BRENDA - Enzyme Database show
show all sequences of 3.4.21.27

Structure and function of factor XI

Emsley, J.; McEwan, P.A.; Gailani, D.; Blood 115, 2569-2577 (2010)

Data extracted from this reference:

Activating Compound
Activating Compound
Commentary
Organism
Structure
activated factor IX
autoactivation, cleavage of FXI at the Arg369-Ile370 bond. activated factor IX is part of a feedback loop that sustains thrombin generation through FIX activation to consolidate coagulation
Homo sapiens
activated factor XII
cleaves FXI at the Arg369-Ile370 bond
Homo sapiens
activated factor XII
activated factor XII-mediated activation of FXI appears to play a central role in formation of pathologic thrombi in murine thrombosis models
Mus musculus
alpha-thrombin
cleaves FXI at the Arg369-Ile370 bond
Homo sapiens
meizothrombin
cleaves FXI at the Arg369-Ile370 bond
Homo sapiens
additional information
platelets may play a role in FXI activation. FXI binding to platelets is not enhanced by kininogen
Homo sapiens
thrombin
activates FXI. FXI activation by thrombin is enhanced by charged substances, such as dextran sulfate or heparin. Thrombin binds to the A1 domain of FXI, with residues Glu66, Lys83, and Gln84 forming part of the binding site. The dimer is required for optimal FXI activation by thrombin
Mus musculus
Crystallization (Commentary)
Crystallization
Organism
full-length zymogen FXI, and the isolated active protease domain of activated factor IX in complex with natural and synthetic inhibitors. Intimate linkage of the apple domains into a disk-like platform around the base of the catalytic domain
Homo sapiens
Engineering
Amino acid exchange
Commentary
Organism
C398Y
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
G104R
this mutation in the PK A2 domain associated with CRM+ PK deficiency causes decreased kininogen binding
Homo sapiens
G155E
is a rare example of a mutation causing CRM+ FXI deficieny
Homo sapiens
G400V
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
additional information
FXI with substitutions for Cys321 still form stable dimers. The Cys321-Cys321 interchain bond forms poorly in FXI with a single alanine substitution of Leu284, Ile290, or Tyr329
Homo sapiens
S225F
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
S248A
binds platelets with reduced affinity compared with wild-type
Homo sapiens
S248N
binds platelets with 5fold reduced affinity compared with wild-type, the A3 domain is probably not affected significantly, as FXI-Asn248 is secreted, activated by activated factor XII, and activates FIX similar to wild-type activated factor IX. Is associated with bleeding and defective FXI binding to platelets but does not affect the activated partial thromboplastin time assay, which does not contain platelets
Homo sapiens
S248Q
binds platelets with reduced affinity compared with wild-type
Homo sapiens
W569S
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
Inhibitors
Inhibitors
Commentary
Organism
Structure
additional information
Arg184 may be part of a switch that holds FXI in an inactive conformation in the zymogen
Homo sapiens
Metals/Ions
Metals/Ions
Commentary
Organism
Structure
Zn2+
in the presence of zinc ions, kininogen is required for optimal FXI binding to GP1b on activated platelets in suspension, enhancing the binding stoichiometry by approximately 2fold
Homo sapiens
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Homo sapiens
P03951
-
-
Mus musculus
-
-
-
Source Tissue
Source Tissue
Commentary
Organism
Textmining
blood
-
Homo sapiens
-
blood plasma
-
Mus musculus
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
factor IX + H2O
-
707856
Homo sapiens
activated factor IX + ?
-
-
-
?
kininogen + H2O
-
707856
Homo sapiens
?
-
-
-
?
additional information
FXI contains 4 apple domains that form a disk structure with extensive interfaces at the base of the catalytic domain. Binding of FXI to platelets requires residues in the FXI A3 domain. Platelets bind and spread on glass coated with FXI
707856
Homo sapiens
?
-
-
-
-
additional information
platelets bind and spread on glass coated with FXI. Platelets from ApoER2-deficient mice do not bind FXI
707856
Mus musculus
?
-
-
-
-
Activating Compound (protein specific)
Activating Compound
Commentary
Organism
Structure
activated factor IX
autoactivation, cleavage of FXI at the Arg369-Ile370 bond. activated factor IX is part of a feedback loop that sustains thrombin generation through FIX activation to consolidate coagulation
Homo sapiens
activated factor XII
cleaves FXI at the Arg369-Ile370 bond
Homo sapiens
activated factor XII
activated factor XII-mediated activation of FXI appears to play a central role in formation of pathologic thrombi in murine thrombosis models
Mus musculus
alpha-thrombin
cleaves FXI at the Arg369-Ile370 bond
Homo sapiens
meizothrombin
cleaves FXI at the Arg369-Ile370 bond
Homo sapiens
additional information
platelets may play a role in FXI activation. FXI binding to platelets is not enhanced by kininogen
Homo sapiens
thrombin
activates FXI. FXI activation by thrombin is enhanced by charged substances, such as dextran sulfate or heparin. Thrombin binds to the A1 domain of FXI, with residues Glu66, Lys83, and Gln84 forming part of the binding site. The dimer is required for optimal FXI activation by thrombin
Mus musculus
Crystallization (Commentary) (protein specific)
Crystallization
Organism
full-length zymogen FXI, and the isolated active protease domain of activated factor IX in complex with natural and synthetic inhibitors. Intimate linkage of the apple domains into a disk-like platform around the base of the catalytic domain
Homo sapiens
Engineering (protein specific)
Amino acid exchange
Commentary
Organism
C398Y
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
G104R
this mutation in the PK A2 domain associated with CRM+ PK deficiency causes decreased kininogen binding
Homo sapiens
G155E
is a rare example of a mutation causing CRM+ FXI deficieny
Homo sapiens
G400V
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
additional information
FXI with substitutions for Cys321 still form stable dimers. The Cys321-Cys321 interchain bond forms poorly in FXI with a single alanine substitution of Leu284, Ile290, or Tyr329
Homo sapiens
S225F
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
S248A
binds platelets with reduced affinity compared with wild-type
Homo sapiens
S248N
binds platelets with 5fold reduced affinity compared with wild-type, the A3 domain is probably not affected significantly, as FXI-Asn248 is secreted, activated by activated factor XII, and activates FIX similar to wild-type activated factor IX. Is associated with bleeding and defective FXI binding to platelets but does not affect the activated partial thromboplastin time assay, which does not contain platelets
Homo sapiens
S248Q
binds platelets with reduced affinity compared with wild-type
Homo sapiens
W569S
exhibits a dominant negative effect when coexpressed with wild-type FXI and is associated with FXI deficiency that may be inherited in a dominant manner
Homo sapiens
Inhibitors (protein specific)
Inhibitors
Commentary
Organism
Structure
additional information
Arg184 may be part of a switch that holds FXI in an inactive conformation in the zymogen
Homo sapiens
Metals/Ions (protein specific)
Metals/Ions
Commentary
Organism
Structure
Zn2+
in the presence of zinc ions, kininogen is required for optimal FXI binding to GP1b on activated platelets in suspension, enhancing the binding stoichiometry by approximately 2fold
Homo sapiens
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
blood
-
Homo sapiens
-
blood plasma
-
Mus musculus
-
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
factor IX + H2O
-
707856
Homo sapiens
activated factor IX + ?
-
-
-
?
kininogen + H2O
-
707856
Homo sapiens
?
-
-
-
?
additional information
FXI contains 4 apple domains that form a disk structure with extensive interfaces at the base of the catalytic domain. Binding of FXI to platelets requires residues in the FXI A3 domain. Platelets bind and spread on glass coated with FXI
707856
Homo sapiens
?
-
-
-
-
additional information
platelets bind and spread on glass coated with FXI. Platelets from ApoER2-deficient mice do not bind FXI
707856
Mus musculus
?
-
-
-
-
General Information
General Information
Commentary
Organism
malfunction
congenital FXI deficiency is associated with a variable, mild to moderate bleeding disorder. Severe deficiency is prevalent in people of Jewish ancestry. The severe mutation Glu117Stop encodes a truncated protein, and homozygotes lack plasma FXI antigen. The more subtle missense mutation Phe283Leu causes a defect in FXI dimer formation. Most cases of FXI deficiency are associated with low plasma levels of FXI protein. Deficiency or inhibition of FXI interferes with platelet accumulation in growing thrombi. A4 domain mutations associated with FXI deficiency interfere with dimerization. FXI-deficient plasma exhibits a prolonged activated partial thromboplastin clotting time
Homo sapiens
malfunction
mice lacking FIX, FXI, or FXII on a background of low tissue factor expression have a severe bleeding disorder but are viable. Superimposing FIX or FXI deficiency on the low tissue factor background results in death in utero from bleeding
Mus musculus
physiological function
FXI is the zymogen of a blood coagulation protease, factor XIa (activated factor IX), that contributes to hemostasis through activation of factor IX. Almost all FXI circulate in a complex with kininogen
Homo sapiens
General Information (protein specific)
General Information
Commentary
Organism
malfunction
congenital FXI deficiency is associated with a variable, mild to moderate bleeding disorder. Severe deficiency is prevalent in people of Jewish ancestry. The severe mutation Glu117Stop encodes a truncated protein, and homozygotes lack plasma FXI antigen. The more subtle missense mutation Phe283Leu causes a defect in FXI dimer formation. Most cases of FXI deficiency are associated with low plasma levels of FXI protein. Deficiency or inhibition of FXI interferes with platelet accumulation in growing thrombi. A4 domain mutations associated with FXI deficiency interfere with dimerization. FXI-deficient plasma exhibits a prolonged activated partial thromboplastin clotting time
Homo sapiens
malfunction
mice lacking FIX, FXI, or FXII on a background of low tissue factor expression have a severe bleeding disorder but are viable. Superimposing FIX or FXI deficiency on the low tissue factor background results in death in utero from bleeding
Mus musculus
physiological function
FXI is the zymogen of a blood coagulation protease, factor XIa (activated factor IX), that contributes to hemostasis through activation of factor IX. Almost all FXI circulate in a complex with kininogen
Homo sapiens
Other publictions for EC 3.4.21.27
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [°C]
Temperature Range [°C]
Temperature Stability [°C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [°C] (protein specific)
Temperature Range [°C] (protein specific)
Temperature Stability [°C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
731462
Pinto
Structure-based design of inhi ...
Homo sapiens
Bioorg. Med. Chem. Lett.
25
1635-1642
2015
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731466
Corte
Pyridine and pyridinone-based ...
Homo sapiens
Bioorg. Med. Chem. Lett.
25
925-930
2015
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731538
Puy
Activated factor XI increases ...
Homo sapiens
Blood
125
1488-1496
2015
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1
1
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732313
Argade
Allosteric inhibition of human ...
Homo sapiens
J. Med. Chem.
57
3559-3569
2014
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11
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732318
Hangeland
Phenylimidazoles as potent and ...
Homo sapiens
J. Med. Chem.
57
9915-9932
2014
-
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-
1
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35
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732897
Faid
Site-specific N-glycosylation ...
Homo sapiens
Proteomics
14
2460-2470
2014
-
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732936
Gailani
-
The mechanism underlying activ ...
Homo sapiens
Thromb. Res.
133
S48-S51
2014
1
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731570
Cho
-
Characterization of binding mo ...
Homo sapiens
Bull. Korean Chem. Soc.
34
1212-1220
2013
-
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42
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731773
Ataullakhanov
Factor XI and traveling waves: ...
Homo sapiens
Expert Rev. Hematol.
6
111-113
2013
2
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2
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731076
Fradera
High-resolution crystal struct ...
Homo sapiens
Acta Crystallogr. Sect. F
68
404-408
2012
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1
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717416
Luszczak
Activated factor XI and tissue ...
Homo sapiens
Blood Coagul. Fibrinolysis
22
473-479
2011
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717844
Su
The role of factor XIa (FXIa) ...
Homo sapiens
J. Biol. Chem.
286
31904-31914
2011
1
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717958
Itakura
Activated factor XI inhibits c ...
Homo sapiens
J. Leukoc. Biol.
90
923-927
2011
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718045
Carrieri
The role of thrombin activatab ...
Homo sapiens
J. Thromb. Haemost.
9
154-162
2011
2
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1
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707308
Schumacher
Inhibition of factor XIa as a ...
Homo sapiens, Mus musculus, Oryctolagus cuniculus, Rattus norvegicus
Arterioscler. Thromb. Vasc. Biol.
30
388-392
2010
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707856
Emsley
Structure and function of fact ...
Homo sapiens, Mus musculus
Blood
115
2569-2577
2010
7
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1
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7
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10
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4
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3
3
-
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710438
Maas
Activated factor V is a cofact ...
Homo sapiens
Proc. Natl. Acad. Sci. USA
107
9083-9087
2010
1
-
1
-
-
-
-
-
-
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1
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1
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-
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-
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-
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-
-
-
717333
Hanessian
Design and synthesis of macroc ...
Homo sapiens
Bioorg. Med. Chem. Lett.
20
6925-6928
2010
-
-
-
-
-
-
5
-
-
-
-
-
-
1
-
-
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-
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-
-
-
-
717420
Cheng
A role for factor XIIa-mediate ...
Mus musculus
Blood
116
3981-3989
2010
2
-
-
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-
-
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1
-
7
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2
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-
-
717742
Navaneetham
Mechanisms and specificity of ...
Homo sapiens
J. Biochem.
148
467-479
2010
-
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-
10
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1
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1
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9
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10
9
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1
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3
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718042
Whelihan
Coagulation procofactor activa ...
Homo sapiens
J. Thromb. Haemost.
8
1532-1539
2010
1
-
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1
1
3
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1
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4
1
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1
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1
3
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1
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4
1
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-
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-
691011
Yang
Characterization of a heparin- ...
Homo sapiens
Biochemistry
48
1517-1524
2009
-
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1
-
9
-
3
9
-
-
1
-
-
1
-
-
1
-
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1
-
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1
-
-
-
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6
-
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1
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1
1
-
9
-
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3
-
9
-
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1
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1
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1
-
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1
-
-
-
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6
-
-
-
-
-
-
-
-
-
-
691466
Kravtsov
Factor XI contributes to throm ...
Homo sapiens
Blood
114
452-458
2009
1
-
1
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-
-
-
-
-
-
-
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1
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1
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1
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1
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2
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-
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-
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-
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-
692544
Wang
A case of factor XI deficiency ...
Homo sapiens
Haemophilia
15
603-606
2009
-
-
-
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1
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1
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1
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-
-
-
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-
-
-
-
-
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-
-
-
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-
707134
Singh
Factor XI deficiency and obste ...
Homo sapiens
Anesth. Analg.
108
1882-1885
2009
-
1
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-
-
-
-
-
-
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1
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1
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-
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-
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-
-
-
-
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-
-
1
1
-
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-
707148
Franchini
The use of desmopressin in con ...
Homo sapiens
Ann. Hematol.
88
931-935
2009
1
1
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-
-
-
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2
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1
1
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-
-
-
-
-
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-
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-
1
1
-
-
-
709721
Gailani
Structural and functional feat ...
Homo sapiens
J. Thromb. Haemost.
7 Suppl 1
75-78
2009
4
-
-
-
-
-
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-
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1
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2
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1
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4
1
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4
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1
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1
-
-
4
1
-
-
-
-
-
-
-
-
-
1
1
-
-
-
709722
Renne
Factor XI deficiency in animal ...
Canis lupus familiaris, Didelphis sp., Felis catus, Homo sapiens, Mus musculus, Ornithorhynchus anatinus
J. Thromb. Haemost.
7 Suppl 1
79-83
2009
4
1
-
-
-
-
-
-
-
-
4
-
-
8
-
-
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-
-
6
-
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2
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-
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4
1
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-
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-
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-
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4
-
-
-
-
-
-
6
-
-
-
2
-
-
-
-
-
-
-
-
1
7
7
1
-
-
710600
Livnat
Recombinant activated factor V ...
Homo sapiens
Thromb. Haemost.
102
487-492
2009
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
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-
-
-
2
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-
-
-
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-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
1
1
-
-
-
683169
Schmidt
Functional role of residue 193 ...
Homo sapiens
Biochemistry
47
1326-1335
2008
-
-
1
-
6
-
4
3
-
1
-
-
-
1
-
-
1
-
-
-
-
-
4
-
1
-
-
2
1
-
-
-
-
-
-
-
-
1
-
-
6
-
-
4
-
3
-
1
-
-
-
-
-
1
-
-
-
-
4
-
1
-
-
2
1
-
-
-
-
-
-
-
-
-
683682
Smith
Characterization of novel form ...
Homo sapiens
J. Biol. Chem.
283
6696-6705
2008
-
-
1
-
2
-
1
3
1
-
-
1
-
1
-
1
1
-
-
2
1
-
4
1
-
-
-
2
-
-
-
-
1
-
-
-
-
1
-
-
2
-
-
1
1
3
1
-
-
1
-
-
1
1
-
2
1
-
4
1
-
-
-
2
-
-
-
-
-
-
-
-
-
-
691470
Kwon
Severe factor XI deficiency in ...
Homo sapiens
Blood Coagul. Fibrinolysis
19
679-683
2008
-
-
-
-
1
-
-
-
-
-
-
-
-
2
-
-
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-
-
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-
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-
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-
-
1
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-
-
-
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-
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-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
693547
Buchanan
Clavatadine A, a natural produ ...
Homo sapiens
J. Med. Chem.
51
3583-3587
2008
-
-
-
1
1
-
2
-
-
-
-
-
-
2
-
-
1
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
1
1
-
2
2
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
695184
Butenas
Factor XIa and tissue factor a ...
Homo sapiens
Thromb. Haemost.
99
142-149
2008
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
683160
Miller
A catalytic domain exosite (Cy ...
Homo sapiens
Biochemistry
46
14450-14460
2007
-
-
1
-
2
-
1
-
1
-
2
1
-
2
-
1
1
-
-
3
-
-
3
1
1
-
-
-
1
-
-
-
1
-
-
-
-
1
-
-
2
-
-
1
1
-
1
-
2
1
-
-
1
1
-
3
-
-
3
1
1
-
-
-
1
-
-
-
-
-
-
-
-
-
683167
Sinha
Macromolecular substrate-bindi ...
Homo sapiens
Biochemistry
46
9830-9839
2007
-
-
1
-
-
-
2
4
1
1
-
1
-
1
-
1
-
-
-
2
-
-
3
1
1
-
-
4
-
-
-
-
6
-
-
-
-
1
-
-
-
-
-
2
6
4
1
1
-
1
-
-
1
-
-
2
-
-
3
1
1
-
-
4
-
-
-
-
-
-
-
-
-
-
684078
Mitchell
Characterisation of five facto ...
Homo sapiens
Thromb. Haemost.
97
884-889
2007
-
-
1
-
6
-
-
-
1
-
-
1
-
1
-
1
-
-
-
1
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
6
-
-
-
-
-
1
-
-
1
-
-
1
-
-
1
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
683157
Rezaie
Contributions of basic amino a ...
Homo sapiens
Biochemistry
45
9427-9433
2006
-
-
1
-
6
-
5
-
1
-
-
1
-
1
-
1
1
-
-
1
-
-
2
-
1
-
-
-
1
-
-
-
1
-
-
-
-
1
-
-
6
-
-
5
1
-
1
-
-
1
-
-
1
1
-
1
-
-
2
-
1
-
-
-
1
-
-
-
-
-
-
-
-
-
683239
Lazarova
Synthesis and in vitro biologi ...
Homo sapiens
Bioorg. Med. Chem. Lett.
16
5022-5027
2006
-
-
-
-
-
-
8
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
7
-
-
-
-
-
-
-
7
8
-
-
-
-
-
1
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
683756
Lin
Design, synthesis, and biologi ...
Homo sapiens
J. Med. Chem.
49
7781-7791
2006
1
1
1
1
1
-
30
-
1
-
-
1
-
3
-
1
-
-
-
1
-
-
3
-
1
-
-
-
1
-
-
-
-
-
30
1
1
1
-
1
1
-
30
30
-
-
1
-
-
1
-
-
1
-
-
1
-
-
3
-
1
-
-
-
1
-
-
-
-
-
-
-
-
-
667059
Jin
Mutation of surface residues t ...
Homo sapiens
Acta Crystallogr. Sect. D
D61
1418-1425
2005
-
1
1
1
-
-
1
-
-
-
1
-
-
1
-
-
1
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
1
1
-
1
-
-
-
1
-
-
-
-
1
-
-
-
-
1
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
667620
Sinha
Factor XIa dimer in the activa ...
Homo sapiens
Biochemistry
44
10416-10422
2005
3
1
1
-
1
-
-
2
-
-
4
-
-
1
-
-
1
-
-
-
-
-
3
2
1
-
-
-
-
-
-
-
-
-
-
3
1
1
-
-
1
-
-
-
-
2
-
-
4
-
-
-
-
1
-
-
-
-
3
2
1
-
-
-
-
-
-
-
-
-
-
-
-
-
669347
Ogawa
Exosite-mediated substrate rec ...
Homo sapiens
J. Biol. Chem.
280
23523-23530
2005
-
-
-
-
-
-
4
3
-
-
2
-
-
1
-
-
1
-
-
1
-
-
3
1
-
-
-
3
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
4
-
3
-
-
2
-
-
-
-
1
-
1
-
-
3
1
-
-
-
3
-
-
-
-
-
-
-
-
-
-
669392
Navaneetham
Structural and mutational anal ...
Homo sapiens
J. Biol. Chem.
280
36165-36175
2005
-
-
1
1
-
-
2
-
-
-
-
-
-
2
-
-
1
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
2
-
-
-
-
-
-
-
-
-
1
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
669420
Jin
Crystal structures of the FXIa ...
Homo sapiens
J. Biol. Chem.
280
4704-4712
2005
3
1
1
1
-
-
3
-
-
-
2
-
-
4
-
-
1
-
-
-
-
-
1
1
-
-
-
-
-
-
-
-
-
-
-
3
1
1
-
1
-
-
-
3
-
-
-
-
2
-
-
-
-
1
-
-
-
-
1
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
669428
Shi
Domain V of beta2-glycoprotein ...
Homo sapiens
J. Biol. Chem.
280
907-912
2005
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
1
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
667583
Pedicord
Substrate-dependent modulation ...
Homo sapiens
Biochemistry
43
11883-11888
2004
-
-
-
-
-
-
2
1
-
-
-
-
-
1
-
-
-
-
-
-
-
-
2
-
-
-
-
1
-
-
-
-
7
-
-
-
-
-
-
-
-
-
-
2
7
1
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
667611
Sinha
Allosteric modification of fac ...
Homo sapiens
Biochemistry
43
7593-7600
2004
-
-
-
-
-
-
4
1
-
-
-
-
-
1
-
-
-
-
-
-
-
-
3
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
4
-
1
-
-
-
-
-
-
-
-
-
-
-
-
3
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
652309
Baird
The interaction of factor XIa ...
Homo sapiens
J. Biol. Chem.
277
38462-38467
2002
-
-
-
-
-
-
1
-
-
-
-
-
-
2
-
-
-
-
-
3
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
3
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
652338
Peek
Unusual proteolytic activation ...
Homo sapiens
J. Biol. Chem.
277
47804-47809
2002
-
-
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
-
-
1
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
1
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
650724
Gailani
Model for a factor IX activati ...
Homo sapiens
Blood
97
3117-3122
2001
-
-
-
-
1
-
-
6
-
-
-
-
-
2
-
-
-
-
-
1
-
-
2
-
-
-
-
6
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
6
-
-
-
-
-
-
-
-
-
1
-
-
2
-
-
-
-
6
-
-
-
-
-
-
-
-
-
-
649981
Badellino
Protease nexin II interactions ...
Homo sapiens
Biochemistry
39
4769-4777
2000
-
-
-
-
-
-
2
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
652013
Baglia
Thrombin-mediated feedback act ...
Homo sapiens
J. Biol. Chem.
275
20514-20519
2000
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
1
-
-
1
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
1
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
36540
Zhang
The mechanism by which heparin ...
Homo sapiens
J. Biol. Chem.
272
26139-26144
1997
-
-
-
-
-
-
1
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36538
Saportio-Irwin
Coagulation factor XIa cleaves ...
Homo sapiens
J. Biol. Chem.
270
26265-26269
1995
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36539
Naito
Activation of human blood coag ...
Homo sapiens
J. Biol. Chem.
266
7353-7358
1991
1
-
-
-
-
-
-
-
-
-
-
-
-
2
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36541
Kalwant
Purification and characterizat ...
Homo sapiens
Biochem. J.
276
237-244
1991
-
-
-
-
-
-
2
1
-
-
2
-
-
3
-
-
1
-
-
2
1
-
1
1
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
2
-
1
-
-
2
-
-
-
-
1
-
2
1
-
1
1
-
-
-
-
1
-
-
-
-
-
-
-
-
-
36531
Meijers
Inhibition of human blood coag ...
Homo sapiens
Biochemistry
27
959-963
1988
-
-
-
-
-
-
1
-
-
-
-
1
-
2
-
-
-
-
-
2
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
1
-
-
-
-
-
2
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36537
Kam
Mechanism-based isocoumarin in ...
Homo sapiens
Biochemistry
27
2547-2557
1988
-
-
-
-
-
-
3
-
-
-
-
1
-
4
-
-
-
-
-
9
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
3
-
-
-
-
-
1
-
-
-
-
-
9
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36536
Sinha
Role of calcium ions and the h ...
Homo sapiens
Biochemistry
26
3768-3775
1987
-
-
-
-
-
-
-
-
-
1
-
1
-
2
-
-
-
-
-
-
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36529
Scott
Human factor XIa cleaves fibri ...
Homo sapiens
Arch. Biochem. Biophys.
249
480-488
1986
1
-
-
-
-
-
-
-
-
-
-
1
-
2
-
-
-
-
-
-
-
-
5
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
5
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36530
Sinha
Functional characterization of ...
Homo sapiens
J. Biol. Chem.
260
10714-10719
1985
-
-
-
-
-
-
-
-
-
-
-
1
-
3
-
-
1
-
-
2
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
1
-
2
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36532
Van der Graaf
Isolation and functional chara ...
Homo sapiens
J. Biol. Chem.
258
9669-9675
1983
-
-
-
-
-
-
-
1
-
1
3
1
-
2
-
-
1
-
-
2
-
-
3
2
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
1
3
1
-
-
-
1
-
2
-
-
3
2
-
-
-
1
-
-
-
-
-
-
-
-
-
-
31351
Lindquist
Activation of bovine factor IX ...
Bos taurus
J. Biol. Chem.
253
1902-1909
1978
-
-
-
-
-
-
-
-
-
-
-
1
-
3
-
-
-
-
-
4
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
4
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
36534
Koide
Bovine factor XI (plasma throm ...
Bos taurus
Methods Enzymol.
45
65-73
1976
-
-
-
-
-
3
-
-
1
1
-
1
-
2
-
1
-
-
-
-
1
1
2
-
1
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
3
-
-
-
-
1
1
-
1
-
-
1
-
-
-
1
1
2
-
1
-
-
-
1
-
-
-
-
-
-
-
-
-
36535
Fujikawa
The mechanism of activation of ...
Bos taurus
Biochemistry
13
4508-4516
1974
-
-
-
-
-
-
-
-
-
-
-
1
-
2
-
-
-
-
-
1
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
1
-
-
2
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-