Information on EC 3.4.21.47 - alternative-complement-pathway C3/C5 convertase and Organism(s) Mus musculus and UniProt Accession P04186

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Mus musculus
UNIPROT: P04186


The taxonomic range for the selected organisms is: Mus musculus

The enzyme appears in selected viruses and cellular organisms

EC NUMBER
COMMENTARY hide
3.4.21.47
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RECOMMENDED NAME
GeneOntology No.
alternative-complement-pathway C3/C5 convertase
CAS REGISTRY NUMBER
COMMENTARY hide
80295-67-6
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SwissProt
Manually annotated by BRENDA team
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
complement component C3 + H2O
complement component C3a + complement component C3b
show the reaction diagram
complement component C5 + H2O
complement component C5a + complement component C5b
show the reaction diagram
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
complement component C3 + H2O
complement component C3a + complement component C3b
show the reaction diagram
complement component C5 + H2O
complement component C5a + complement component C5b
show the reaction diagram
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
mannan-binding lectin
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mannan-binding lectin (MBL) promotes activation of complement component C3 through the combined action of MBL-associated serine proteases MASP-1 and MASP-2 without appreciable involvement of the alternative pathway
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properdin
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facilitates AP complement activation by stabilizing the C3 convertase C3bBb, essential for AP complement activation induced by bacterial lipopolysacharide (LPS) and lipooligosacharide (LOS) and other AP complement activators
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
Streptococcus pneumoniae induces increased gene expression of factor B of the alternative complement pathway and C3 in mouse middle ear epithelium. Activation of factor B and C3 in the middle ear lavage fluids is significantly greater than in simultaneously obtained serum samples
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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enzyme component C3 deletion mutant. Acute lung inflammatory injury is greatly attenuated in lungs of mutant animals, and C5a levels in broncheolar lavage fluids of mutants greatly reduced in presence of antithrombin III or hirudin, compared to wild-type. Plasma from mutant animals contains threefold higher levels of thrombin activity and higher levels of prothrombin mRNA, prothrombin and thrombin protein in liver of mutants
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
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studies on role of properdin in AP complement initiation for understanding the selective predisposition of properdin-deficient patients to meningococcal infection
medicine
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Streptococcus pneumoniae induces increased gene expression of factor B of the alternative complement pathway and C3 in mouse middle ear epithelium. Activation of factor B and C3 in the middle ear lavage fluids is significantly greater than in simultaneously obtained serum samples. Complement C3 activation and opsonophagocytosis of Streptococcus pneumoniae are greatly attenuated in factor B- and factor B/C2-deficient mice. Local complement activation is an important host innate immune response and activation of the alternative complement pathway represents one of the innate immune defense mechanisms against pneumococcal infection during the early stage of acute otitis media