3.4.21.47: alternative-complement-pathway C3/C5 convertase
This is an abbreviated version!
For detailed information about alternative-complement-pathway C3/C5 convertase, go to the full flat file.
Word Map on EC 3.4.21.47
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3.4.21.47
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hemolytic
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igg
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glomerulonephritis
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erythrocyte
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nephritic
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autoantibody
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membranoproliferative
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complement-mediated
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c3nefs
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decay-accelerating
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cell-bound
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opsonization
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fluid-phase
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zymosan
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allotypes
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uremic
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glomerulopathy
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surface-bound
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complement-activating
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c5b
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anaphylatoxins
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glyoxalase
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immunofixation
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hemoglobinuria
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medicine
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eculizumab
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hypocomplementemic
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nef
- 3.4.21.47
-
hemolytic
- igg
- glomerulonephritis
- erythrocyte
-
nephritic
- autoantibody
-
membranoproliferative
-
complement-mediated
-
c3nefs
-
decay-accelerating
-
cell-bound
-
opsonization
-
fluid-phase
- zymosan
- allotypes
-
uremic
- glomerulopathy
-
surface-bound
-
complement-activating
- c5b
- anaphylatoxins
- glyoxalase
-
immunofixation
- hemoglobinuria
- medicine
- eculizumab
-
hypocomplementemic
- nef
Reaction
Cleavage of Arg-/-Ser bond in complement component C3 alpha-chain to yield C3a and C3b, and Arg-/- bond in complement component C5 alpha-chain to yield C5a and C5b =
Synonyms
(C3b)n,Bb, (CVF)-dependent glycine-rich-beta-glucoprotein, alternative C3 convertase, alternative C5 convertase, alternative complement pathway C3 convertase, alternative complement pathway C3(C5) convertase, alternative pathway C3 convertase, alternative pathway C3 convertase complex, alternative pathway C3-convertase, alternative pathway C3/C5 convertase, alternative pathway C5 convertase, alternative pathway of complement C3/C5 convertase, alternative-complement-pathway C3/C5 convertase, AP C3 convertase, AP C3-convertase, AP C3/C5 convertase, AP C5 convertase, APC C3/C5 convertase, C3 convertase, C3 proactivator, C3/C5 convertase, C3b,Bb, C3b2, C3bB complex, C3bBb, C3bBb convertase, C3bBb(C3b)n, C3bBb3b, C3bBbC3b, C5 convertase, cobra venom factor-dependent C3 convertase, complement alternative pathway C3 convertase, complement C 3(C 5) convertase (amplification), complement C5 convertase, complement component C3/C5 convertase (alternative), convertase, complement C3(C5) (amplification), CVF,Bb, CVFh,Bb, CVFn,Bb, GBG, Glycine-rich beta glycoprotein, heat-labile factor, PBF2, proenzyme factor B, properdin factor B
ECTree
3.4.21.47 alternative-complement-pathway C3/C5 convertaseAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 3.4.21.47 - alternative-complement-pathway C3/C5 convertase
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NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
C2 fragment + H2O
?
-
C-terminal fragment originating from the processing of meningococcal proteases
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-
?
complement component C3 zymogen + H2O
complement component C3b + complement component C3a
activation
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-
?
complement component C5 zymogen + H2O
complement component C5b + complement component C5a
activation
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-
?
neisserial heparin binding antigen + H2O
?
-
also known as GNA2132 (genome-derived Neisseria antigen 2132)
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-
?
complement component C3 + H2O
complement component C3a + complement component C3b
-
-
-
-
?
complement component C3 + H2O
complement component C3a + complement component C3b
-
-
-
?
complement component C3 + H2O
complement component C3a + complement component C3b
-
-
-
-
?
complement component C3 + H2O
complement component C3a + complement component C3b
-
-
-
?
complement component C3 + H2O
complement component C3a + complement component C3b
-
complement component C3 is the preferred substrate. Cleavage of the preferred C3 substrate and deposition of C3b effectively switches the output of the enzyme from C3b to C5b, resulting in initiation of the cytosolic process of complement
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?
complement component C3 + H2O
complement component C3a + complement component C3b
-
-
-
?
complement component C3 + H2O
complement component C3a + complement component C3b
-
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
?
complement component C5 + H2O
complement component C5a + complement component C5b
-
-
-
-
?
additional information
?
-
-
the enzyme is involved in the alternative pathway of human complement
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-
?
additional information
?
-
-
the activation peptide, C5a, possesses potent spasmogenic and chemotactic activity
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-
?
additional information
?
-
-
the enzyme has a very short half-life. Dissociation of the two noncovalently bound subunits proceeds with a half-life of 1-3 min at 37°C under physiological conditions, and this rate increases greatly if regulatory proteins are present. Numerous decay-accelerating proteins are present in plasma and on host cells that bind to the noncatalytic subunit C3b and increase the rate at which the catalytic subunit Bb is released into the medium. Bb loses its enzymatic activity and its ability to bind to C3b upon release. Although C3b is able to rebind Bb and reform the enzyme, the interaction with most decay-accelerating factors also leads to permanent proteolytic interaction of the cell-bound subunit C3b by a fluid-phase protease Factor I. These regulatory events limit cleavage of C3, reduce release of the anaphylatoxin C3a and control the formation of more efficient C5 convertase enzymes
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?
