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

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