Reference on EC 3.4.21.47 - alternative-complement-pathway C3/C5 convertase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Pangburn, M.K.; Muller-Eberhard, H.J.
The C3 convertase of the alternative pathway of human complement. Enzymic properties of the bimolecular proteinase
Biochem. J.
235
723-730
1986
Homo sapiens
DiScipio, R.G.; Smith, C.A.; Muller-Eberhard, H.J.; Hugli, T.E.
The activation of human complement component C5 by a fluid phase C5 convertase
J. Biol. Chem.
258
10629-10636
1983
Homo sapiens
Gutierrez, J.C.; Gtze, O.; Caporale, L.H.
An improved fluorogenic substrate for the alternative complement pathway C3/5 converting enzyme CVFBb
Biochim. Biophys. Acta
744
276-280
1983
Homo sapiens
Vogel, C.W.; Muller-Eberhard, H.J.
The cobra venom factor-dependent C3 convertase of human complement. A kinetic and thermodynamic analysis of a protease acting on its natural high molecular weight substrate
J. Biol. Chem.
257
8292-8299
1982
Homo sapiens
Schwaeble, W.; Luttig, B.; Sokolowski, T.; Estaller, C.; Weiss, E.H.; Meyer zum Bschenfelde, K.H.; Whaley, K.; Dippold, W.
Human complement factor B: functional properties of a recombinant zymogen of the alternative activation pathway convertase
Immunobiology
188
221-232
1993
Homo sapiens
Williams, S.C.; Sim, R.B.
Dye-ligand affinity purification of human complement factor B and beta 2 glycoprotein I
J. Immunol. Methods
157
25-30
1993
Homo sapiens
Hourcade, D.E.; Mitchell, L.M.; Oglesby, T.J.
A conserved element in the serine protease domain of complement factor B
J. Biol. Chem.
273
25996-26000
1998
Homo sapiens
Sumiyoshi, K.; Andoh, A.; Fujiyama, Y.; Sakumoto, H.; Bamba, T.
Characterization of complement C3, C4, and factor B molecules in human bile
J. Gastroenterol.
32
230-235
1997
Homo sapiens
Fu, Q.; McPhie, P.; Gowda, D.C.
Methionine modification impairs the C5-cleavage function of cobra venom factor-dependent C3/C5 convertase
Biochem. Mol. Biol. Int.
45
133-144
1998
Homo sapiens
Berkovich, SA.; O'Keefe, M.C.; Hensley, P.; Caporale, L.
Effect of N-methylation on the modulation by synthetic peptides of the activity of the complement-factor-B-derived serine proteinase CVFBb
Biochem. J.
270
531-537
1990
Homo sapiens
Jelezarova, E.; Vogt, A.; Lutz, H.U.
Interaction of C3b(2)-IgG complexes with complement proteins properdin, factor B and factor H: implications for amplification
Biochem. J.
349
217-223
2000
Homo sapiens
Pangburn, M.K.; Rawal, N.
Structure and function of complement C5 convertase enzymes
Biochem. Soc. Trans.
30
1006-1010
2002
Homo sapiens
Hourcade, D.E.; Mitchell, L.; Kuttner-Kondo, L.A.; Atkinson, J.P.; Medof, M.E.
Decay-accelerating factor (DAF), complement receptor 1 (CR1), and factor H dissociate the complement AP C3 convertase (C3bBb) via sites on the type A domain of Bb
J. Biol. Chem.
277
1107-1112
2002
Homo sapiens
Rawal, N.; Pangburn, M.K.
Functional role of the noncatalytic subunit of complement C5 convertase
J. Immunol.
164
1379-1385
2000
Homo sapiens
Koelln, J.; Bredehorst, R.; Spillner, E.
Engineering of human complement component C3 for catalytic inhibition of complement
Immunol. Lett.
98
49-56
2005
Homo sapiens
Hourcade, D.E.
The role of properdin in the assembly of the alternative pathway C3 convertases of complement
J. Biol. Chem.
281
2128-2132
2006
Homo sapiens
Selander, B.; Martensson, U.; Weintraub, A.; Holmstroem, E.; Matsushita, M.; Thiel, S.; Jensenius, J.C.; Truedsson, L.; Sjoeholm, A.G.
Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2
J. Clin. Invest.
116
1425-1434
2006
Homo sapiens
Koelln, J.; Spillner, E.; Andrae, J.; Klensang, K.; Bredehorst, R.
Complement inactivation by recombinant human C3 derivatives
J. Immunol.
173
5540-5545
2004
Homo sapiens
Ponnuraj, K.; Xu, Y.; Macon, K.; Moore, D.; Volanakis, J.E.; 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
14
17-28
2004
Homo sapiens (P00751)
Jelezarova, E.; Lutz, H.U.
IgG naturally occurring antibodies stabilize and promote the generation of the alternative complement pathway C3 convertase
Mol. Immunol.
42
1393-1403
2005
Homo sapiens
Huber-Lang, M.; Sarma, J.V.; Zetoune, F.S.; Rittirsch, D.; Neff, T.A.; McGuire, S.R.; Lambris, J.D.; Warner, R.L.; Flierl, M.A.; Hoesel, L.M.; Gebhard, F.; Younger, J.G.; Drouin, S.M.; Wetsel, R.A.; Ward, P.A.
Generation of C5a in the absence of C3: a new complement activation pathway
Nat. Med.
12
682-687
2006
Mus musculus
Moller-Kristensen, M.; Thiel, S.; Sjoeholm, A.; Matsushita, M.; Jensenius, J.C.
Cooperation between MASP-1 and MASP-2 in the generation of C3 convertase through the MBL pathway
Int. Immunol.
19
141-149
2007
Homo sapiens (P00751), Mus musculus (P04186)
Kimura, Y.; Miwa, T.; Zhou, L.; Song, W.C.
Activator-specific requirement of properdin in the initiation and amplification of the alternative pathway complement
Blood
111
732-740
2008
Homo sapiens (P00751), Homo sapiens, Mus musculus (P04186), Mus musculus
Harris, C.L.; Pettigrew, D.M.; Lea, S.M.; Morgan, B.P.
Decay-accelerating factor must bind both components of the complement alternative pathway C3 convertase to mediate efficient decay
J. Immunol.
178
352-359
2007
Homo sapiens (P00751)
Lutz, H.U.; Fumia, S.; Schurtenberger, C.; Alaia, V.
Opinion paper: Stimulation of complement amplification or activation of the alternative pathway of complement?
Mol. Immunol.
44
3862-3865
2007
Homo sapiens (P00751)
Bexborn, F.; Andersson, P.O.; Chen, H.; Nilsson, B.; Ekdahl, K.N.
The tick-over theory revisited: Formation and regulation of the soluble alternative complement C3 convertase (C3(H(2)O)Bb)
Mol. Immunol.
45
2370-2379
2008
Homo sapiens (P00751)
Wiesmann, C.; Katschke, K.J.; Yin, J.; Helmy, K.Y.; Steffek, M.; Fairbrother, W.J.; McCallum, S.A.; Embuscado, L.; DeForge, L.; Hass, P.E.; van Lookeren Campagne, M.
Structure of C3b in complex with CRIg gives insights into regulation of complement activation
Nature
444
217-220
2006
Homo sapiens (P00751)
Ganter, M.T.; Brohi, K.; Cohen, M.J.; Shaffer, L.A.; Walsh, M.C.; Stahl, G.L.; Pittet, J.F.
Role of the alternative pathway in the early complement activation following major trauma
Shock
28
29-34
2007
Homo sapiens (P00751)
Rawal, N.; Rajagopalan, R.; Salvi, V.P.
Activation of complement component C5: comparison of C5 convertases of the lectin pathway and the classical pathway of complement
J. Biol. Chem.
283
7853-7863
2008
Homo sapiens
Ariki, S.; Takahara, S.; Shibata, T.; Fukuoka, T.; Ozaki, A.; Endo, Y.; Fujita, T.; Koshiba, T.; Kawabata, S.
Factor C acts as a lipopolysaccharide-responsive C3 convertase in horseshoe crab complement activation
J. Immunol.
181
7994-8001
2008
Tachypleus tridentatus (B6ZH52)
Torreira, E.; Tortajada, A.; Montes, T.; Rodriguez de Cordoba, S.; 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. USA
106
882-887
2009
Homo sapiens (P00751)
Heinen, S.; Hartmann, A.; Lauer, N.; Wiehl, U.; Dahse, H.M.; Schirmer, S.; Gropp, K.; Enghardt, T.; Wallich, R.; Haelbich, S.; Mihlan, M.; Schloetzer-Schrehardt, U.; Zipfel, P.F.; Skerka, C.
Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation
Blood
114
2439-2447
2009
Homo sapiens
Roumenina, L.T.; Jablonski, M.; Hue, C.; Blouin, J.; Dimitrov, J.D.; Dragon-Durey, M.A.; Cayla, M.; Fridman, W.H.; Macher, M.A.; Ribes, D.; Moulonguet, L.; Rostaing, L.; Satchell, S.C.; Mathieson, P.W.; Sautes-Fridman, C.; Loirat, C.; Regnier, C.H.; Halbwachs-Mecarelli, L.; Fremeaux-Bacchi, V.
Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome
Blood
114
2837-2845
2009
Rattus norvegicus
Lindorfer, M.A.; Pawluczkowycz, A.W.; Peek, E.M.; Hickman, K.; Taylor, R.P.; Parker, C.J.
A novel approach to preventing the hemolysis of paroxysmal nocturnal hemoglobinuria: both complement-mediated cytolysis and C3 deposition are blocked by a monoclonal antibody specific for the alternative pathway of complement
Blood
115
2283-2291
2010
Homo sapiens
Katschke, K.J.; Stawicki, S.; Yin, J.; Steffek, M.; Xi, H.; Sturgeon, L.; Hass, P.E.; Loyet, K.M.; Deforge, L.; Wu, Y.; van Lookeren Campagne, M.; Wiesmann, C.
Structural and functional analysis of a C3b-specific antibody that selectively inhibits the alternative pathway of complement
J. Biol. Chem.
284
10473-10479
2009
Homo sapiens
Torreira, E.; Tortajada, A.; Montes, T.; Rodriguez de Cordoba, S.; Llorca, O.
Coexistence of closed and open conformations of complement factor B in the alternative pathway C3bB(Mg2+) proconvertase
J. Immunol.
183
7347-7351
2009
Homo sapiens
Strobel, S.; Zimmering, M.; Papp, K.; Prechl, J.; Jozsi, M.
Anti-factor B autoantibody in dense deposit disease
Mol. Immunol.
47
1476-1483
2010
Homo sapiens
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.; Gros, P.
Structural and functional implications of the alternative complement pathway C3 convertase stabilized by a staphylococcal inhibitor
Nat. Immunol.
10
721-727
2009
Homo sapiens
Wu, J.; Wu, Y.Q.; Ricklin, D.; Janssen, B.J.; Lambris, J.D.; Gros, P.
Structure of complement fragment C3b-factor H and implications for host protection by complement regulators
Nat. Immunol.
10
728-733
2009
Homo sapiens
Laursen, N.S.; Gordon, N.; Hermans, S.; Lorenz, N.; Jackson, N.; Wines, B.; Spillner, E.; Christensen, J.B.; Jensen, M.; Fredslund, F.; Bjerre, M.; Sottrup-Jensen, L.; Fraser, J.D.; Andersen, G.R.
Structural basis for inhibition of complement C5 by the SSL7 protein from Staphylococcus aureus
Proc. Natl. Acad. Sci. USA
107
3681-3686
2010
Homo sapiens
Camous, L.; Roumenina, L.; Bigot, S.; Brachemi, S.; Fremeaux-Bacchi, V.; Lesavre, P.; Halbwachs-Mecarelli, L.
Complement alternative pathway acts as a positive feedback amplification of neutrophil activation
Blood
117
1340-1349
2011
Homo sapiens
Fridkis-Hareli, M.; Storek, M.; Mazsaroff, I.; Risitano, A.M.; Lundberg, A.S.; Horvath, C.J.; Holers, V.M.
Design and development of TT30, a novel C3d-targeted C3/C5 convertase inhibitor for treatment of human complement alternative pathway-mediated diseases
Blood
118
4705-4713
2011
Homo sapiens
Li, Q.; Li, Y.X.; Stahl, G.L.; Thurman, J.M.; He, Y.; Tong, H.H.
Essential role of factor B of the alternative complement pathway in complement activation and opsonophagocytosis during acute pneumococcal otitis media in mice
Infect. Immun.
79
2578-2585
2011
Mus musculus
Hourcade, D.E.; Mitchell, L.M.
Access to the complement factor B scissile bond is facilitated by association of factor B with C3b protein
J. Biol. Chem.
286
35725-35732
2011
Homo sapiens
Martinez-Barricarte, R.; Heurich, M.; Valdes-Canedo, F.; Vazquez-Martul, E.; Torreira, E.; Montes, T.; Tortajada, A.; Pinto, S.; Lopez-Trascasa, M.; Morgan, B.P.; Llorca, O.; Harris, C.L.; Rodriguez de Cordoba, S.
Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation
J. Clin. Invest.
120
3702-3712
2010
Homo sapiens
Luo, S.; Hartmann, A.; Dahse, H.; Skerka, C.; Zipfel, P.
Secreted pH-regulated antigen 1 of Candida albicans blocks activation and conversion of complement C3
J. Immunol.
185
2164-2173
2010
Homo sapiens
Forneris, F.; Ricklin, D.; Wu, J.; Tzekou, A.; Wallace, R.; Lambris, J.; Gros, P.
Structures of C3b in complex with factors B and D give insight into complement convertase formation
Science
330
1816-1820
2010
Homo sapiens (P01024)
Hebecker, M.; Jozsi, M.
Factor H-related protein 4 activates complement by serving as a platform for the assembly of alternative pathway C3 convertase via its interaction with C3b protein
J. Biol. Chem.
287
19528-19536
2012
Homo sapiens (P00751), Homo sapiens
Lee, M.; Guo, J.P.; McGeer, E.G.; McGeer, P.L.
Aurin tricarboxylic acid self-protects by inhibiting aberrant complement activation at the C3 convertase and C9 binding stages
Neurobiol. Aging
34
1451-1461
2013
Homo sapiens (P00751)
Okroj, M.; Holmquist, E.; King, B.C.; Blom, A.M.
Functional analyses of complement convertases using C3 and C5-depleted sera
PLoS ONE
7
e47245
2012
Homo sapiens (P00751)
Alcorlo, M.; Tortajada, A.; Rodriguez de Cordoba, S.; Llorca, O.
Structural basis for the stabilization of the complement alternative pathway C3 convertase by properdin
Proc. Natl. Acad. Sci. USA
110
13504-13509
2013
Homo sapiens (P00751)
Ruggenenti, P.; Daina, E.; Gennarini, A.; Carrara, C.; Gamba, S.; Noris, M.; Rubis, N.; Peraro, F.; Gaspari, F.; Pasini, A.; Rigotti, A.; Lerchner, R.M.; Santoro, D.; Pisani, A.; Pasi, A.; Remuzzi, G.; Remuzzi, G.
C5 convertase blockade in membranoproliferative glomerulonephritis a single-arm clinical trial
Am. J. Kidney Dis.
74
224-238
2019
Homo sapiens
Berends, E.T.; Gorham, R.D.; Ruyken, M.; Soppe, J.A.; Orhan, H.; Aerts, P.C.; de Haas, C.J.; Gros, P.; Rooijakkers, S.H.
Molecular insights into the surface-specific arrangement of complement C5 convertase enzymes
BMC Biol.
13
93
2015
Homo sapiens
Heesterbeek, D.; Bardoel, B.; Parsons, E.; Bennett, I.; Ruyken, M.; Doorduijn, D.; Gorham, R.J.; Berends, E.; Pyne, A.; Hoogenboom, B.; Rooijakkers, S.
Bacterial killing by complement requires membrane attack complex formation via surface-bound C5 convertases
EMBO J.
38
e99852
2019
Homo sapiens
Zwarthoff, S.A.; Berends, E.T.M.; Mol, S.; Ruyken, M.; Aerts, P.C.; Jozsi, M.; de Haas, C.J.C.; Rooijakkers, S.H.M.; Gorham, R.D.
Functional characterization of alternative and classical pathway C3/C5 convertase activity and inhibition using purified models
Front. Immunol.
9
1691
2018
Homo sapiens
Fridkis-Hareli, M.; Storek, M.; Or, E.; Altman, R.; Katti, S.; Sun, F.; Peng, T.; Hunter, J.; Johnson, K.; Wang, Y.; Lundberg, A.S.; Mehta, G.; Banda, N.K.; Michael Holers, V.
The human complement receptor type 2 (CR2)/CR1 fusion protein TT32, a novel targeted inhibitor of the classical and alternative pathway C3 convertases, prevents arthritis in active immunization and passive transfer mouse models
Mol. Immunol.
105
150-164
2019
Homo sapiens
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