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coagulation factor XI; FXI; plasma thromboplastin antecedent; PTA; contains: coagulation factor XIa heavy chain; coagulation factor XIa light chain (F11)

Function: - precursor for coagulation factor XIa - activated by factor XIIa (or XII), which cleaves each polypeptide after Arg-387 into the light chain, which contains the active site, & the heavy chain, which associates with high molecular weight (HMW) kininogen - homodimer; disulfide-linked - after activation the heavy & light chains are also linked by a disulfide bond Structure: - belongs to the peptidase S1 family, plasma kallikrein subfamily - contains 4 apple domains - contains 1 peptidase S1 domain Compartment: secreted Alternative splicing: named isoforms=2 Expression: - isoform 2 is produced by platelets & megakaryocytes but absent from other blood cells Pathology: - defects in F11 are the cause of factor XI deficiency Laboratory: - F11 gene mutation

Interactions

molecular events

Related

coagulation cascade coagulation factor XIa factor XI deficiency; plasma thromboplastin antecedent deficiency; Rosenthal syndrome

Specific

factor IXI variant

General

coagulation factor enzyme precursor (zymogen)

Properties

SIZE: entity length = 625 aa MW = 70 kD COMPARTMENT: plasma MOTIF: signal sequence {1-18} MOTIF: Ser phosphorylation site {S17} Apple 1 {20-103} MOTIF: cysteine residue {C20} MODIFICATION: cysteine residue {C103} Thr phosphorylation site {T22} cysteine residue {C29} MODIFICATION: cysteine residue {C-INTERCHAIN} cysteine residue {C46} MODIFICATION: cysteine residue {C76} cysteine residue {C50} MODIFICATION: cysteine residue {C56} cysteine residue {C56} MODIFICATION: cysteine residue {C50} cysteine residue {C76} MODIFICATION: cysteine residue {C46} N-glycosylation site {N90} cysteine residue {C103} MODIFICATION: cysteine residue {C20} Apple 2 {110-193} MOTIF: cysteine residue {C110} MODIFICATION: cysteine residue {C193} N-glycosylation site {N126} cysteine residue {C136} MODIFICATION: cysteine residue {C165} cysteine residue {C140} MODIFICATION: cysteine residue {C146} cysteine residue {C146} MODIFICATION: cysteine residue {C140} cysteine residue {C165} MODIFICATION: cysteine residue {C136} cysteine residue {C193} MODIFICATION: cysteine residue {C110} Apple 3 {200-283} MOTIF: cysteine residue {C200} MODIFICATION: cysteine residue {C283} cysteine residue {C226} MODIFICATION: cysteine residue {C255} cysteine residue {C230} MODIFICATION: cysteine residue {C236} cysteine residue {C236} MODIFICATION: cysteine residue {C230} cysteine residue {C255} MODIFICATION: cysteine residue {C226} cysteine residue {C283} MODIFICATION: cysteine residue {C200} Apple 4 {291-374} MOTIF: cysteine residue {C291} MODIFICATION: cysteine residue {C374} cysteine residue {C317} MODIFICATION: cysteine residue {C346} cysteine residue {C321} MODIFICATION: cysteine residue {C327} cysteine residue {C327} MODIFICATION: cysteine residue {C321} cysteine residue {C339} MODIFICATION: cysteine residue {C-INTERCHAIN} cysteine residue {C346} MODIFICATION: cysteine residue {C317} N-glycosylation site {N353} cysteine residue {C374} MODIFICATION: cysteine residue {C291} cysteine residue {C380} MODIFICATION: cysteine residue {C-INTERCHAIN} S1 domain {388-623} MOTIF: cysteine residue {C416} MODIFICATION: cysteine residue {C432} histidine residue {H431} cysteine residue {C432} MODIFICATION: cysteine residue {C416} N-glycosylation site {N450} aspartate residue {D480} N-glycosylation site {N491} cysteine residue {C514} MODIFICATION: cysteine residue {C581} cysteine residue {C545} MODIFICATION: cysteine residue {C560} binding site SITE: 547-550 FOR-BINDING-OF: heparin cysteine residue {C560} MODIFICATION: cysteine residue {C545} cysteine residue {C571} MODIFICATION: cysteine residue {C599} serine residue {S575} cysteine residue {C581} MODIFICATION: cysteine residue {C514} cysteine residue {C599} MODIFICATION: cysteine residue {C571} PRECURSOR-FOR: coagulation factor XIa

Database Correlations

OMIM correlations MORBIDMAP 264900 UniProt P03951 PFAM correlations Entrez Gene 2160 Kegg hsa:2160 ENZYME 3.4.21.27

References

  1. UniProt :accession P03951
  2. Wikipedia; Note: factor XI entry http://en.wikipedia.org/wiki/factor_XI
  3. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/F11
  4. SeattleSNPs http://pga.gs.washington.edu/data/f11/