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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