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  Coagulation factor XI

SourceHomo sapiens (human)
Taxonomy Homo sapiens Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.
Keywords3D-structure; Alternative splicing; Blood coagulation; Complete proteome; Direct protein sequencing; Disease mutation; Disulfide bond; Glycoprotein; Heparin-binding; Hydrolase; Phosphoprotein; Polymorphism; Protease; Repeat; Secreted; Serine protease; Signal.
Details
Function: Factor XI triggers the middle phase of the intrinsic pathway of blood coagulation by activating factor IX.

Post-translational modification: 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.

Similarity: Belongs to the peptidase S1 family. Plasma kallikrein subfamily. Contains 4 apple domains. Contains 1 peptidase S1 domain.

Subcellular location: Secreted.

Subunit structure: Homodimer; disulfide-linked. After activation the heavy and light chains are also linked by a disulfide bond.

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

Disease: Defects in F11 are the cause of F11 deficiency.

Alternative products: Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P03951-1; Sequence=Displayed; Name=2; Synonyms=Platelet; IsoId=P03951-2; Sequence=VSP_005388.

Sequence length: 625 AA.

Sequence
MIFLYQVVHFILFTSVSGECVTQLLKDTCFEGGDITTVFTPSAKYCQVVCTYHPRCLLFT
FTAESPSEDPTRWFTCVLKDSVTETLPRVNRTAAISGYSFKQCSHQISACNKDIYVDLDM
KGINYNSSVAKSAQECQERCTDDVHCHFFTYATRQFPSLEHRNICLLKHTQTGTPTRITK
LDKVVSGFSLKSCALSNLACIRDIFPNTVFADSNIDSVMAPDAFVCGRICTHHPGCLFFT
FFSQEWPKESQRNLCLLKTSESGLPSTRIKKSKALSGFSLQSCRHSIPVFCHSSFYHDTD
FLGEELDIVAAKSHEACQKLCTNAVRCQFFTYTPAQASCNEGKGKCYLKLSSNGSPTKIL
HGRGGISGYTLRLCKMDNECTTKIKPRIVGGTASVRGEWPWQVTLHTTSPTQRHLCGGSI
IGNQWILTAAHCFYGVESPKILRVYSGILNQSEIKEDTSFFGVQEIIIHDQYKMAESGYD
IALLKLETTVNYTDSQRPICLPSKGDRNVIYTDCWVTGWGYRKLRDKIQNTLQKAKIPLV
TNEECQKRYRGHKITHKMICAGYREGGKDACKGDSGGPLSCKHNEVWHLVGITSWGEGCA
QRERPGVYTNVVEYVDWILEKTQAV
Accession NumberP03951 
PubMed ID3636155, 2827746, 9593722, 15815621, 15489334, 1998667, 11412111, 16335952, 18187866, 19159218, 2813350, 1547342, 7888672, 7669672, 9401068, 9787168, 10027710, 10606881, 10391209, 11895778, 15026311, 15180874, 15953011 
CEX DBHS_F11
CTD DB2160
DIP DBDIP-29085N
eggNOG DBprNOG12288
Ensembl DBENST00000264691, ENST00000403665
Genecard DBGC04P187424
GeneID DB2160
GermOnline DBENSG00000088926
GO DB0005615, 0016020, 0008201, 0004252, 0007596, 0031639, 0051919, 0006508
HGNC DB3529
HOGENOM DBHBG281927
InterPro DBIPR000177, IPR003014, IPR003609, IPR018114, IPR001254, IPR001314, IPR009003
H-InvDBHIX0031358
IPI DBIPI00008556, IPI00216588
KEGGhsa:2160
NCBIM13142, AAA52487, M20218, AAA51985, M18296, M21184, M18298, M18299, M18300, M18301, M18302, M18303, M18304, M19417, M20217, AF045649, AAC24506, AY191837, AAN85554, AC110771.3, AAY40901, BC119014, AAI19015, BC122863, AAI22864, NP_000119
NMPDR fig|9606.3.peg.24953
OMAGSPTKIL
OMIM105200, 134820, 202400, 134830, 202400, 134850, 202400, 176930, 601367, 134390, 188055, 227400, 600880, 601367, 612309, 227500, 134500, 306700, 300746, 306900, 227600, 176860, 188050, 612283, 612304, 176880, 612336, 264900, 612416
Orphanet DB329
OrthoDBEOG9ZCWPQ
PDB1XX9_A, 1XX9_B, 1XXD_A, 1XXD_B, 1XXF_A, 1XXF_B, 1ZHM_A, 1ZHP_A, 1ZHR_A, 1ZJD_A, 1ZLR_A, 1ZMJ_A, 1ZML_A, 1ZMN_A, 1ZOM_A, 1ZPB_A, 1ZPC_A, 1ZPZ_A, 1ZRK_A, 1ZSJ_A, 1ZSK_A, 1ZSL_A, 1ZTJ_A, 1ZTK_A, 1ZTL_A, 2F83_A, 2FDA_A, 2J8J_A, 2J8J_B, 2J8L_A, 2J8L_B, 3BG8_A
PfamPF00024, PF00089
PharmaGKBPA24968
PROSITE DBPS00495, PS50948, PS50240, PS00134, PS00135
SMART DBSM00223, SM00020
UCSCuc003iza.1
UniGeneHs.1430, Hs.714107



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