Any feedback?
Please rate this page
(enzyme.php)
(0/150)

BRENDA support

BRENDA Home
show all | hide all No of entries

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

for references in articles please use BRENDA:EC3.4.21.47
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
EC Tree
Specify your search results
Select one or more organisms in this record: ?
This record set is specific for:
Mus musculus
UNIPROT: P04186 not found.
Show additional data
Do not include text mining results
Include (text mining) results
Include results (AMENDA + additional results, but less precise)
Word Map
The taxonomic range for the selected organisms is: Mus musculus
The enzyme appears in selected viruses and cellular organisms
Reaction Schemes
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
c3bbb, c5 convertase, properdin factor b, alternative pathway c3 convertase, c3b,bb, heat-labile factor, c3/c5 convertase, c3 proactivator, cvf,bb, ap c3 convertase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
alternative-complement-pathway C3/C5 convertase
-
C3 convertase
-
(C3b)n,Bb
-
-
-
-
(CVF)-dependent glycine-rich-beta-glucoprotein
-
-
-
-
alternative complement pathway C3(C5) convertase
-
-
-
-
C3 convertase
-
-
-
-
C3 proactivator
-
-
-
-
C3/C5 convertase
-
-
-
-
C3b,Bb
-
-
-
-
C5 convertase
-
-
-
-
cobra venom factor-dependent C3 convertase
-
-
-
-
complement C 3(C 5) convertase (amplification)
-
-
-
-
complement component C3/C5 convertase (alternative)
-
-
-
-
convertase, complement C3(C5) (amplification)
-
-
-
-
CVF,Bb
-
-
-
-
GBG
-
-
-
-
Glycine-rich beta glycoprotein
-
-
-
-
heat-labile factor
-
-
-
-
PBF2
-
-
-
-
proenzyme factor B
-
-
-
-
properdin factor B
-
-
-
-
CAS REGISTRY NUMBER
COMMENTARY hide
80295-67-6
-
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 SUBSTRATE
NATURAL 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
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
mannan-binding lectin
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
-
properdin
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
-
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
SwissProt
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
CFAB_MOUSE
761
0
85004
Swiss-Prot
Secretory Pathway (Reliability: 1)
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
-
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
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
-
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
studies on role of properdin in AP complement initiation for understanding the selective predisposition of properdin-deficient patients to meningococcal infection
medicine
-
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
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
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
Manually annotated by BRENDA team
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)
Manually annotated by BRENDA team
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
Manually annotated by BRENDA team
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
Manually annotated by BRENDA team