All three pathways merge at the proteolytic cleavage of component C3 by C3 convertases to form two fragments C3b and C3a. The cleavage of component C3 exposes a reactive thioester bond on C3b, leading to the covalent attachment of C3b to glycoproteins on the target cell surface (Law SK et al. 1979; Tack BF et al. 1980). The opsonization with C3b enables the recruitment of phagocytes (Newman SL et al. 1985; Gadjeva M et al. 1998). In addition, C3b anchors the assembly of C3/C5 convertases leading to an amplification of C3 cleavage and effecting C5 activation (Fearon DT 1979; Takata Y et al 1987; Kinoshita T et al. 1988). Moreover, the activation of C3b exposes binding sites for factors B, H and I, properdin, decay accelerating factor (DAF), membrane cofactor protein (MCP), complement receptor 1 (CR1) and microbial molecules such as vaccinia virus complement-control protein and staphylococcal complement inhibitor (SCIN) from Staphylococcus aureus (Forneris F et al. 2010; Morgan HP et al. 2011; Nilsson SC et al. 2010; Lambris JD et al. 1984; Medof ME et al.1984; Barilla-LaBarca ML et al. 2002; Smith BO et al. 2002; Bernet J et al. 2004; Garcia Bl et al. 2010) . C3b associates with these molecules to mediate the activation, amplification and regulation of the complement response.", "cellular_location": "extracellular region, plasma membrane", "input_participant_list": [], "output_participant_list": [], "enzyme_list": [ "P00751-1", "P06681-1" ], "evidence": [ { "id": "R-HSA-166817", "database": "Reactome", "url": "https://www.reactome.org/PathwayBrowser/#R-HSA-166817" } ] }, { "id": "R-HSA-173636", "name": "Formation of classic pathway C5 convertase", "equation": "", "summary": "C5 convertases are serine proteases that cleave C5 with high efficiency; the C3 convertases can cleave C5 but have a poor affinity for C5, with a Km of 6-9 microM. The high affinity C5 convertases are generated when the low affinity C3/C5 convertases such as C4b:C2a deposit C3b by cleaving native C3. These C3b-containing C3/C5 convertases have Km values of 0.005 microM, well below the normal concentration of C5 in blood (0.37 microM). They have very low Vmax rates, just one C5 cleaved per 1\u20134 min per enzyme (Rawal & Pangburn 1998).", "cellular_location": "plasma membrane", "input_participant_list": [], "output_participant_list": [], "enzyme_list": [ "P06681-1" ], "evidence": [ { "id": "R-HSA-173636", "database": "Reactome", "url": "https://www.reactome.org/PathwayBrowser/#R-HSA-173636" } ] }, { "id": "R-HSA-173680", "name": "Activation of C5", "equation": "", "summary": "Cleavage of C5 by C5 convertases is the last enzymatic step in the complement activation. C5 convertases are formed when C3b molecule is covalently deposited in the immediate vicinity of pre-assembled C3 convertases switching them to C5 convertases C4bC2aC3b and C3bBbC3b (Takata Y et al 1987; Kinoshita T et al. 1988; Rawal N and Pangburn MK 2001, 2003). The additional C3b acts like an anvil for C5; it interacts with C5 and presents C5 in the correct conformation for cleavage by the C2a or Bb enzyme. The proteolytic cleavage of C5 generates the small fragment C5a and the large fragment C5b.
C5b initiates an assembly of terminal complement components (C6-C9) leading to the formation of membrane attack complex (MAC) on the target surface (Aleshin AE et al. 2012; Hadders MA et al. 2012). MAC disrupts the cell membrane causing a subsequent cell death through osmotic lysis.
Anaphylatoxin C5a mediates pro-inflammatory and immunemodulatory signals via its receptors C5aR and C5L2. The anaphylatoxin receptors are found on surfaces of phagocytes as well as other cell types. In inflammation, they induce cytokine production, degranulation and chemotaxis of leukocytes (Monk PN et al. 2007).
",
"cellular_location": "extracellular region, plasma membrane",
"input_participant_list": [],
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"P00751-1",
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-173680",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-173680"
}
]
},
{
"id": "R-HSA-977375",
"name": "CR1 binds C3bBb/C4bC2a",
"equation": "",
"summary": "Complement Receptor 1 (CR1) is a widely distributed cell surface protein that is a decay accelerating factor for the conventional (C4bC2a) and alternative (C3bBb) C3 convertases (Coico & Sunshine 2009).",
"cellular_location": "plasma membrane",
"input_participant_list": [],
"output_participant_list": [],
"enzyme_list": [
"P00751-1",
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-977375",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-977375"
}
]
},
{
"id": "R-HSA-977619",
"name": "CD55 (DAF) promotes C3bBb/C4bC2a dissociation",
"equation": "",
"summary": "Decay accelerating factor (DAF, CD55) is a widely distributed membrane protein. It accelerates the dissociation of C3bBb and C4C2a, thereby inhibiting the amplification of complement. DAF can bind C3b and Bb but must bind both for efficient decay acceleration. The regulatory function of DAF is believed to be inhibition of activated C3 convertase enzymes rather than binding of inactive proenzymes (Harris et al. 2007).",
"cellular_location": "extracellular region, plasma membrane",
"input_participant_list": [],
"output_participant_list": [],
"enzyme_list": [
"P00751-1",
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-977619",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-977619"
}
]
},
{
"id": "R-HSA-977629",
"name": "Displacement of C2a/Bb by CR1",
"equation": "",
"summary": "Complement Receptor 1 (CR1) displaces the activated enzyme components Bb and C2a from the conventional and alternative C3 convertases C4bC2a and C3bBb, respectivley.",
"cellular_location": "extracellular region, plasma membrane",
"input_participant_list": [],
"output_participant_list": [],
"enzyme_list": [
"P00751-1",
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-977629",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-977629"
}
]
},
{
"id": "R-HSA-981535",
"name": "CD55 (DAF) binds C3bBb, C4bC2a",
"equation": "",
"summary": "Decay-accelerating-factor (DAF, CD55) is a membrane- bound complement regulatory protein that inhibits autologous complement cascade activation. It is expressed on all cells that are in close contact with serum complement proteins, but also on cells outside the vascular space and on tumour cells. DAF binds to C3bBb and C4bC2a on cell surfaces, accelerating their dissociation and thereby inhibiting the amplification of complement. DAF can bind C3b and Bb, and must bind both for efficient decay acceleration. Although it can bind the inactive proenzymes C3b and C4b, the regulatory function of DAF is believed to be inhibition of activated C3 convertase enzymes (Harris et al. 2007).;",
"cellular_location": "plasma membrane",
"input_participant_list": [],
"output_participant_list": [],
"enzyme_list": [
"P00751-1",
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-981535",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-981535"
}
]
},
{
"id": "R-HSA-981621",
"name": "C3 convertases spontaneously dissociate",
"equation": "",
"summary": "C3b:Bb is naturally labile with a half-life of ~90 s. unless bound to properdin on the cell surface (Medicus et al. 1976). C4bC2a is also unstable, lasting at best a few minutes (Kerr et al. 1980). Decay is associated with the release of the Bb or C2a fragments respectively into the fluid phase. The liberated C3b/C4b is able to re-bind Bb/C2a if Factor B/C2 are present.",
"cellular_location": "extracellular region, plasma membrane",
"input_participant_list": [],
"output_participant_list": [],
"enzyme_list": [
"P00751-1",
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-981621",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-981621"
}
]
},
{
"id": "R-HSA-981648",
"name": "C4b binding protein binds C4bC2a",
"equation": "",
"summary": "C4 binding protein accelerates the decay of C4bC2a in a dose-dependent fashion, without causing degradation of C4b, and is presumed to bind to the convertase to mediate this effect.",
"cellular_location": "plasma membrane",
"input_participant_list": [],
"output_participant_list": [],
"enzyme_list": [
"P06681-1"
],
"evidence": [
{
"id": "R-HSA-981648",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-981648"
}
]
},
{
"id": "R-HSA-981680",
"name": "C4b binding protein displaces C2a",
"equation": "",
"summary": "C4 binding protein accelerates the decay of C4bC2a in a dose-dependent fashion. The mechanism of this is poorly understood, but is distinct from Factor I mediated degradation of C4b and believed to represent the displacement of C2a from specific binding sites on C4b (Gigli et al. 1979).",
"cellular_location": "extracellular region, plasma membrane",
"input_participant_list": [],
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"enzyme_list": [
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"evidence": [
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"id": "R-HSA-981680",
"database": "Reactome",
"url": "https://www.reactome.org/PathwayBrowser/#R-HSA-981680"
}
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}
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