Literature for peptidase S01.196: complement factor Bb

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates Pharma

(Topics flags: A Assay, S Structure, M Mutation, K Knockout, P Specificity, I Inhibitor, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

    2026
  1. Ma,C., Su,J., Liu,J., Zhao,T., Li,X., Cai,S., Ji,X., Wu,J., Wang,L. and Xu,D.<br>Discovery of (3R,4R)-15: An Advanced Factor B Inhibitor Entering Phase 3 for Complement-Mediated Diseases<br>J Med Chem (2026) 69, 867-894. PubMed  Europe PubMed DOI
  2. 2025
  3. Kavanagh,D., Barratt,J., Schubart,A., Webb,N.J.A., Meier,M. and Fakhouri,F.<br>Factor B as a therapeutic target for the treatment of complement-mediated diseases<br>Front Immunol (2025) 16, 1537974-1537974. PubMed  Europe PubMed DOI  PMC  EPMC  V
  4. 2023
  5. Schubart,A., Flohr,S., Junt,T. and Eder,J.<br>Low-molecular weight inhibitors of the alternative complement pathway<br>Immunol Rev (2023) 313, 339-357. PubMed  Europe PubMed DOI
  6. 2022
  7. Laich,A., Patel,H., Zarantonello,A., Sim,R.B. and Inal,J.M.<br>C2 by-pass: Cross-talk between the complement classical and alternative pathways<br>Immunobiology (2022) 227, 152225-152225. PubMed  Europe PubMed DOI
  8. Lorentzen,J., Pedersen,D.V., Gadeberg,T.A.F. and Andersen,G.R.<br>Structure determination of an unstable macromolecular complex enabled by nanobody-peptide bridging<br>Protein Sci (2022) 31, e4432-e4432. PubMed  Europe PubMed DOI  PMC  EPMC
  9. 2021
  10. Takahashi,K., Banda,N.K., Holers,V.M. and Van Cott,E.M.<br>Complement component factor B has thrombin-like activity<br>Biochem Biophys Res Commun (2021) 552, 17-22. PubMed  Europe PubMed DOI  PMC  EPMC
  11. 2020
  12. Mainolfi,N., Ehara,T., Karki,R.G., Anderson,K., Mac Sweeney,A., Liao,S.M., Argikar,U.A., Jendza,K., Zhang,C., Powers,J., Klosowski,D.W., Crowley,M., Kawanami,T., Ding,J., April,M., Forster,C., Serrano-Wu,M., Capparelli,M., Ramqaj,R., Solovay,C., Cumin,F., Smith,T.M., Ferrara,L., Lee,W., Long,D., Prentiss,M., de Erkenez,A., Yang,L., Liu,F., Sellner,H., Sirockin,F., Valeur,E., Erbel,P., Ostermeier,D., Ramage,P., Gerhartz,B., Schubart,A., Flohr,S., Gradoux,N., Feifel,R., Vogg,B., Wiesmann,C., Maibaum,J., Eder,J., Sedrani,R., Harrison,R.A., Mogi,M., Jaffee,B.D. and Adams,C.M.<br>Discovery of 4-((2S,4S)-4-ethoxy-1-((5-methoxy-7-methyl-1H-indol-4-yl)methyl)piperidin-2-yl)benzoic acid (LNP023), a factor B inhibitor specifically designed to be applicable to treating a diverse array of complement mediated diseases<br>J Med Chem (2020) PubMed  Europe PubMed DOI  I
  13. 2019
  14. Peng,M., Li,Z., Niu,D., Liu,X., Dong,Z. and Li,J.<br>Complement factor B/C2 in molluscs regulates agglutination and illuminates evolution of the Bf/C2 family<br>FASEB J (2019) 33, 13323-13333. PubMed  Europe PubMed DOI
  15. Schubart,A., Anderson,K., Mainolfi,N., Sellner,H., Ehara,T., Adams,C.M., Mac Sweeney,A., Liao,S.M., Crowley,M., Littlewood-Evans,A., Sarret,S., Wieczorek,G., Perrot,L., Dubost,V., Flandre,T., Zhang,Y., Smith,R.J.H., Risitano,A.M., Karki,R.G., Zhang,C., Valeur,E., Sirockin,F., Gerhartz,B., Erbel,P., Hughes,N., Smith,T.M., Cumin,F., Argikar,U.A., Haraldsson,B., Mogi,M., Sedrani,R., Wiesmann,C., Jaffee,B., Maibaum,J., Flohr,S., Harrison,R. and Eder,J.<br>Small-molecule factor B inhibitor for the treatment of complement-mediated diseases<br>Proc Natl Acad Sci U S A (2019) 116, 7926-7931. PubMed  Europe PubMed DOI  I
  16. 2013
  17. Slade,C., Bosco,J., Unglik,G., Bleasel,K., Nagel,M. and Winship,I.<br>Deficiency in complement factor B<br>N Engl J Med (2013) 369, 1667-1669. PubMed  Europe PubMed DOI  M
  18. 2012
  19. Forneris,F., Wu,J. and Gros,P.<br>The modular serine proteases of the complement cascade<br>Curr Opin Struct Biol (2012) 22, 333-341. PubMed  Europe PubMed DOI  V
  20. Kerr,M.A.<br>Factor B and the alternative pathway C3/C5 convertase<br>[ISSN:978-0-12-407742-3] (2012) 3, 2869-2875. DOI
  21. 2011
  22. Laursen,N.S., Andersen,K.R., Braren,I., Spillner,E., Sottrup-Jensen,L. and Andersen,G.R.<br>Substrate recognition by complement convertases revealed in the C5-cobra venom factor complex<br>EMBO J (2011) 30, 606-616. PubMed  Europe PubMed DOI
  23. 2010
  24. Forneris,F., Ricklin,D., Wu,J., Tzekou,A., Wallace,R.S., Lambris,J.D. and Gros,P.<br>Structures of C3b in complex with factors B and D give insight into complement convertase formation<br>Science (2010) 330, 1816-1820. PubMed  Europe PubMed DOI
  25. 2009
  26. Krishnan,V., Ponnuraj,K., Xu,Y., Macon,K., Volanakis,J.E. and Narayana,S.V.<br>The crystal structure of cobra venom factor, a cofactor for C3- and C5-convertase CVFBb<br>Structure (2009) 17, 611-619. PubMed  Europe PubMed DOI  PMC  EPMC
  27. Rooijakkers,S.H., Wu,J., Ruyken,M., van Domselaar,R., Planken,K.L., Tzekou,A., Ricklin,D., Lambris,J.D., Janssen,B.J., van Strijp,J.A. and Gros,P.<br>Structural and functional implications of the alternative complement pathway C3 convertase stabilized by a staphylococcal inhibitor<br>Nat Immunol (2009) 10, 721-727. PubMed  Europe PubMed DOI  PMC  EPMC  S
  28. Ruiz-Gomez,G., Lim,J., Halili,M.A., Le,G.T., Madala,P.K., Abbenante,G. and Fairlie,D.P.<br>Structure-activity relationships for substrate-based inhibitors of human complement factor B<br>J Med Chem (2009) 52, 6042-6052. PubMed  Europe PubMed DOI  I
  29. Torreira,E., Tortajada,A., Montes,T., Rodriguez de Cordoba,S. and Llorca,O.<br>3D structure of the C3bB complex provides insights into the activation and regulation of the complement alternative pathway convertase<br>Proc Natl Acad Sci U S A (2009) 106, 882-887. PubMed  Europe PubMed DOI  PMC  EPMC
  30. 2008
  31. Bexborn,F., Andersson,P.O., Chen,H., Nilsson,B. and Ekdahl,K.N.<br>The tick-over theory revisited: formation and regulation of the soluble alternative complement C3 convertase (C3(H2O)Bb)<br>Mol Immunol (2008) 45, 2370-2379. PubMed  Europe PubMed DOI  PMC  EPMC
  32. 2007
  33. Goicoechea de Jorge,E., Harris,C.L., Esparza-Gordillo,J., Carreras,L., Arranz,E.A., Garrido,C.A., Lopez-Trascasa,M., Sanchez-Corral,P., Morgan,B.P. and Rodriguez,D.C.<br>Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome<br>Proc Natl Acad Sci USA (2007) 104, 240-245. PubMed  Europe PubMed DOI  M
  34. Harris,C.L., Pettigrew,D.M., Lea,S.M. and Morgan,B.P.<br>Decay-accelerating factor must bind both components of the complement alternative pathway C3 convertase to mediate efficient decay<br>J Immunol (2007) 178, 352-359. PubMed  Europe PubMed
  35. Le,G.T., Abbenante,G. and Fairlie,D.P.<br>Profiling the enzymatic properties and inhibition of human complement factor B<br>J Biol Chem (2007) 282, 34809-34816. PubMed  Europe PubMed DOI  A  P  I
  36. Milder,F.J., Gomes,L., Schouten,A., Janssen,B.J., Huizinga,E.G., Romijn,R.A., Hemrika,W., Roos,A., Daha,M.R. and Gros,P.<br>Factor B structure provides insights into activation of the central protease of the complement system<br>Nat Struct Mol Biol (2007) 14, 224-228. PubMed  Europe PubMed DOI  S
  37. 2005
  38. Yoshizaki,F.Y., Ikawa,S., Satake,M., Satoh,N. and Nonaka,M.<br>Structure and the evolutionary implication of the triplicated complement factor B genes of a urochordate ascidian, Ciona intestinalis<br>Immunogenetics (2005) 56, 930-942. PubMed  Europe PubMed DOI
  39. 2004
  40. Bhattacharya,A.A., Lupher,M.L., Jr., Staunton,D.E. and Liddington,R.C.<br>Crystal structure of the A domain from complement factor B reveals an integrin-like open conformation<br>Structure (2004) 12, 371-378. PubMed  Europe PubMed DOI
  41. Kerr,M.A.<br>Factor B and the alternative pathway C3/C5 convertase<br>[ISSN:0-12-079610-4] (2004) 2, 1632-1637.  V
  42. [YEAR:9-4-2004]Ponnuraj,K., Xu,Y., Macon,K., Moore,D., Volanakis,J.E. and Narayana,S.V.<br>Structural analysis of engineered Bb fragment of complement factor B: insights into the activation mechanism of the alternative pathway C3-convertase<br>Mol Cell (9-4-2004) 14, 17-28. PubMed  Europe PubMed DOI  S
  43. 2002
  44. Nakao,M., Matsumoto,M., Nakazawa,M., Fujiki,K. and Yano,T.<br>Diversity of complement factor B/C2 in the common carp (Cyprinus carpio): three isotypes of B/C2-A expressed in different tissues<br>Dev Comp Immunol (2002) 26, 533-541. PubMed  Europe PubMed DOI
  45. 2001
  46. Xu,Y., Narayana,S.V. and Volanakis,J.E.<br>Structural biology of the alternative pathway convertase<br>Immunol Rev (2001) 180, 123-135. PubMed  Europe PubMed DOI
  47. 2000
  48. [YEAR:1-9-2000]Hinshelwood,J. and Perkins,S.J.<br>Conformational changes during the assembly of factor B from its domains by (1)H NMR spectroscopy and molecular modelling: their relevance to the regulation of factor B activity<br>J Mol Biol (1-9-2000) 301, 1267-1285. PubMed  Europe PubMed DOI
  49. [YEAR:21-4-2000]Hinshelwood,J. and Perkins,S.J.<br>Metal-dependent conformational changes in a recombinant vWF-A domain from human factor B: a solution study by circular dichroism, fourier transform infrared and (1)H NMR spectroscopy<br>J Mol Biol (21-4-2000) 298, 135-147. PubMed  Europe PubMed DOI
  50. [YEAR:17-1-2000]Jing,H., Xu,Y.Y., Carson,M., Moore,D., Macon,K.J., Volanakis,J.E. and Narayana,S.V.L.<br>New structural motifs on the chymotrypsin fold and their potential roles in complement factor B<br>EMBO J (17-1-2000) 19, 164-173. PubMed  Europe PubMed DOI  PMC  EPMC  S
  51. Sim,R.B. and Laich,A.<br>Serine proteases of the complement system<br>Biochem Soc Trans (2000) 28, 545-550. PubMed  Europe PubMed
  52. [YEAR:7-1-2000]Xu,Y., Circolo,A., Jing,H., Wang,Y., Narayana,S.V. and Volanakis,J.E.<br>Mutational analysis of the primary substrate specificity pocket of complement factor B. Asp(226) is a major structural determinant for P(1)-Arg binding<br>J Biol Chem (7-1-2000) 275, 378-385. PubMed  Europe PubMed DOI
  53. 1999
  54. [YEAR:1-3-1999]Hourcade,D.E., Mitchell,L.M. and Oglesby,T.J.<br>Mutations of the type A domain of complement factor B that promote high-affinity C3b-binding<br>J Immunol (1-3-1999) 162, 2906-2911. PubMed  Europe PubMed
  55. 1998
  56. Geserick,G., Otremba,P., Schroder,H., Stradmann-Bellinghausen,B. and Schneider,P.M.<br>Reference typing report for complement factor B<br>Exp Clin Immunogenet (1998) 15, 261-263. PubMed  Europe PubMed
  57. [YEAR:2-10-1998]Hourcade,D.E., Mitchell,L.M. and Oglesby,T.J.<br>A conserved element in the serine protease domain of complement factor B<br>J Biol Chem (2-10-1998) 273, 25996-26000. PubMed  Europe PubMed DOI
  58. [YEAR:16-1-1998]Taylor,P.R., Nash,J.T., Theodoridis,E., Bygrave,A.E., Walport,M.J. and Botto,M.<br>A targeted disruption of the murine complement factor B gene resulting in loss of expression of three genes in close proximity, factor B, C2, and D17H6S45<br>J Biol Chem (16-1-1998) 273, 1699-1704. PubMed  Europe PubMed DOI  K
  59. 1994
  60. Fritzinger,D.C., Bredehorst,R. and Vogel,C.W.<br>Molecular cloning and derived primary structure of cobra venom factor<br>Proc Natl Acad Sci U S A (1994) 91, 12775-12779. PubMed  Europe PubMed DOI  PMC  EPMC
  61. [YEAR:15-11-1994]Kato,Y., Salter-Cid,L., Flajnik,M.F., Kasahara,M., Namikawa,C., Sasaki,M. and Nonaka,M.<br>Isolation of the Xenopus complement factor B complementary DNA and linkage of the gene to the frog MHC<br>J Immunol (15-11-1994) 153, 4546-4554. PubMed  Europe PubMed
  62. 1993
  63. [YEAR:6-7-1993]<br>Nomenclature for human complement factor B. WHO-IUIS Nomenclature Sub-Committee<br>J Immunol Methods (6-7-1993) 163, 9-11. PubMed  Europe PubMed DOI
  64. Horiuchi,T., Kim,S., Matsumoto,M., Watanabe,I., Fujita,S. and Volanakis,J.E.<br>Human complement factor B: cDNA cloning, nucleotide sequencing, phenotypic conversion by site-directed mutagenesis and expression<br>Mol Immunol (1993) 30, 1587-1592. PubMed  Europe PubMed DOI
  65. [YEAR:15-10-1993]Kjalke,M., Welinder,K.G. and Koch,C.<br>Structural analysis of chicken factor B-like protease and comparison with mammalian complement proteins factor B and C2<br>J Immunol (15-10-1993) 151, 4147-4152. PubMed  Europe PubMed
  66. [YEAR:1-10-1993]Perkins,S.J. and Smith,K.F.<br>Identity of the putative serine-proteinase fold in proteins of the complement system with nine relevant crystal structures<br>Biochem J (1-10-1993) 295, 109-114. PubMed  Europe PubMed  PMC  EPMC  S
  67. Schwaeble,W., Luttig,B., Sokolowski,T., Estaller,C., Weiss,E.H., Meyer zum Buschenfelde,K.H., Whaley,K. and Dippold,W.<br>Human complement factor B: functional properties of a recombinant zymogen of the alternative activation pathway convertase<br>Immunobiology (1993) 188, 221-232. PubMed  Europe PubMed DOI
  68. 1992
  69. Kam,C.M., Oglesby,T.J., Pangburn,M.K., Volanakis,J.E. and Powers,J.C.<br>Substituted isocoumarins as inhibitors of complement serine proteases<br>J Immunol (1992) 149, 163-168. PubMed  Europe PubMed  I
  70. 1991
  71. [YEAR:1-3-1991]Niemann,M.A., Bhown,A.S. and Miller,E.J.<br>The principal site of glycation of human complement factor B<br>Biochem J (1-3-1991) 274, 473-480. PubMed  Europe PubMed  PMC  EPMC
  72. 1990
  73. Sanchez-Corral,P., Anton,L.C., Alcolea,J.M., Marques,G., Sanchez,A. and Vivanco,F.<br>Proteolytic activity of the different fragments of factor B on the third component of complement (C3). Involvement of the N-terminal domain of Bb in magnesium binding<br>Mol Immunol (1990) 27, 891-900. PubMed  Europe PubMed DOI
  74. Volanakis,J.E.<br>Participation of C3 and its ligands in complement activation<br>Curr Top Microbiol Immunol (1990) 153, 1-21. PubMed  Europe PubMed
  75. 1987
  76. [YEAR:15-3-1987]Kam,C.M., McRae,B.J., Harper,J.W., Niemann,M.A., Volanakis,J.E. and Powers,J.C.<br>Human complement proteins D, C2, and B. Active site mapping with peptide thioester substrates<br>J Biol Chem (15-3-1987) 262, 3444-3451. PubMed  Europe PubMed
  77. 1983
  78. Campbell,R.D. and Porter,R.R.<br>Molecular cloning and characterization of the gene coding for human complement protein factor B<br>Proc Natl Acad Sci U S A (1983) 80, 4464-4468. PubMed  Europe PubMed  PMC  EPMC
  79. 1982
  80. [YEAR:10-9-1982]Smith,C.A., Vogel,C.W. and Muller-Eberhard,H.J.<br>Ultrastructure of cobra venom factor-dependent C3/C5 convertase and its zymogen, factor B of human complement<br>J Biol Chem (10-9-1982) 257, 9879-9882. PubMed  Europe PubMed
  81. 1981
  82. Kerr,M.A.<br>Human factor B<br>Methods Enzymol (1981) 80, 102-112. PubMed  Europe PubMed DOI  V