AF045649 mRNA. Translation: AAC24506.1.
AY191837 Genomic DNA. Translation: AAN85554.1.
AC110771 Genomic DNA. Translation: AAY40901.1.
CH471056 Genomic DNA. Translation: EAX04621.1.
BC119014 mRNA. Translation: AAI19015.1.
BC122863 mRNA. Translation: AAI22864.1.
CCDSiCCDS3847.1. [P03951-1]
PIRiA27431. KFHU1.
RefSeqiNP_000119.1. NM_000128.3. [P03951-1]
UniGeneiHs.1430.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1XX9X-ray2.20A/B388-625[»]
1XXDX-ray2.91A/B388-625[»]
1XXFX-ray2.60A/B388-625[»]
1ZHMX-ray1.96A388-624[»]
1ZHPX-ray2.70A388-625[»]
1ZHRX-ray1.73A388-625[»]
1ZJDX-ray2.60A388-624[»]
1ZLRX-ray2.50A388-624[»]
1ZMJX-ray2.00A388-624[»]
1ZMLX-ray2.25A388-625[»]
1ZMNX-ray2.05A388-625[»]
1ZOMX-ray2.25A388-624[»]
1ZPBX-ray2.10A388-624[»]
1ZPCX-ray2.60A388-624[»]
1ZPZX-ray2.50A388-625[»]
1ZRKX-ray2.30A388-625[»]
1ZSJX-ray1.90A388-625[»]
1ZSKX-ray1.90A388-625[»]
1ZSLX-ray2.05A388-625[»]
1ZTJX-ray2.05A388-624[»]
1ZTKX-ray2.50A388-624[»]
1ZTLX-ray2.60A388-624[»]
2F83X-ray2.87A1-625[»]
2FDAX-ray2.00A388-625[»]
2J8JNMR-A/B290-379[»]
2J8LNMR-A/B290-379[»]
3BG8X-ray1.60A388-625[»]
3SORX-ray1.80A388-625[»]
3SOSX-ray2.58A388-625[»]
4CR5X-ray2.00A388-625[»]
4CR9X-ray1.70A388-625[»]
4CRAX-ray1.80A388-625[»]
4CRBX-ray1.85A388-625[»]
4CRCX-ray1.60A388-625[»]
4CRDX-ray2.10A388-625[»]
4CREX-ray1.73A388-625[»]
4CRFX-ray2.30A388-625[»]
4CRGX-ray1.25A388-625[»]
4D76X-ray1.77A388-625[»]
4D7FX-ray1.62A388-625[»]
4D7GX-ray2.33A388-625[»]
4NA7X-ray2.80A388-625[»]
4NA8X-ray2.30A388-625[»]
4TY6X-ray1.85A388-625[»]
H375-387[»]
4TY7X-ray2.09A388-625[»]
4WXIX-ray2.60A388-625[»]
4X6MX-ray2.40A388-625[»]
4X6NX-ray2.10A388-625[»]
H375-387[»]
4X6OX-ray2.10A388-625[»]
4X6PX-ray1.93A/B388-625[»]
4Y8XX-ray1.90A388-625[»]
4Y8YX-ray2.60A388-625[»]
4Y8ZX-ray2.20A388-625[»]
5E2OX-ray2.08A388-625[»]
5E2PX-ray2.11A388-625[»]
5EODX-ray3.10A20-625[»]
5EOKX-ray2.80A20-625[»]
5EXLX-ray2.30A388-625[»]
5EXMX-ray2.09A388-625[»]
5EXNX-ray1.49A388-625[»]
5I25X-ray2.85A19-625[»]
ProteinModelPortaliP03951.
SMRiP03951. Positions 20-623.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108458. 6 interactions.
DIPiDIP-29085N.
IntActiP03951. 1 interaction.
STRINGi9606.ENSP00000384957.

Chemistry

BindingDBiP03951.
ChEMBLiCHEMBL2820.
DrugBankiDB00100. Coagulation Factor IX.
GuidetoPHARMACOLOGYi2360.

Protein family/group databases

MEROPSiS01.213.

PTM databases

iPTMnetiP03951.
PhosphoSiteiP03951.

Polymorphism and mutation databases

BioMutaiF11.
DMDMi119762.

Proteomic databases

PaxDbiP03951.
PeptideAtlasiP03951.
PRIDEiP03951.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000264692; ENSP00000264692; ENSG00000088926. [P03951-2]
ENST00000403665; ENSP00000384957; ENSG00000088926. [P03951-1]
GeneIDi2160.
KEGGihsa:2160.
UCSCiuc003iza.2. human. [P03951-1]

Organism-specific databases

CTDi2160.
GeneCardsiF11.
HGNCiHGNC:3529. F11.
MalaCardsiF11.
MIMi264900. gene.
612416. phenotype.
neXtProtiNX_P03951.
Orphaneti329. Congenital factor XI deficiency.
PharmGKBiPA27941.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3627. Eukaryota.
COG5640. LUCA.
GeneTreeiENSGT00760000118962.
HOGENOMiHOG000112467.
HOVERGENiHBG000399.
InParanoidiP03951.
KOiK01323.
OMAiGWGYRKL.
OrthoDBiEOG091G0AH5.
PhylomeDBiP03951.
TreeFamiTF343687.

Enzyme and pathway databases

BRENDAi3.4.21.27. 2681.
ReactomeiR-HSA-140837. Intrinsic Pathway of Fibrin Clot Formation.
SABIO-RKP03951.

Miscellaneous databases

ChiTaRSiF11. human.
EvolutionaryTraceiP03951.
GeneWikiiFactor_XI.
GenomeRNAii2160.
PMAP-CutDBP03951.
PROiP03951.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000088926.
CleanExiHS_F11.
ExpressionAtlasiP03951. baseline and differential.
GenevisibleiP03951. HS.

Family and domain databases

CDDicd00190. Tryp_SPc. 1 hit.
InterProiIPR000177. Apple.
IPR003609. Pan_app.
IPR009003. Peptidase_S1_PA.
IPR001314. Peptidase_S1A.
IPR001254. Trypsin_dom.
IPR018114. TRYPSIN_HIS.
IPR033116. TRYPSIN_SER.
[Graphical view]
PfamiPF00024. PAN_1. 4 hits.
PF00089. Trypsin. 1 hit.
[Graphical view]
PRINTSiPR00005. APPLEDOMAIN.
PR00722. CHYMOTRYPSIN.
SMARTiSM00223. APPLE. 4 hits.
SM00020. Tryp_SPc. 1 hit.
[Graphical view]
SUPFAMiSSF50494. SSF50494. 1 hit.
PROSITEiPS00495. APPLE. 4 hits.
PS50948. PAN. 4 hits.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiFA11_HUMAN
AccessioniPrimary (citable) accession number: P03951
Secondary accession number(s): D3DP64, Q4W5C2, Q9Y495
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 23, 1986
Last sequence update: October 23, 1986
Last modified: September 7, 2016
This is version 202 of the entry and version 1 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

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: 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. Peptidase families
    Classification of peptidase families and list of entries
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.