Basic Information | Overview of PTM Sites | Experimental PTM Sites | Protein-Protein Interactions | Drug and Disease Associations | Related Literatures
Basic Information
Protein Name :  Coagulation factor IX  

UniProtKB / Swiss-Prot ID :  FA9_HUMAN

Gene Name (Synonyms) : 
F9  

Species :  Homo sapiens (Human). 

Subcellular Localization :  Secreted. 

Protein Function :  Factor IX is a vitamin K-dependent plasma protein that participates in the intrinsic pathway of blood coagulation by converting factor X to its active form in the presence of Ca(2+) ions, phospholipids, and factor VIIIa. 

Protein Sequence MQRVNMIMAESPGLITICLLGYLLSAECTVFLDHENANKILNRPKRYNSGKLEEFVQGNLERECMEEKCS...
Predicted Secondary Structure CCCCHHHHCCCHHHEEEEEEEEECCCCCCCCCCHHHCCEEECCCCCEEEEECCCCCCCCCCCCCCCCCCC...
Protein Variant
LocationDescription
7I -> F. VAR_006520
17I -> N (in HEMB; severe; UK 22). VAR_006521
28C -> R (in HEMB; moderate; HB130). VAR_006522
28C -> Y (in HEMB). VAR_017343
30V -> I (in HEMB). VAR_006523
37A -> T (in warfarin sensitivity; reducedaffinity of the glutamate carboxylase for
43R -> L (in HEMB; severe; Bendorf, Beuten,Gleiwitz, etc.).
43R -> Q (in HEMB; severe; San Dimas,Oxford-3, Strasbourg-2, etc.).
43R -> W (in HEMB; severe; Boxtel, Heiden,Lienen, etc.).
45K -> N (in HEMB; severe; Seattle E). VAR_006527
46R -> S (in HEMB; severe; Cambridge). VAR_006528
46R -> T (in HEMB; severe). VAR_006529
48N -> I (in HEMB; severe; Calgary-16). VAR_006530
49S -> P (in HEMB). VAR_006531
52L -> S (in HEMB; severe; Gla mutant). VAR_017344
53E -> A (in HEMB; severe; Oxford-B2; Glamutant).
54E -> G (in HEMB; severe; HB151; Glamutant).
55F -> C (in HEMB). VAR_006534
58G -> A (in HEMB; severe; Hong Kong-1). VAR_006535
58G -> R (in HEMB; severe; Los Angeles-4). VAR_006536
62Missing (in HEMB; severe). VAR_006537
66E -> V (in HEMB; moderate). VAR_006538
67E -> K (in HEMB; severe; Nagoya-4; Glamutant).
71F -> S (in HEMB; severe). VAR_006540
73E -> K (in HEMB; severe; Seattle-3; Glamutant).
73E -> V (in HEMB; severe; Chongqing; Glamutant).
75R -> Q (in HEMB; mild). VAR_017308
79E -> D (in HEMB). VAR_017309
84T -> R (in HEMB). VAR_017345
91Y -> C (in HEMB; moderate). VAR_006543
93D -> G (in HEMB; moderate; Alabama). VAR_006544
96Q -> P (in HEMB; severe; New London). VAR_006545
97C -> S (in HEMB). VAR_006546
101P -> R (in HEMB). VAR_006547
102C -> R (in HEMB; severe; Basel). VAR_006548
106G -> D (in HEMB). VAR_017346
106G -> S (in HEMB; mild; Durham). VAR_006549
108C -> S (in HEMB). VAR_006550
110D -> N (in HEMB; severe; Oxford-D1). VAR_006551
112I -> S (in HEMB). VAR_006552
113N -> K (in HEMB; mild). VAR_006553
115Y -> C (in HEMB; severe). VAR_006554
119C -> F (in HEMB; severe). VAR_006555
119C -> R (in HEMB; Iran). VAR_006556
124E -> K (in HEMB). VAR_017347
125G -> E (in HEMB). VAR_006557
125G -> R (in HEMB). VAR_017348
125G -> V (in HEMB). VAR_006558
129Missing (in HEMB). VAR_006559
134C -> Y (in HEMB). VAR_017349
136I -> T (in HEMB; mild). VAR_006560
139G -> D (in HEMB; severe). VAR_006561
139G -> S (in HEMB). VAR_006562
155C -> F (in HEMB; severe). VAR_006563
160G -> E (in HEMB; mild). VAR_006564
167Q -> H (in HEMB; mild). VAR_006565
169S -> C (in HEMB). VAR_017350
170C -> F (in HEMB). VAR_017351
178C -> R (in HEMB). VAR_006566
178C -> W (in HEMB; severe). VAR_006567
191R -> C (in HEMB; moderate; Albuquerque,Cardiff-1, etc.).
191R -> H (in HEMB; moderate; Chapel-Hill,Chicago-2, etc.).
194T -> A (in dbSNP:rs6048). VAR_011773
226R -> G (in HEMB; severe; Madrid). VAR_006571
226R -> Q (in HEMB; severe; Hilo andNovara).
226R -> W (in HEMB; severe; Nagoya-1,Dernbach, Deventer, Idaho, etc.).
227V -> D (in HEMB; mild). VAR_006573
227V -> F (in HEMB; Milano). VAR_017310
228V -> F (in HEMB; severe; Kashihara). VAR_017311
228V -> L (in HEMB; mild; Cardiff-2). VAR_006574
241Q -> H (in HEMB). VAR_006575
241Q -> K (in HEMB). VAR_017352
252C -> S (in HEMB; severe; this is themutation in the index case of the
252C -> Y (in HEMB). VAR_017353
253G -> E (in HEMB; severe). VAR_006576
253G -> R (in HEMB; severe; Luanda). VAR_006577
265A -> T (in HEMB; mild). VAR_006578
268C -> W (in HEMB; moderate). VAR_017313
279A -> T (in HEMB; mild). VAR_006579
283N -> D (in HEMB; severe). VAR_006580
286Missing (in HEMB; severe). VAR_006581
291E -> V (in HEMB; Monschau). VAR_017314
294R -> G (in HEMB; severe). VAR_006582
294R -> Q (in HEMB; mild to moderate;Dreihacken, Penafiel and Seattle-4).
302H -> R (in HEMB). VAR_006584
306N -> S (in HEMB; mild). VAR_017315
316I -> F (in HEMB). VAR_006585
318L -> R (in HEMB). VAR_017354
321L -> Q (in HEMB; severe). VAR_006586
333P -> H (in HEMB; severe). VAR_006587
333P -> T (in HEMB). VAR_017355
342T -> K (in HEMB; mild). VAR_006588
342T -> M (in HEMB; moderate). VAR_006589
344I -> L (in HEMB). VAR_017356
351G -> D (in HEMB). VAR_006590
356W -> C (in HEMB; severe). VAR_006591
357G -> E (in HEMB; severe; Amagasaki). VAR_006592
357G -> R (in HEMB). VAR_017316
362K -> E (in HEMB; moderate). VAR_006593
363G -> W (in HEMB). VAR_006594
366A -> D (in HEMB). VAR_006595
379R -> G (in HEMB; moderate). VAR_006596
379R -> Q (in HEMB; severe; Iceland-1,London and Sesimbra).
382C -> Y (in HEMB). VAR_006598
383L -> F (in HEMB). VAR_017358
383L -> I (in HEMB). VAR_017357
384R -> L (in THPH8; factor IX Padua; higherspecific activity than wild-type).
387K -> E (in HEMB; mild). VAR_006599
390I -> F (in HEMB; severe). VAR_006600
394M -> K (in HEMB). VAR_006601
395F -> I (in HEMB). VAR_017359
395F -> L (in HEMB). VAR_017360
396C -> F (in HEMB). VAR_017361
396C -> S (in HEMB; severe). VAR_006602
397A -> P (in HEMB; mild; Hong Kong-11). VAR_017317
404R -> T (in HEMB). VAR_006603
407C -> R (in HEMB). VAR_017362
407C -> S (in HEMB; severe). VAR_006604
410D -> H (in HEMB; Mechtal). VAR_017318
411S -> G (in HEMB; Varel). VAR_017320
411S -> I (in HEMB; Schmallenberg). VAR_017319
412G -> E (in HEMB). VAR_017363
413G -> R (in HEMB; moderate to severe). VAR_006605
414P -> T (in HEMB; Bergamo). VAR_017321
419V -> E (in HEMB; moderately severe). VAR_006606
424F -> V (in HEMB). VAR_006607
426T -> P (in HEMB; severe; Barcelos). VAR_006608
430S -> T (in HEMB). VAR_006609
431W -> G (in HEMB). VAR_006610
431W -> R (in HEMB; moderate). VAR_006611
432G -> S (in HEMB; severe). VAR_006612
432G -> V (in HEMB; severe). VAR_006613
433E -> A (in HEMB). VAR_006614
433E -> K (in HEMB). VAR_006615
435C -> Y (in HEMB). VAR_017364
436A -> V (in HEMB; moderately severe;Niigata).
442G -> E (in HEMB). VAR_017365
442G -> R (in HEMB; severe; Angers). VAR_017322
443I -> T (in HEMB; moderately severe; LongBeach, Los Angeles and Vancouver).
445T -> TIYT (in HEMB; severe; Lousada). VAR_006617
449R -> Q (in HEMB; mild). VAR_006618
449R -> W (in HEMB; mild). VAR_006619
450Y -> C (in HEMB; severe). VAR_006620
453W -> R (in HEMB). VAR_017324
454I -> T (in HEMB; Italy). VAR_006621
461T -> P (in dbSNP:rs4149751). VAR_014308
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Overview of Protein Modification Sites with Functional and Structural Information
Accessible Surface Area (ASA)
Pred. Secondary
Real Secondary
Disorder Prediction
Protein Domain
&
Experimental PTM Sites
Predicted PTM Sites
Protein Variant
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Experimental Post-Translational Modification Sites Download
Locations
Modification
Substrate Sites
&
Secondary Structure
Accessible Surface Area (%)
Resource
Reference
Structural Characterization
Orthologous
Protein Cluster
534-carboxyglutamateSGKLEEFVQ
EEECCCCCC
53.53HPRD
Link
534-carboxyglutamate.SGKLEEFVQ
EEECCCCCC
53.53UniProtKB
Link
544-carboxyglutamateGKLEEFVQG
EECCCCCCC
62.65HPRD
Link
544-carboxyglutamate.GKLEEFVQG
EECCCCCCC
62.65UniProtKB
Link
614-carboxyglutamateQGNLERECM
CCCCCCCCC
50.89HPRD
Link
614-carboxyglutamate.QGNLERECM
CCCCCCCCC
50.89UniProtKB
Link
634-carboxyglutamateNLERECMEE
CCCCCCCCC
28.92HPRD
Link
634-carboxyglutamate.NLERECMEE
CCCCCCCCC
28.92UniProtKB
Link
664-carboxyglutamateRECMEEKCS
CCCCCCCCC
67.00HPRD
Link
664-carboxyglutamate.RECMEEKCS
CCCCCCCCC
67.00UniProtKB
Link
674-carboxyglutamateECMEEKCSF
CCCCCCCCC
32.26HPRD
Link
674-carboxyglutamate.ECMEEKCSF
CCCCCCCCC
32.26UniProtKB
Link
724-carboxyglutamateKCSFEEARE
CCCCCCEEE
34.16HPRD
Link
724-carboxyglutamate.KCSFEEARE
CCCCCCEEE
34.16UniProtKB
Link
734-carboxyglutamateCSFEEAREV
CCCCCEEEE
51.52HPRD
Link
734-carboxyglutamate.CSFEEAREV
CCCCCEEEE
51.52UniProtKB
Link
764-carboxyglutamateEEAREVFEN
CCEEEEECC
59.62HPRD
Link
764-carboxyglutamate.EEAREVFEN
CCEEEEECC
59.62UniProtKB
Link
794-carboxyglutamateREVFENTER
EEEECCCCC
69.30HPRD
Link
794-carboxyglutamate.REVFENTER
EEEECCCCC
69.30UniProtKB
Link
824-carboxyglutamateFENTERTTE
ECCCCCCCC
60.66HPRD
Link
824-carboxyglutamate.FENTERTTE
ECCCCCCCC
60.66UniProtKB
Link
864-carboxyglutamateERTTEFWKQ
CCCCCCCCC
49.23HPRD
Link
864-carboxyglutamate.ERTTEFWKQ
CCCCCCCCC
49.23UniProtKB
Link
99O-linked (Glc...)./FTId=CAR_000009.DQCESNPCL
CCCCCCCCC
54.41UniProtKB
Link-
99O-linked (Xyl...) (glycosaminoglycan)DQCESNPCL
CCCCCCCCC
54.41OGlycBase
Link-
107O-linked (Fuc)LNGGSCKDD
CCCCEEECC
21.28OGlycBase
Link-
107O-linked (Fuc...).LNGGSCKDD
CCCCEEECC
21.28UniProtKB
Link-
110(3R)-3-hydroxyaspartate.GSCKDDINS
CEEECCCCC
67.37UniProtKB
Link-
114PhosphoserineDDINSYECW
CCCCCCEEE
23.73PhosphoELM
Link-
114Phosphoserine.DDINSYECW
CCCCCCEEE
23.73UniProtKB
Link-
134S-cysteinyl 3-(oxidosulfanyl)alanine (Cys-Cys)LDVTCNIKN
EEECCCCCC
5.07HPRD
Link-
141S-cysteinyl 3-(oxidosulfanyl)alanine (Cys-Cys)KNGRCEQFC
CCCCCCCEE
5.23HPRD
Link-
145S-cysteinyl 3-(oxidosulfanyl)alanine (Cys-Cys)CEQFCKNSA
CCCEEEECC
3.99HPRD
Link-
155S-cysteinyl 3-(oxidosulfanyl)alanine (Cys-Cys)NKVVCSCTE
CCEEEEECC
2.87HPRD
Link-
157S-cysteinyl 3-(oxidosulfanyl)alanine (Cys-Cys)VVCSCTEGY
EEEEECCCC
3.21HPRD
Link-
170S-cysteinyl 3-(oxidosulfanyl)alanine (Cys-Cys)NQKSCEPAV
CCCCCCCCC
10.53HPRD
Link-
191noneSKLTRAETV
CCCCCEECC
40.64HPRD
Link-
201Sulfotyrosine.PDVDYVNST
CCCCCCCCC
11.02UniProtKB
Link-
203N-linked (Glc...)VDYVNSTEA
CCCCCCCCC
36.66HPRD
Link-
203N-linked (GlcNAc...)VDYVNSTEA
CCCCCCCCC
36.66OGlycBase
Link-
203N-linked (GlcNAc...).VDYVNSTEA
CCCCCCCCC
36.66UniProtKB
Link-
204PhosphoserineDYVNSTEAE
CCCCCCCCC
19.99Phosphositeplus
Link-
204Phosphoserine.DYVNSTEAE
CCCCCCCCC
19.99UniProtKB
Link-
205O-linked (GalNAc...)YVNSTEAET
CCCCCCCCC
33.73OGlycBase
Link-
205O-linked (GalNAc...).YVNSTEAET
CCCCCCCCC
33.73UniProtKB
Link-
213N-linked (Glc...)TILDNITQS
CCCCCEECC
35.44HPRD
Link-
213N-linked (GlcNAc...)TILDNITQS
CCCCCEECC
35.44OGlycBase
Link-
213N-linked (GlcNAc...).TILDNITQS
CCCCCEECC
35.44UniProtKB
Link-
215O-linked (GalNAc...)LDNITQSTQ
CCCEECCCE
23.79OGlycBase
Link-
215O-linked (GalNAc...).LDNITQSTQ
CCCEECCCE
23.79UniProtKB
Link-
226noneNDFTRVVGG
CCEECCCCC
22.55HPRD
Link-
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Protein-Protein Interactions
      Interacting Protein      
Interaction type
Source ID
      Resource      
      Pubmed ID      
Domain-Domain Interactions
LRP1_HUMANin vitroHPRD:02385HPRD12522212
THRB_HUMANin vitroHPRD:02385HPRD15039440
FA8_HUMANin vitroHPRD:02385HPRD11278963
11830468
FA7_HUMANin vitroHPRD:02385HPRD2248955
VKGC_HUMANENSP00000218099STRING
VWF_HUMANENSP00000218099STRING
FA11_HUMANENSP00000218099STRING
FURIN_HUMANENSP00000218099STRING
ZFHX3_HUMANENSP00000218099STRING
A4_HUMANENSP00000218099STRING
GPIX_HUMANENSP00000218099STRING
THRB_HUMANENSP00000218099STRING
GPV_HUMANENSP00000218099STRING
GP1BA_HUMANENSP00000218099STRING
TF_HUMANENSP00000218099STRING
FA8_HUMANENSP00000218099STRING
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Disease Reference
Kegg disease
OMIM disease
306900Hemophilia B (HEMB)
Note=Mutations in position 43 (Oxford-3, San Dimas) and 46 (Cambridge) prevents cleavage of the propeptide, mutation in position 93 (Alabama) probably fails to bind to cell membranes, mutation in position 191 (Chapel-Hill) or in position 226 (Nagoya OR Hilo) prevent cleavage of the activation peptide.
300807
Drug Reference
There are no disease associations of PTM sites.
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Related Literatures of Post-Translational Modification
O-linked Glycosylation
ReferencePubMed
"Activation peptide of human factor IX has oligosaccharides O-glycosidically linked to threonine residues at 159 and 169.";
Agarwala K.L., Kawabata S., Takao T., Murata H., Shimonishi Y.,Nishimura H., Iwanaga S.;
Biochemistry 33:5167-5171(1994).
Cited for: GLYCOSYLATION AT THR-205 AND THR-215.
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Basic Information | Overview of PTM Sites | Experimental PTM Sites | Protein-Protein Interactions | Drug and Disease Associations | Related Literatures