Coagulation Factor IX (F9)

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HEMB; FIX; GLA Domain; PTC; Anti Hemophilic Factor B; Christmas Factor; Plasma Thromboplastic Component; Christmas Disease; Hemophilia B

Coagulation Factor IX (F9)
  • Factor IX  is one of the serine proteases (EC 3.4.21.22) of the coagulation system; it belongs to peptidase family S1. Deficiency of this protein causes hemophilia B. It was discovered after a young boy named Stephen Christmas was found to be lacking this exact factor, leading to hemophilia, in 1952. Factor IX is inactive unless activated by factor XIa (of the contact pathway) or factor VIIa (of the tissue factor pathway). When activated into factor IXa, in the presence of Ca2+, membrane phospholipids, and a Factor VIII cofactor, it hydrolyses one arginine-isoleucine bond in factor X to form factor Xa.Deficiency of factor IX causes Christmas disease (hemophilia B). Over 100 mutations of factor IX have been described; some cause no symptoms, but many lead to a significant bleeding disorder.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA840Hu01 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA840Hu01 Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
MAA840Hu22 Monoclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
LAA840Hu91 HRP-Linked Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC.
MAA840Hu21 Monoclonal Antibody to Coagulation Factor IX (F9) WB
MAA840Hu23 Monoclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
Assay Kits SEA840Hu ELISA Kit for Coagulation Factor IX (F9) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA840Mu01 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
RPA840Mu02 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA840Mu01 Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
PAA840Mu02 Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
Assay Kits SEA840Mu ELISA Kit for Coagulation Factor IX (F9) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA840Ra01 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA840Ra01 Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
MAA840Ra21 Monoclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
Assay Kits SEA840Ra ELISA Kit for Coagulation Factor IX (F9) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Oryctolagus cuniculus (Rabbit)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA840Rb01 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
RPA840Rb02 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
RPA840Rb03 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA840Rb51 Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
LAA840Rb71 Biotin-Linked Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC.
MAA840Rb21 Monoclonal Antibody to Coagulation Factor IX (F9) WB
MAA840Rb22 Monoclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Coagulation Factor IX (F9) CLIA Kit Customized Service Offer
n/a ELISA Kit for Coagulation Factor IX (F9) ELISA Kit Customized Service Offer

Organism species: Bos taurus; Bovine (Cattle)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA840Bo01 Recombinant Coagulation Factor IX (F9) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA840Bo01 Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC; IP.
LAA840Bo71 Biotin-Linked Polyclonal Antibody to Coagulation Factor IX (F9) WB; IHC; ICC.
Assay Kits n/a CLIA Kit for Coagulation Factor IX (F9) CLIA Kit Customized Service Offer
n/a ELISA Kit for Coagulation Factor IX (F9) ELISA Kit Customized Service Offer
  1. "Isolation and characterization of a cDNA coding for human factor IX."Proc. Natl. Acad. Sci. U.S.A. 79:6461-6464(1982) [PubMed] [Europe PMC] [Abstract]
  2. "Isolation of a human anti-haemophilic factor IX cDNA clone using a unique 52-base synthetic oligonucleotide probe deduced from the amino acid sequence of bovine factor IX."Nucleic Acids Res. 11:2325-2335(1983) [PubMed] [Europe PMC] [Abstract]
  3. "The gene structure of human anti-haemophilic factor IX."EMBO J. 3:1053-1060(1984) [PubMed] [Europe PMC] [Abstract]
  4. "Nucleotide sequence of the gene for human factor IX (antihemophilic factor B)."Biochemistry 24:3736-3750(1985) [PubMed] [Europe PMC] [Abstract]
  5. "Evidence for a prevalent dimorphism in the activation peptide of human coagulation factor IX."Proc. Natl. Acad. Sci. U.S.A. 82:2847-2851(1985) [PubMed] [Europe PMC] [Abstract]
  6. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  7. "The DNA sequence of the human X chromosome." Nature 434:325-337(2005) [PubMed] [Europe PMC] [Abstract]
  8. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  9. "The Arg-4 mutant factor IX Strasbourg 2 shows a delayed activation by factor XIa."Nouv. Rev. Fr. Hematol. 35:473-480(1993) [PubMed] [Europe PMC] [Abstract]
  10. "Isolation and characterization of human factor IX cDNA: identification of Taq I polymorphism and regional assignment."Somat. Cell Mol. Genet. 10:465-473(1984) [PubMed] [Europe PMC] [Abstract]
  11. "Blood clotting factor IX BM Nagoya. Substitution of arginine 180 by tryptophan and its activation by alpha-chymotrypsin and rat mast cell chymase."J. Biol. Chem. 264:21257-21265(1989) [PubMed] [Europe PMC] [Abstract]
  12. "Modification of the N-terminus of human factor IX by defective propeptide cleavage or acetylation results in a destabilized calcium-induced conformation: effects on phospholipid binding and activation by factor XIa."Biochem. J. 323:629-636(1997) [PubMed] [Europe PMC] [Abstract]
  13. "Genomic amplification with transcript sequencing."Science 239:491-494(1988) [PubMed] [Europe PMC] [Abstract]
  14. "A deletion located in the 3' non translated part of the factor IX gene responsible for mild haemophilia B."Thromb. Haemost. 70:370-371(1993) [PubMed] [Europe PMC] [Abstract]
  15. "The occurrence of beta-hydroxyaspartic acid in the vitamin K-dependent blood coagulation zymogens."Biochem. Biophys. Res. Commun. 115:8-14(1983) [PubMed] [Europe PMC] [Abstract]
  16. "Activation of human factor IX (Christmas factor)."J. Clin. Invest. 61:1528-1538(1978) [PubMed] [Europe PMC] [Abstract]
  17. "Derivatives of blood coagulation factor IX contain a high affinity Ca2+-binding site that lacks gamma-carboxyglutamic acid."J. Biol. Chem. 259:5698-5704(1984) [PubMed] [Europe PMC] [Abstract]
  18. ErratumJ. Biol. Chem. 260:2583-2583(1985)
  19. "Identification of a disaccharide (Xyl-Glc) and a trisaccharide (Xyl2-Glc) O-glycosidically linked to a serine residue in the first epidermal growth factor-like domain of human factors VII and IX and protein Z and bovine protein Z."J. Biol. Chem. 264:20320-20325(1989) [PubMed] [Europe PMC] [Abstract]
  20. "A new trisaccharide sugar chain linked to a serine residue in the first EGF-like domain of clotting factors VII and IX and protein Z."Adv. Exp. Med. Biol. 281:121-131(1990) [PubMed] [Europe PMC] [Abstract]
  21. "Role of gamma-carboxyglutamic acid residues in the binding of factor IXa to platelets and in factor-X activation."Blood 79:398-405(1992) [PubMed] [Europe PMC] [Abstract]
  22. "Human factor IX has a tetrasaccharide O-glycosidically linked to serine 61 through the fucose residue."J. Biol. Chem. 267:17520-17525(1992) [PubMed] [Europe PMC] [Abstract]
  23. "Activation peptide of human factor IX has oligosaccharides O-glycosidically linked to threonine residues at 159 and 169."Biochemistry 33:5167-5171(1994) [PubMed] [Europe PMC] [Abstract]
  24. "Partial phosphorylation of serine-68 in EGF-1 of human factor IX."(In) Proceedings of XIth international conference on methods in protein structure analysis, pp.50-50, Annecy (1996)
  25. "Posttranslational modifications of recombinant myotube-synthesized human factor IX."Blood 97:130-138(2001) [PubMed] [Europe PMC] [Abstract]
  26. "Physiological fIXa activation involves a cooperative conformational rearrangement of the 99-loop."J. Biol. Chem. 278:4121-4126(2003) [PubMed] [Europe PMC] [Abstract]
  27. "Rumi functions as both a protein O-glucosyltransferase and a protein O-xylosyltransferase."Proc. Natl. Acad. Sci. U.S.A. 108:16600-16605(2011) [PubMed] [Europe PMC] [Abstract]
  28. "Identification of protein O-glycosylation site and corresponding glycans using liquid chromatography-tandem mass spectrometry via mapping accurate mass and retention time shift."J. Chromatogr. A 1371:136-145(2014) [PubMed] [Europe PMC] [Abstract]
  29. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  30. "Structure of the metal-free gamma-carboxyglutamic acid-rich membrane binding region of factor IX by two-dimensional NMR spectroscopy."J. Biol. Chem. 270:7980-7987(1995) [PubMed] [Europe PMC] [Abstract]
  31. "Structure of the calcium ion-bound gamma-carboxyglutamic acid-rich domain of factor IX."Biochemistry 34:12126-12137(1995) [PubMed] [Europe PMC] [Abstract]
  32. "Identification of the phospholipid binding site in the vitamin K-dependent blood coagulation protein factor IX."J. Biol. Chem. 271:16227-16236(1996) [PubMed] [Europe PMC] [Abstract]
  33. "Refinement of the NMR solution structure of the gamma-carboxyglutamic acid domain of coagulation factor IX using molecular dynamics simulation with initial Ca2+ positions determined by a genetic algorithm."Biochemistry 36:2132-2138(1997) [PubMed] [Europe PMC] [Abstract]
  34. "Sequence-specific 1H NMR assignments, secondary structure, and location of the calcium binding site in the first epidermal growth factor like domain of blood coagulation factor IX."Biochemistry 30:7402-7409(1991) [PubMed] [Europe PMC] [Abstract]
  35. "The three-dimensional structure of the first EGF-like module of human factor IX: comparison with EGF and TGF-alpha."Protein Sci. 1:81-90(1992) [PubMed] [Europe PMC] [Abstract]
  36. "The structure of a Ca(2+)-binding epidermal growth factor-like domain: its role in protein-protein interactions."Cell 82:131-141(1995) [PubMed] [Europe PMC] [Abstract]
  37. "Coagulation factor IXa: the relaxed conformation of Tyr99 blocks substrate binding."Structure 7:989-996(1999) [PubMed] [Europe PMC] [Abstract]
  38. "Crystal structure of the calcium-stabilized human factor IX Gla domain bound to a conformation-specific anti-factor IX antibody."J. Biol. Chem. 279:14338-14346(2004) [PubMed] [Europe PMC] [Abstract]
  39. "Structural basis of the cofactor- and substrate-assisted activation of human coagulation factor IXa."Structure 17:1669-1678(2009) [PubMed] [Europe PMC] [Abstract]
  40. "Studies of benzothiophene template as potent factor IXa (FIXa) inhibitors in thrombosis."J. Med. Chem. 53:1465-1472(2010) [PubMed] [Europe PMC] [Abstract]
  41. "Structure based drug design: development of potent and selective factor IXa (FIXa) inhibitors." J. Med. Chem. 53:1473-1482(2010) [PubMed] [Europe PMC] [Abstract]
  42. "Molecular basis of factor IXa recognition by heparin-activated antithrombin revealed by a 1.7-A structure of the ternary complex."Proc. Natl. Acad. Sci. U.S.A. 107:645-650(2010) [PubMed] [Europe PMC] [Abstract]
  43. "Molecular pathology of haemophilia B."EMBO J. 8:1067-1072(1989) [PubMed] [Europe PMC] [Abstract]
  44. "Assessing the underlying pattern of human germline mutations: lessons from the factor IX gene."FASEB J. 6:2767-2774(1992) [PubMed] [Europe PMC] [Abstract]
  45. "Haemophilia B: database of point mutations and short additions and deletions -- fourth edition, 1993."Nucleic Acids Res. 21:3075-3087(1993) [PubMed] [Europe PMC] [Abstract]
  46. "Identification of the molecular defect in factor IX Chapel Hill: substitution of histidine for arginine at position 145."Proc. Natl. Acad. Sci. U.S.A. 80:4200-4202(1983) [PubMed] [Europe PMC] [Abstract]
  47. "Defective propeptide processing of blood clotting factor IX caused by mutation of arginine to glutamine at position -4."Cell 45:343-348(1986) [PubMed] [Europe PMC] [Abstract]
  48. "Factor IXAlabama: a point mutation in a clotting protein results in hemophilia B."Blood 69:140-143(1987) [PubMed] [Europe PMC] [Abstract]
  49. "Genetic defect responsible for the dysfunctional protein: factor IX (Long Beach)."Blood 72:820-822(1988) [PubMed] [Europe PMC] [Abstract]
  50. "Blood clotting factor IX Niigata: substitution of alanine-390 by valine in the catalytic domain."J. Biochem. 104:878-880(1988) [PubMed] [Europe PMC] [Abstract]
  51. "Functional consequences of an arginine180 to glutamine mutation in factor IX Hilo."Blood 73:1540-1544(1989) [PubMed] [Europe PMC] [Abstract]
  52. "Mutations in the catalytic domain of human coagulation factor IX: rapid characterization by direct genomic sequencing of DNA fragments displaying an altered melting behavior."Genomics 4:266-272(1989) [PubMed] [Europe PMC] [Abstract]
  53. "Functionally important regions of the factor IX gene have a low rate of polymorphism and a high rate of mutation in the dinucleotide CpG."Am. J. Hum. Genet. 45:448-457(1989) [PubMed] [Europe PMC] [Abstract]
  54. "Factor IX Cardiff: a variant factor IX protein that shows abnormal activation is caused by an arginine to cysteine substitution at position 145."Br. J. Haematol. 72:556-560(1989) [PubMed] [Europe PMC] [Abstract]
  55. "Blood clotting factor IX Kashihara: amino acid substitution of valine-182 by phenylalanine."J. Biochem. 105:756-759(1989) [PubMed] [Europe PMC] [Abstract]
  56. "Factor IX San Dimas. Substitution of glutamine for Arg-4 in the propeptide leads to incomplete gamma-carboxylation and altered phospholipid binding properties."J. Biol. Chem. 264:11401-11406(1989) [PubMed] [Europe PMC] [Abstract]
  57. "Three point mutations in the factor IX genes of five hemophilia B patients. Identification strategy using localization by altered epitopes in their hemophilic proteins."J. Clin. Invest. 84:113-118(1989) [PubMed] [Europe PMC] [Abstract]
  58. "Factor IX Chongqing: a new mutation in the calcium-binding domain of factor IX resulting in severe hemophilia B."Thromb. Haemost. 63:24-26(1990) [PubMed] [Europe PMC] [Abstract]
  59. "A mutation adjacent to the beta cleavage site of factor IX (valine 182 to leucine) results in mild haemophilia Bm."Br. J. Haematol. 75:217-221(1990) [PubMed] [Europe PMC] [Abstract]
  60. "Mutations in hemophilia Bm occur at the Arg180-Val activation site or in the catalytic domain of factor IX."J. Biol. Chem. 265:10876-10883(1990) [PubMed] [Europe PMC] [Abstract]
  61. "Factor IX Amagasaki: a new mutation in the catalytic domain resulting in the loss of both coagulant and esterase activities."Biochemistry 30:11286-11291(1991) [PubMed] [Europe PMC] [Abstract]
  62. "Isoleucine-397 is changed to threonine in two females with hemophilia B."Nucleic Acids Res. 19:1165-1165(1991) [PubMed] [Europe PMC] [Abstract]
  63. "Hemophilia B caused by five different nondeletion mutations in the protease domain of factor IX."Blood 79:1225-1232(1992) [PubMed] [Europe PMC] [Abstract]
  64. "Characterization of the original Christmas disease mutation (cysteine 206-->serine): from clinical recognition to molecular pathogenesis."Thromb. Haemost. 67:63-65(1992) [PubMed] [Europe PMC] [Abstract]
  65. "Single-strand conformation polymorphism (SSCP) analysis of the molecular pathology of hemophilia B."Hum. Mutat. 2:355-361(1993) [PubMed] [Europe PMC] [Abstract]
  66. "Factor IX gene mutations causing haemophilia B: comparison of SSC screening versus systematic DNA sequencing and diagnostic applications."Hum. Genet. 94:287-290(1994) [PubMed] [Europe PMC] [Abstract]
  67. "A novel mutation (Val-373 to Glu) in the catalytic domain of factor IX, resulting in moderately/severe hemophilia B in a southern French patient."Hum. Mutat. 3:156-158(1994) [PubMed] [Europe PMC] [Abstract]
  68. "Identification of mutations in four hemophilia B patients of Turkish origin, including a novel deletion of base 6411."Hum. Mutat. 4:163-165(1994) [PubMed] [Europe PMC] [Abstract]
  69. "Twenty-five novel mutations of the factor IX gene in haemophilia B."Hum. Mutat. 6:346-348(1995) [PubMed] [Europe PMC] [Abstract]
  70. "A mutation in the propeptide of factor IX leads to warfarin sensitivity by a novel mechanism."J. Clin. Invest. 98:1619-1625(1996) [PubMed] [Europe PMC] [Abstract]
  71. "Mutations associated with hemophilia B in Turkish patients."Hum. Mutat. 10:76-79(1997) [PubMed] [Europe PMC] [Abstract]
  72. "Hemophilia B in a female carrier due to skewed inactivation of the normal X-chromosome."Am. J. Hematol. 58:72-76(1998) [PubMed] [Europe PMC] [Abstract]
  73. "Five novel factor IX mutations in unrelated hemophilia B patients."Hum. Mutat. Suppl. 1:S301-S303(1998) [PubMed] [Europe PMC] [Abstract]
  74. "Germline mutations in Peruvian patients with hemophilia B: pattern of mutation in Amerindians is similar to the putative endogenous germline pattern."Hum. Mutat. 11:372-376(1998) [PubMed] [Europe PMC] [Abstract]
  75. "Molecular analysis of hemophilia B in Poland: 12 novel mutations of the factor IX gene."Acta Biochim. Pol. 46:721-726(1999) [PubMed] [Europe PMC] [Abstract]
  76. "Identification of twenty-one new mutations in the factor IX gene by SSCP analysis."Hum. Mutat. 13:160-165(1999) [PubMed] [Europe PMC] [Abstract]
  77. "Characterization of single-nucleotide polymorphisms in coding regions of human genes."Nat. Genet. 22:231-238(1999) [PubMed] [Europe PMC] [Abstract]
  78. ErratumNat. Genet. 23:373-373(1999)
  79. "Factor IX gene sequencing by a simple and sensitive 15-hour procedure for haemophilia B diagnosis: identification of two novel mutations."Br. J. Haematol. 111:549-551(2000) [PubMed] [Europe PMC] [Abstract]
  80. "Molecular pathology of haemophilia B in Turkish patients: identification of a large deletion and 33 independent point mutations."Br. J. Haematol. 120:656-659(2003) [PubMed] [Europe PMC] [Abstract]
  81. "Molecular analyses in hemophilia B families: identification of six new mutations in the factor IX gene."Haematologica 88:235-236(2003) [PubMed] [Europe PMC] [Abstract]
  82. "X-linked thrombophilia with a mutant factor IX (factor IX Padua)."N. Engl. J. Med. 361:1671-1675(2009) [PubMed] [Europe PMC] [Abstract]
  83. "Genetic determinants of immunogenicity to factor IX during the treatment of haemophilia B."Haemophilia 21:210-218(2015) [PubMed] [Europe PMC] [Abstract]