ENTRY EC 3.4.21.41
NAME Complement subcomponent C1r-
CLASS Hydrolases
Acting on peptide bonds (peptidases)
Serine endopeptidases
REACTION Selective cleavage of Lys(or Arg)+Ile bond in complement
subcomponent C1s to form C1s- (EC 3.4.21.42)
SUBSTRATE Complement subcomponent C1s
H2O
PRODUCT Complement subcomponent C1s-
COMMENT Activated from proenzyme C1r in plasma during activation of the
complement system by the 'classical' route. A homologue of
chymotrypsin.
DISEASE MIM: 216950 Complement component-1, r subcomponent; C1r/C1s
deficiency, combined (1)
MOTIF PS: PS00134 [LIVM]-[ST]-A-[STAG]-H-C
PS: PS00135 [DNSTAGC]-[GSTAPIMVQH]-x(2)-G-[DE]-S-G-[GS]-[SAPHV]-
[LIVMFYWH]-[LIVMFYSTANQH]
GENES HSA: C1R(Hs.1279)
DBLINKS University of Geneva ENZYME DATA BANK: 3.4.21.41
WIT (What Is There) Metabolic Reconstruction: 3.4.21.41
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